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Alagille Syndrome. Valencia, CA: Children’s Liver Association for Support Services. 2006. 2 p.

This brochure describes Alagille syndrome, a multi-system hereditary disorder that often presents with symptoms involving the liver during infancy and early childhood. The classic syndrome involves five distinct findings: chronic cholestasis (reduced bile flow), congenital heart disease, bone defects, eye findings, and typical facial features. The management of children with Alagille syndrome is aimed primarily at preventing complications and treating symptoms. Treatment includes vitamin supplementation, drug therapy for pruritus (itching), and liver transplantation. The brochure concludes with a description of the work and goals of the Children’s Liver Association for Support Services (CLASS), a non-profit organization dedicated to addressing the needs of families coping with childhood liver disease and transplantation. The brochure also includes a form with which readers can request to be placed on the mailing list of CLASS.

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Biliary Atresia. Valencia, CA: Children’s Liver Association for Support Services. 2006. 2 p.

Biliary atresia is a progressive inflammatory process that begins soon after birth. In this condition, bile is trapped inside the liver and rapidly causes damage and scarring to the liver cells. This brochure discusses the diagnosis, treatment, and possible complications related to this pediatric liver disease called biliary atresia. The usual history of the disease is a full term infant who appears normal at birth but develops jaundice after the age of two to three weeks. The infant has yellow eyes and skin, light colored stools and dark urine caused by the buildup of bilirubin in the blood. Once the diagnosis is confirmed, the preferred treatment is to remove the blocked biliary ducts outside the liver and attach the small intestine directly to the liver at the spot where bile is found or expected to drain (this operation is called the Kasai procedure). Bile flow is re-established in approximately 80 percent of infants who are operated on when younger than three months of age. Therefore, early diagnosis is very important. The author notes that liver transplantation also plays an important role in the long-term treatment of biliary atresia. The brochure concludes with a description of the work and goals of the Children’s Liver Association for Support Services (CLASS), a non-profit organization dedicated to addressing the needs of families coping with childhood liver disease and transplantation.

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Cholestasis Post Liver Transplantation. IN: Lindor, K.; Talwalkar, J., eds. Cholestatic Liver Disease. Totowa, NJ: Humana Press. 2008. pp 171-182.

This chapter on cholestasis that occurs after liver transplantation is from a book that offers health care providers an overview of cholestatic liver disease; cholestasis is defined as a liver disorder characterized by impaired bile flow. The chapter covers biliary complications, preservation or reperfusion injury and ABO incompatibility, small-for-size syndrome, hepatic artery thrombosis, infectious complications, drug-induced acute cellular rejection, chronic rejection, and recurrent disease, including primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC), and viral hepatitis. The authors caution that cholestasis can occur anytime throughout the posttransplant period, may be intrahepatic or extrahepatic in origin, and has a very broad differential diagnosis. Careful diagnostic imaging of the biliary tree is an important first step in the workup, followed by liver biopsy if clinically indicated. 1 table. 50 references.

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Diagnosis and Treatment of Autoimmune Hepatitis. Gastroenterology Clinics of North America. 37(2): 461-478.June 2008.

This article about the diagnosis and treatment of autoimmune hepatitis is from an issue of Gastroenterology Clinics of North America that focuses on eosinophilic and autoimmune gastrointestinal disease. The authors describe autoimmune hepatitis (AIH) as a hepatitis of unknown cause, known as idiopathic, characterized by inflammation of the liver, presence of auto-antibodies, and evidence of increased gamma globulins in the serum. AIH is considered to be an interaction between the immune system, auto-antigens, and unknown triggering factors. The authors provide a brief summary of the diagnosis of AIH, epidemiologic factors, the natural history of AIH, an approach to the treatment and follow-up of AIH, and the role of liver transplantation in the treatment of AIH. This immune disease affecting the liver responds well to prednisone or a combination of prednisone and azathioprine; most patients can be brought into remission, although many will require maintenance therapy with low-dose levels of these drugs. The authors stress that not all patients with AIH need to be treated, even once the diagnosis is confirmed. Drug therapy should be considered in patients who have cirrhosis if biopsy demonstrates considerable inflammation. Liver transplantation should be considered in patients who have decompensated cirrhosis from AIH or in those patients who have severe fulminant hepatitis who fail to respond to initial therapy. 3 figures. 3 tables. 60 references.

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Gut Eosinophilia in Food Allergy and Systemic and Autoimmune Diseases. Gastroenterology Clinics of North America. 37(2): 307-332. June 2008.

This article about gut eosinophilia in food allergy and in systemic and autoimmune diseases is from an issue of Gastroenterology Clinics of North America that focuses on eosinophilic and autoimmune gastrointestinal disease. Eosinophilic gastroenteritis is a rare disease characterized by marked tissue eosinophilia in any layer of the gut wall; however, many diseases can cause increased gut eosinophilia. The first section of the article focuses on allergic reactions to food, which are an important cause of gut eosinophilia. Not all adverse reactions to food are IgE mediated, and most cases of IgE-mediated food allergy do not have eosinophilic gastroenteritis. The authors performed a literature review of these heterogeneous conditions and summarize their findings in this article. Specific conditions covered include parasitic infections, Helicobacter pylori infections, cytomegalovirus, drug-associated eosinophilia, liver transplantation, acute graft-versus-host disease (GVHD), inflammatory fibroid polyps, hypereosinophilic syndrome, systemic sclerosis, systemic lupus erythematosus, pseudotumoral enterocolitis, paraneoplastic syndrome, and malignant T-cell lymphoma. 2 figures. 3 tables. 144 references.

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Hepatitis C. Annals of Internal Medicine. 148(11): 1-16. June 2008.

This continuing education program, an In The Clinic supplement to the Annals of Internal Medicine, reviews the prevention, screening, diagnosis, and treatment of hepatitis C. The material presents the answers to common questions in each of these areas, supplemented by evidence from clinical studies that supports the implementation of best practices. Topics include the risk factors for hepatitis C virus (HCV) infection; how to reduce the risk of HCV transmission, particularly in the health care setting; screening and diagnostic tests for HCV infection; the symptoms of HCV, which range from asymptomatic individuals to chronic infection to liver cirrhosis; the indications for liver biopsy; the use of dietary and other lifestyle interventions in the management of HCV infection, including complementary and alternative medicine (CAM) approaches; when drug therapy for HCV is appropriate and the contraindications for drug therapy; treatment regimens and how to choose which drug regimen is appropriate for which patient; vaccinating patients with HCV; the clinical management of patients who do not respond to hepatitis C therapy or who relapse after an initial response; the side effects of hepatitis C drugs and strategies to minimize and manage side effects; how to evaluate the response to hepatitis C drug therapy; liver transplantation in patients with HCV infection; and the risks for hepatocellular carcinoma in patients with HCV infection, including the indications for routine screening for this cancer. A final section considers strategies for practice improvement. Appended to the instructional materials is a patient handout with basic information about hepatitis C, as well as a posttest with which readers can obtain continuing medical education (CME) credits. 1 figure. 4 tables. 53 references.

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Primary Biliary Cirrhosis. IN: Lindor, K.; Talwalkar, J., eds. Cholestatic Liver Disease. Totowa, NJ: Humana Press. 2008. pp 45-66.

This chapter on primary biliary cirrhosis (PBC) is from a book that offers health care providers an overview of cholestatic liver disease; cholestasis is defined as a liver disorder characterized by impaired bile flow. Primary biliary cirrhosis is a chronic progressive cholestatic liver disease that primarily affects middle-aged women. The chapter covers pathogenesis, epidemiology, clinical features, diagnosis, treatment, natural history and prognosis, and liver transplantation for PBC. The authors note that the pathogenesis of this disease is unknown, but some research points to genetic and environmental factors that may initiate the autoimmune process. Patients are often asymptomatic at the time of their diagnosis, but as inflammation destroys the bile ducts and fibrosis develops, symptoms of fatigue and pruritus may become present. Ursodeoxycholic acid (UDCA) is the only recommended treatment and may improve liver biochemistries, delay progression of fibrosis and development of esophageal varices, and may improve survival in selected patients. Liver transplantation is the only definitive therapy once end-stage liver disease occurs. 1 table. 153 references.

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Primary Sclerosing Cholangitis. IN: Lindor, K.; Talwalkar, J., eds. Cholestatic Liver Disease. Totowa, NJ: Humana Press. 2008. pp 67-84. Available from Humana Press. 999 Riverview Dr, Suite 208, Totowa, NJ 07512. Email: humana@humanapr.com. Website: www.humanapress.com. Price: $89.95.

This chapter on primary sclerosing cholangitis (PSC) is from a book that offers health care providers an overview of cholestatic liver disease; cholestasis is defined as a liver disorder characterized by impaired bile flow. Primary sclerosing cholangitis is a chronic, progressive cholestatic liver disease characterized by fibrosis of the intrahepatic and extrahepatic bile ducts. PSC is often associated with inflammatory bowel disease (IBD). The chapter covers diagnosis, pathogenesis, natural history, malignancy risk, and management of patients with PSC. The author notes that liver biopsy may be useful in helping establish the diagnosis of PSC, particularly if the patient’s cholangiograms are normal. PSC is a progressive disease that slowly advances over time and may shorten life expectancy. The most severe complication that can develop is bile duct cancer. No effective medical therapy is yet available for the underlying disease; however, liver transplantation is an option for patients with end-stage liver disease. 4 figures. 1 table. 91 references.

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Cirrhosis: What You Need to Know About This Potentially Life-Threatening Liver Condition. New York, NY: American Liver Foundation. 2007. 2 p.

This brochure from the American Liver Foundation reviews the physiology of the liver and the problems associated with cirrhosis, which is defined as the replacement of normal liver tissue with nonliver scar tissue. As cirrhosis continues, the liver is left without enough functioning tissue to perform properly. The liver is needed to convert food into nutrients and stored energy, detoxify substances that are harmful to the body, process medications, store vitamins and minerals, and make bile, which is used for the digestion of fats. Written in a question-and-answer format, the brochure discusses the causes of cirrhosis; alcoholism and cirrhosis; other causes of cirrhosis, including chronic viral hepatitis, nonalcoholic steatohepatitis (NASH), and bile duct disease; inherited diseases and cirrhosis; the symptoms and complications of cirrhosis; diagnostic tests that may be used to confirm cirrhosis; and treatment options. In the early stages of alcoholic cirrhosis, an effective treatment is abstaining from alcohol and following a nutritious diet. In advanced stages, cirrhosis is a life-threatening condition that can only be treated with liver transplantation. The back cover of the brochure lists facts about cirrhosis in a summarized format. Contact information for the American Liver Foundation is provided.

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Diagnosis and Long-Term Management of Wilson Disease. Gastroenterology and Hepatology. 3(1): 27-29. January 2007.

This article answers common questions that physicians may have regarding the diagnosis and long-term management of patients with Wilson disease. The article begins with a review of the pathophysiology of Wilson disease, an inherited disorder of copper metabolism. Dietary copper is absorbed by the gut and accumulates in the liver, leading to oxidative damage within the liver cells; this damage leads to the development of steatohepatitis, followed by fibrosis and cirrhosis. The author describes the natural course of the disease, the typical initial presentation of patients with Wilson disease, treatment options for these patients, salvage therapy other than transplantation for patients with advanced chronic Wilson disease, the prioritization of patients with Wilson disease for liver transplantation, the long-term prognosis of Wilson disease patients who respond to standard medical therapy, and associated conditions and complications in older patients with Wilson disease. 4 references.

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Endoscopic Management of Primary Sclerosing Cholangitis: State of the Art. American Journal of Gastroenterology. 102:S32-S37 p. 2007.

Primary sclerosing cholangitis (PSC) is a chronic, idiopathic disorder of the bile ducts that progresses from inflammation to fibrotic stricturing in the bile ducts, which in turn leads to biliary obstruction and, ultimately, chronic liver disease. This article brings readers up-to-date on the use of endoscopy in the management of patients with PSC. Fifty to 80 percent of patients with PSC have coexisting inflammatory bowel disease (IBD), more commonly presenting as ulcerative colitis (UC) than Crohn’s disease. The author discusses the clinical presentation of PSC, diagnostic approaches, the medical and surgical treatments used to manage PSC, cholangiographic findings and endoscopic management, and managing strictures. The author describes endoscopic retrograde cholangiopancreatography (ERCP). ERCP is a technique that has increasingly-limited purely diagnostic application in the management of PSC but is of great use in tissue acquisition and in the treatment of cholestatic symptoms in the long-term management of the complications of PSC, notably those due to biliary strictures and bile duct stones. Liver transplantation remains the only definitive treatment for PSC. 3 figures. 32 references.

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Liver Transplants: What You Need to Know. New York, NY: American Liver Foundation. 2007. 2 p.

Liver transplantation is needed for patients who are likely to die because of liver failure. In a living donor transplantation, a segment of a healthy person’s liver is transplanted into the sick patient. In cadaveric donation, the deceased donors typically die of accidents or head injuries and have arranged in advance to be an organ donor or their family grants permission for organ donation. This brochure from the American Liver Foundation reviews the indications for liver transplantation and what recipients can expect before and after a liver transplant. Written in a question-and-answer format, the brochure discusses when liver transplants may be needed; eligibility for liver transplant; the average amount of time patients wait for a donated liver; where donated livers come from; what happens during transplant surgery; the risks of liver transplants; the side effects after receiving a liver transplant, including side effects due to immunosuppressive drugs; the lifestyle changes that may happen after liver transplantation; the prognosis for liver recipients; and the importance of becoming registered as an organ donor. The back cover of the brochure lists facts about transplantation in a summarized format. Contact information for the American Liver Foundation is provided.

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Management of Alcoholic Hepatitis. Gastroenterology and Hepatology. 3(2): 97-99. February 2007.

This article answers common questions physicians may have about the management of alcoholic hepatitis. Topics covered include the pathophysiology of alcoholic hepatitis, the presenting symptoms and attributes of patients with alcoholic hepatitis, the typical course of treatment in these patients, the impact of cessation of drinking alcohol on the course and effects of alcoholic hepatitis, concerns regarding liver transplantation for patients with severe alcoholic hepatitis, and new research in this area. The hallmark of alcoholic hepatitis is jaundice. Most clinicians in the United States do not perform a liver biopsy to make the diagnosis; rather, they base diagnosis on a long history of alcohol use, elevated bilirubin, characteristic AST and ALT values, and no evidence of other liver diseases. The author cautions that approximately 20 to 50 percent of people who have alcoholic hepatitis also have chronic hepatitis C infection. After diagnosis, most patients are treated with oral prednisolone or pentoxifylline. Abstinence from alcohol is required for long-term survival. 6 references.

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Pediatric Liver Transplantation. Gastroenterology and Hepatology. 3(3): 194-196. March 2007.

This article answers questions physicians may have about pediatric liver transplantation, one-third of which is performed to treat biliary atresia, a fibro-inflammatory disorder of the extrahepatobiliary ducts. The author reports on the experiences with pediatric liver transplantation at his institution, describing how patients are referred to the transplant center, how monitoring and supportive care are provided to pediatric transplant candidates, specific concerns for pediatric transplantation, the donor pool for pediatric transplant candidates, and the inclusion of age as a factor for determining a patient’s prioritization within the donor pool. The author notes that most children receive a technical variant gift, which is a reduced-size, split, or living-donor graft rather than a whole liver graft. The advantages of this approach are to reduce mortality on the transplant waiting list. However, these techniques incur a heightened risk of additional postoperative complications following the very complex surgical procedures required with this type of transplantation. 7 references.

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Screening And Outcomes in Biliary Atresia: Summary of a National Institutes of Health Workshop. Hepatology. 46(2): 566-581. August 2007.

Biliary atresia is a congenital disorder characterized by persistent jaundice and progressive cholestasis, caused by the obstruction of both extrahepatic and intrahepatic bile ducts. This article summarizes a National Institutes of Health (NIH) workshop held in Bethesda, Maryland, in September 2006, on screening and outcomes in biliary atresia, a common cause of end-stage liver disease in infants and the leading indication for liver transplantation in children. The authors stress that earlier diagnosis, when the child is younger than 30 to 45 days of life, is associated with improved outcomes following the Kasai portoenterostomy and longer survival with their native liver. However, early diagnosis is not easy because the problem is quite rare, there is a high incidence of hyperbilirubinemia in the newborn period, and routine health care visits occur after the child is 1 month of age. The prognosis after portoenterostomy include the age of the child at surgery, the center's experience, the presence of associated congenital anomalies, and the postoperative occurrence of cholangitis. The authors note that the most promising screening strategies in infants are early measurements of serum conjugated bilirubin and a stool color card given to new parents that alerts them and their primary care providers to acholic stools. 3 figures. 4 tables. 121 references.

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Terlipressin For The Treatment of Hepatorenal Syndrome. Gastroenterology and Hepatology. 3(10): 773-774. October 2007.

This article describes the use of terlipressin for the treatment of hepatorenal syndrome, a complication of the portal hypertension that occurs in patients with cirrhosis. The author answers common questions about hepatorenal syndrome and the use of terlipressin, including the rationale for the use of this drug, research studies on the use of terlipressin for hepatorenal syndrome, the difficulties of conducting clinical research on patients with a disease that has a rapid clinical course and a high mortality rate, new directions for future research, the use of terlipressin in patients undergoing liver transplantation, and how future monitoring of patients with cirrhosis could be improved to optimize terlipressin therapy. Terlipressin is an analog of the vasoconstrictor vasopressin, which is used to treat bleeding esophageal varices, another complication of portal hypertension. Terlipressin, a prodrug of vasopressin, is a much safer drug than vasopressin, with fewer side effects. Terlipressin is often given in combination with albumin, which expands the blood volume, resulting in improved renal function in patients with hepatorenal syndrome. 5 references.

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Autoimmune Hepatitis. IN: Lichtenstein, G.; Reddy, K.R.; Faust, T., eds. Clinician’s Guide to Liver Disease. Thorofare, NJ: Slack Incorporated. 2006. pp 105-120.

This chapter about autoimmune hepatitis is from a user-friendly reference book that provides gastroenterologists with an overview of the management of acute and chronic liver disease. The author describes autoimmune hepatitis as a condition of unresolving liver inflammation, which primarily affects young women. The condition is characterized by elevated liver enzymes, hypergammaglobulinemia, serum autoantibodies, interface hepatitis, plasma cell infiltrates on liver biopsy, extrahepatic manifestations, and steroid responsiveness. The chapter provides a brief historical perspective and discussion of epidemiology. It discusses pathogenesis, natural history and prognosis, clinical features, diagnosis and diagnostic tests, complications, and treatment options including medial therapy and liver transplantation. The author cautions that diagnosis of autoimmune hepatitis requires a high index of clinical suspicion because several liver conditions can mimic the disease. Most patients respond to immunosuppressive medications with improvement in clinical symptoms, biochemical liver tests, hepatic histology, and patient survival; however, relapse is common. Up to 40 percent of patients progress to cirrhosis despite therapy. The chapter includes tables and a patient care algorithm and concludes with a list of references. 1 figure. 6 tables. 19 references.

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Benign Lesions of the Liver. Gastroenterology and Hepatology. 2(5): 325-326. May 2006.

This article describes benign lesions of the liver and patient care strategies for people who have these tumors. Benign lesions of the liver are generally of three types: hemangioma, focal nodular hyperplasia (FNH), and hepatic adenoma. Hemangiomas contain fibrous tissue and small blood vessels than can range in size from less than 1 centimeter in diameter to over 10 centimeters. FNH lesions are also solid with a typical central scar and nodule formation. Hepatic adenoma is an infrequently encountered, typically solitary lesion which carries a small risk of spontaneous rupture or malignancy. The author discusses indications for screening patients for these lesions, the interplay between liver lesions and oral contraceptive (OC) use, presenting symptoms that should prompt screening for these lesions, the lack of medical therapies for these lesions, surgical treatment (i.e., operative resection), the importance of differentiating between benign lesions and those that are potentially cancerous, the use of liver biopsy, and liver transplantation in patients with benign lesions (usually reserved only for those patients with other systemic diseases that also present with multiple hepatic adenomas). 5 references.

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Biliary Atresia. Bethesda, MD: National Digestive Diseases Information Clearinghouse. 2006. 6 p.

This fact sheet reviews the problem of biliary atresia, a serious but rare disease of the liver that affects newborn infants. Biliary atresia is defined as the loss or lack of the bile ducts that drain bile from the liver. Bile is made by the liver and passes through the bile ducts and into the intestines where it helps to digest food, fats, and cholesterol. The fact sheet reviews the symptoms, causes, diagnosis, surgical treatments, and postoperative care of biliary atresia. Diagnostic tests that may be used to confirm the condition include physical exam, ultrasound of the abdomen and liver, liver scans, and liver biopsy. The usual surgical correction for biliary atresia is the Kasai procedure (hepato-porto-enterostomy), in which a loop of intestine is brought up to replace the bile ducts and drain the liver. The benefits of this surgery make the early diagnosis of biliary atresia very important, preferably before the infant is several months old and has suffered permanent liver damage. Liver transplantation may be required in infants for whom the Kasai procedure is not effective or successful. The fact sheet concludes with a sidebar about current research studies on biliary atresia, a brief list of resource organizations for readers wanting additional information, and a description of the activities of the National Digestive Diseases Information Clearinghouse. 1 figure.

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Biliary Complications of Liver Transplantation. IN: Clavien, P.; Baillie, J., eds. Diseases of the Gallbladder and Bile Ducts: Diagnosis and Treatment. 2nd ed. Williston, VT: Blackwell Publishing Inc. 2006. pp 289-305.

This chapter on biliary complications of liver transplantation is from a textbook that provides a comprehensive and critical approach to both established and new diagnostic and therapeutic modalities for diseases of the gallbladder and bile ducts. The authors caution that biliary complications as well as the therapeutic interventions that follow may lead to a decreased quality of life. Biliary tract complications occur in 11 to 31 percent of liver transplantations. They describe the types of biliary reconstruction used in liver transplantation, the advantages and disadvantages associated with each type of reconstruction, the most common post-transplantation biliary complications and their causes, the techniques used to evaluate and treat biliary complications, and the role of biliary complications in living donor liver transplantation. The chapter includes a summary of objectives, a list of suggested readings, extensive references, and a set of self-test questions that focus on the material covered in the chapter. 11 figures. 3 tables. 61 references.

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Clinician’s Guide to Liver Disease. Thorofare, NJ: Slack Incorporated. 2006. 356 p.

This user-friendly reference book provides gastroenterologists with an overview of the management of acute and chronic liver disease. The book offers 16 chapters: evaluation of the liver patient, cirrhosis and its complications, acute and chronic viral hepatitis, primary biliary cirrhosis and primary sclerosing cholangitis, autoimmune hepatitis, nonalcoholic fatty liver disease, metabolic liver disease, vascular diseases involving the liver, benign and malignant tumors of the liver, liver disease in pregnancy, postoperative jaundice, nonviral infections of the liver, hepatopulmonary syndrome, portopulmonary hypertension, liver transplantation, and drug hepatotoxicity. Each chapter presents an introduction, consideration of etiology, epidemiology, clinical presentation and symptoms, risk factors, complications, and treatment approaches. The chapters include charts and figures and conclude with a list of references. Most chapters include patient care algorithms. A subject index concludes the volume.

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Diseases of the Gallbladder and Bile Ducts: Diagnosis and Treatment. 2nd ed. Williston, VT: Blackwell Publishing Inc. 2006. 428 p.

This textbook provides a comprehensive and critical approach to both established and new diagnostic and therapeutic modalities for diseases of the gallbladder and bile ducts. The book was written by a multidisciplinary panel of international experts with extensive experience in this population of patients. The book offers 23 chapters in six sections: anatomy, pathophysiology, and epidemiology of the biliary system; diagnostic and therapeutic approaches for the biliary tree and gallbladder; specific conditions; the intrahepatic and extrahepatic bile ducts; intrahepatic cholestasis; and the pediatric population. Specific topics include noninvasive imaging, endoscopic diagnosis and treatment, percutaneous biliary imaging and intervention, radiation therapy, surgery, laparoscopic treatment, laparoscopic biliary injuries, treatment for biliary malignancies, the gallbladder, gallstones, acute cholangitis, cystic diseases of the biliary system, biliary complications of liver transplantation, primary sclerosing cholangitis, cholangiocarcinoma, primary biliary cirrhosis, and biliary disease in infants and children. Each chapter includes a summary of objectives, a list of suggested readings, extensive references, and a set of self-test questions that focus on the material covered in the chapter. The book is illustrated with black-and-white photographs and line drawings; one section of color plates is included. The book concludes with the answers to the self-test study questions and a detailed subject index.

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End-Stage Liver Disease. IN: Nilsson, K.R.; Piccini, J.P., eds. Osler Medical Handbook. Philadelphia, PA: Saunders. 2006. pp. 402-417.

Chronic liver disease includes a broad differential diagnosis of infectious, autoimmune, inherited, metabolic, toxic, and acquired origins. This chapter on end-stage liver disease is from a handbook that provides the essentials of diagnosis and treatment, as well as the latest in evidence-based medicine, for residents working bedside, in-patient care. The chapter begins with a presentation of essential Fast Facts and concludes with Pearls and Pitfalls useful to the practicing internist. The body of the chapter is divided into sections: Epidemiology, Clinical Presentation, Diagnosis, and Management. Specific topics covered include the most common causes of cirrhosis in the United States, notably alcoholic liver disease and chronic hepatitis C infection; nonalcoholic fatty liver disease (NAFLD); the causes of the clinical problems associated with cirrhosis; the risk factors for hepatic encephalopathy, including infection, dehydration, hypokalemia, alkalosis, and sedating medications; the risk of liver cancer in patients with cirrhosis; autoimmune hepatitis; the diagnostic tests used to confirm metabolic and inherited causes of chronic liver disease; and the use of the Model for End-Stage Liver Disease (MELD) score to predict mortality, particularly for those patients waiting for a liver transplantation. The chapter concludes with a list of references, each labeled with a 'strength of evidence' grade to help readers determine the type of research available in that reference source. 1 figure. 2 tables. 35 references.

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Hepatopulmonary Syndrome. IN: Lichtenstein, G.; Reddy, K.R.; Faust, T., eds. Clinician’s Guide to Liver Disease. Thorofare, NJ: Slack Incorporated. 2006. pp 271-282.

This chapter about hepatopulmonary syndrome (HPS) is from a user-friendly reference book that provides gastroenterologists with an overview of the management of acute and chronic liver disease. The authors define HPS as a clinical triad of liver disease, hypoxemia, and intrapulmonary vasodilation. The two types of HPS are differentiated by the presence or absence of significant arteriovenous malformations. The characteristic clinical presentation, consisting of dyspnea, orthodeoxia, cyanosis, and clubbing, is usually present. Only a few nonsurgical treatments have been successful in small studies. Liver transplantation is the mainstay of therapy for HPS and usually results in complete resolution of intrapulmonary shunting. The authors focus on the epidemiology, pathophysiology, diagnosis, and treatment of HPS. A diagnostic algorithm is included. 1 figure. 2 tables. 48 references.

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Liver Disease in Patients with Diabetes Mellitus. Journal of Clinical Gastroenterology. 40(1): 68-76. January 2006.

In addition to the well-known cardiovascular, renal, and ophthalmologic complications of diabetes, liver-related complications occur commonly and are often underrecognized. This article reviews the relationship between diabetes mellitus and two common liver diseases: chronic hepatitis C and nonalcoholic fatty liver disease. The author also discusses the association of diabetes and cirrhosis, acute liver failure, hepatocellular carcinoma, and outcomes following orthotopic liver transplantation. The liver plays a significant role in energy homeostasis and glucose metabolism; insulin enhances glycogen synthesis within the liver and prevents glucose production. These normal physiologic processes become dysregulated with insulin resistance and type 2 diabetes mellitus. Insulin resistance may work synergistically with hepatitis C infection to make changes in the liver, in the form of steatosis, inflammation, and fibrosis development. Once this occurs, progression to diabetes may occur in patients with underlying genetic susceptibility. Current treatment for preventing liver complications is focused on therapies that improve underlying insulin resistance, including weight loss or drug therapy. 1 figure. 2 tables. 158 references.

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Liver Transplantation. IN: Lichtenstein, G.; Reddy, K.R.; Faust, T., eds. Clinician’s Guide to Liver Disease. Thorofare, NJ: Slack Incorporated. 2006. pp 295-320.

This chapter about liver transplantation is from a user-friendly reference book that provides gastroenterologists with an overview of the management of acute and chronic liver disease. The authors introduce the chapter by noting that a significant predictor of post-orthotopic liver transplantation (OLT) survival is the severity of hepatocellular dysfunction and associated debility at the time of surgery. The medical management of OLT has evolved since the early 1980s, with the goals shifting from prevention of allograft rejection to prevention and treatment of recurrent disease. Recurrence of hepatitis C virus (HCV) is currently a major challenge in this field because HCV is the most common indication for OLT and graft reinfection is universal with a lack of effective prevention strategies. The chapter covers the pretransplant evaluation, the listing criteria and policies of the United Network for Organ Sharing (UNOS), surgical techniques, immunosuppression, posttransplant management, and long-term management issues. 4 figures. 13 tables. 16 references.

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Management of the Cirrhotic Patient Before Liver Transplantation: The Role of the Referring Gastroenterologist. Gastroenterology and Hepatology. 2(5): 346-354. May 2006.

The referral of a patient with cirrhosis to a liver transplant center for evaluation is usually made by a local gastroenterologist. This article reviews the role of the referring gastroenterologist in the ongoing management of the patient with liver cirrhosis before liver transplantation. The role of the referring gastroenterologist begins with early identification and modification of high-risk behaviors, which may delay listing a patient for liver transplantation. The gastroenterologist must also understand what constitutes a timely referral of a patient to a liver transplant center and the consequences of late referral. Although patients experience the inevitable deterioration of their liver function, which in turn advances their priority on the liver transplant waiting list, the referring gastroenterologist must also anticipate medical complications of cirrhosis, and should initiate appropriate surveillance and prevention programs to detect and prevent these complications. Another important role of the referring gastroenterologist is as a link of information between the patient and the transplant center. The author concludes that adherence to these guidelines will increase the probability that a patient with chronic liver failure will undergo successful liver transplantation and will decrease post-transplant morbidity. 1 figure. 5 tables. 75 references.

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Medical Care of the Patient with Compensated Cirrhosis. Gastroenterology and Hepatology. 2(2): 124-133. February 2006.

This article reviews the surveillance and health maintenance recommendations that should be followed for every patient with compensated cirrhosis to delay or prevent the serious complications of cirrhosis. The authors note that although most gastroenterologists and hepatologists are comfortable managing the serious complications related to cirrhosis, many fail to provide the necessary education, prevention, and treatment for non-life-threatening problems associated with cirrhosis. Topics covered include the prognosis of compensated cirrhosis, patient education, alcohol use, acetaminophen use, Vibrio vulnificus infections, vitamin and mineral supplements, weight control, immunizations, dental hygiene, screening for osteoporosis, the primary prophylaxis of variceal hemorrhage, surveillance for hepatocellular carcinoma (liver cancer), medication use in patients with cirrhosis, screening and managing nonliver diseases in patients with cirrhosis, and when to refer for liver transplantation. The authors conclude that patient education, appropriate surveillance and preventive strategies, and regular monitoring of the patient’s condition can improve quality of life and can delay or prevent many of the serious complications associated with cirrhosis. 4 tables. 97 references.

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Natural History of Nonalcoholic Steatohepatitis. Journal of Clinical Gastroenterology. 40(Suppl 1): S11-S16. March 2006.

This review article covers the natural history of nonalcoholic steatohepatitis, a variation of nonalcoholic fatty liver disease (NAFLD). NAFLD is being increasingly recognized as one of the most common chronic liver diseases. The authors note that the natural history of this liver disease remains unclear due to its indolent nature, the paucity of prospective studies, and the lack of consensus regarding the various forms of this disorder. Patients with nonalcoholic steatohepatitis (NASH) appear to have a higher likelihood of progression to cirrhosis. Obesity and possibly type 2 diabetes appear to be associated with an increased risk of fibrosis. There is also an increased risk of liver cancer and end-stage liver disease among patients with NASH-related cirrhosis and those with cryptogenic cirrhosis, which likely represents a late stage of NAFLD. The authors also briefly review the recurrence of NASH in patients who have undergone liver transplantation for NASH-related end-stage liver disease. They conclude that long-term survival in patients with NAFLD may be slightly worse than survival from all-cause chronic liver disease, although survival appears to be higher than among patients with fatty liver from alcohol abuse, and is comparable to patients with chronic hepatitis C infection. 6 figures. 19 references.

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Portopulmonary Hypertension. IN: Lichtenstein, G.; Reddy, K.R.; Faust, T., eds. Clinician’s Guide to Liver Disease. Thorofare, NJ: Slack Incorporated. 2006. pp 283-294.

This chapter about portopulmonary hypertension (PPHTN) is from a user-friendly reference book that provides gastroenterologists with an overview of the management of acute and chronic liver disease. The authors define PPHTN as a pulmonary vascular disorder in patients with portal hypertension. The clinical presentation and consequences are highly variable, ranging from mild to severe pulmonary hypertension, the latter associated with a high mortality rate, with or without liver transplantation. The authors note that treatment with intravenous agents that cause pulmonary vasodilation or vascular remodeling has made it possible for some patients with significant PPHTN to successfully undergo orthotopic liver transplantation (OLT). The authors focus on the clinical features, diagnosis, treatment, and prognosis of PPHTN. A diagnostic algorithm is included. 1 figure. 1 table. 63 references.

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Primary Biliary Cirrhosis and Primary Sclerosing Cholangitis. IN: Lichtenstein, G.; Reddy, K.R.; Faust, T., eds. Clinician’s Guide to Liver Disease. Thorofare, NJ: Slack Incorporated. 2006. pp 87-104.

This chapter about primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) is from a user-friendly reference book that provides gastroenterologists with an overview of the management of acute and chronic liver disease. The authors define PBC as a chronic cholestatic, autoimmune liver disease that predominantly affects middle-aged women. Over time, fibrosis, cirrhosis, and complications of portal hypertension and cholestasis may develop in patients with PBC. Ursodeoxycholic acid has been shown to improve cholestatic liver-associated enzymes and to improve transplant-free survival, although the effect is not certain. Liver transplantation is the treatment of choice for patients with advanced PBC. Primary sclerosing cholangitis is a chronic cholestatic liver disease that primary affects young to middle-aged men, who typically have concurrent inflammatory bowel disease (IBD). PSC patients are at risk for bacterial cholangitis, cholangiocarcinoma, and complications of liver failure and cholestasis. Although there is no proven therapy specific for PSC, medical therapies should be directed toward managing the complications of portal hypertension and progressive cholestasis. Liver transplantation is the only option that has been clearly shown to improve patient survival. 5 tables. 20 references.

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Primary Biliary Cirrhosis. IN: Clavien, P.; Baillie, J., eds. Diseases of the Gallbladder and Bile Ducts: Diagnosis and Treatment. 2nd ed. Williston, VT: Blackwell Publishing Inc. 2006. pp 341-352.

This chapter on primary biliary cirrhosis (PBC) is from a textbook that provides a comprehensive and critical approach to both established and new diagnostic and therapeutic modalities for diseases of the gallbladder and bile ducts. The authors begin with a discussion of nomenclature, noting that health care providers must communicate clearly to patients the discrepancy between the name PBC and a patient’s actual clinical status. PBC is characterized by destruction of the interlobular bile ducts with duct invasion by chronic inflammatory cells and apoptosis of biliary epithelial cells, leading to progressive bile duct loss, fibrosis, and eventual cirrhosis. The authors describe the multifactorial etiology of PBC, the role of genetic factors in its development, the importance of early diagnosis even in asymptomatic patients, and the management of PBC and its complications. They note that no curative medical therapy for PBC exists; however, medications are available that slow down the progression of the disease and can relieve symptoms in the majority of patients. Some patients with PBC will develop end-stage liver failure and require liver transplantation. The chapter includes a summary of objectives, a list of suggested readings, extensive references, and a set of self-test questions that focus on the material covered in the chapter. 2 figures. 4 tables. 63 references.

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Evaluation of the Patient for Liver Transplantation. Hepatology. 41(6): 1-26. June 2005.

Liver transplantation is the most effective treatment for many patients with acute or chronic liver failure resulting from a variety of causes. This article presents the practice guideline from the American Association for the Study of Liver Diseases (AASLD) on the evaluation of patients for liver transplantation. These recommendations are based on a formal review and analysis of the published literature on the topic; several consensus conferences among experts; the American College of Physicians' Manual for Assessing Health Practices and Designing Practice Guidelines; guideline policies produced by professional organizations, including the AASLD and the American Gastroenterological Association; and the authors’ experience in the specified topic. Topics include the indications of liver transplantation, when evaluation for transplantation should be considered, determining the need for liver transplantation, and recipient evaluation at the transplant center. The article offers recommendations for patients with the hepatopulmonary syndrome, portopulmonary hypertension, obesity, cigarette smoking, kidney failure, extrahepatic malignancies, osteoporosis, HIV infection, surgical contraindications, and psychosocial problems. The authors discuss specific indications for liver transplantation, including chronic noncholestatic liver disorders, chronic hepatitis C, chronic hepatitis B, autoimmune hepatitis, alcoholic cirrhosis, cholestatic liver disorders, primary biliary cirrhosis, primary sclerosing cholangitis, childhood cholestatic diseases, metabolic diseases, alpha-1-antitrypsin disease, Wilson disease, nonalcoholic steatohepatitis and cryptogenic cirrhosis, hereditary hemochromatosis, neonatal hemochromatosis, tyrosinemia and glycogen storage disease, metabolic diseases with severe extrahepatic manifestations, amyloidosis and hyperoxaluria, urea cycle and branched-chain amino acid disorders, hepatic malignancies, hepatocellular carcinoma, hepatoblastoma, fibrolamellar hepatocellular carcinoma and hemangioendothelioma, cholangiocarcinoma, and fulminant hepatic failure. The article includes 76 specific recommendations for the evaluation of the patient for liver transplantation. 3 tables. 328 references.

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Gastrointestinal Conditions. Journal of the American College of Surgeons. 201(5): 940- 947. December 2005.

This article reports on new developments in surgery for gastrointestinal conditions, emphasizing new approaches to common diseases, and highlighting several common gastrointestinal diseases in which there is an emerging consensus. The author reviewed the major surgery journals for selection of articles about major advances in this area. Topics include hernia, small bowel obstruction, nasogastric decompression, bariatric surgery, gastroesophageal reflux disease, peptic ulcer disease, gastric cancer, hepatic (liver) resection, liver transplantation, portal hypertension, laparoscopic bile duct injuries, chronic pancreatitis, acute pancreatitis, hemorrhoids, preoperative bowel preparation, acute diverticulitis, and laparoscopic colectomy. In each area, the author briefly summarizes the main research studies of the past year. 44 references.

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Liver Transplantation: An Overview, A Guide. Birmingham, England: Children's Liver Disease Foundation. 2005. 10 p.

This brochure helps parents and caregivers of children who are undergoing liver transplantation to understand the surgery and postoperative care. The author stresses that recent technical advances, which include improved surgical techniques, more effective anti-rejection (immunosuppressive) medications, and greater understanding of the postoperative care requirements have improved overall survival rates. Topics include the reasons for transplantation, timing the transplantation surgery based on the child’s age, preoperative assessment, coping with the waiting period, the different types of liver transplantation available, hospital care and the immediate postoperative period, and possible complications, including biliary problems, bowel perforation, and infection. An additional section reminds parents of the adjustment issues they may face when the child comes home from the hospital. The brochure also explains the work of the England-based Children’s Liver Disease Foundation, an organization started in 1980 by a group of parents who wanted to support research for children’s liver diseases and to help families coping with liver disease. A form with which readers can donate to the Children’s Liver Disease Foundation is provided. A blank page is also provided for notes; another blank form is included to record healthcare providers’ contact information.

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Management of Hepatocellular Carcinoma. Hepatology. 42(5): 1208-1236. November 2005.

This article presents the practice guideline from the American Association for the Study of Liver Diseases (AASLD) on the diagnosis, staging, and treatment of patients with hepatocellular carcinoma (HCC). These recommendations are based on a formal review and analysis of the published literature on the topic; several consensus conferences among experts; the American College of Physicians' Manual for Assessing Health Practices and Designing Practice Guidelines; guideline policies produced by professional organizations, including the AASLD and the American Gastroenterological Association; and the authors’ experience in the specified topic. Topics include surveillance for hepatocellular carcinoma, definition of the at-risk population, predictive factors for HCC, recommended surveillance intervals, the diagnosis of HCC, staging systems, treatment options, surgical resection, the risk of recurrence, liver transplantation, living donor transplantation, posttransplantation management, and future perspectives. The article includes 21 specific recommendations for the evaluation of the patient for liver transplantation. 3 figures. 4 tables. 322 references.

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Primary Biliary Cirrhosis. New England Journal of Medicine. 353(12): 1261-1273. September 22, 2005.

This article reviews advances in primary biliary cirrhosis (PBC), a slowly progressive, autoimmune disease of the liver that primarily affects women. The authors review the typical clinical findings in PBC, pathological findings, natural history and prognosis, causes, environmental factors, autoimmune responses, treatment of symptoms and complications, treatment of underlying disease, and ideas for future research. Common complications of PBC include pruritus, osteoporosis, hyperlipidemia, and portal hypertension. Treatment strategies reviewed include ursodeoxycholic acid (UDCA), colchicines and methotrexate, other drug therapies, and orthotopic liver transplantation. 4 figures. 2 tables. 98 references.

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Primary Sclerosing Cholangitis. IN: Clavien, P.; Baillie, J., eds. Diseases of the Gallbladder and Bile Ducts: Diagnosis and Treatment. 2nd ed. Williston, VT: Blackwell Publishing Inc. 2006. pp 306-331.

This chapter on primary sclerosing cholangitis (PSC) is from a textbook that provides a comprehensive and critical approach to both established and new diagnostic and therapeutic modalities for diseases of the gallbladder and bile ducts. PSC is characterized by inflammation, destruction, and eventual fibrosis of intrahepatic and extrahepatic bile ducts. The authors discuss classification, the diagnostic tests used to confirm and stage PSC, the possible etiologies of PSC, clinical manifestations, natural history of PSC, drug therapy, management of the complications of PSC, endoscopic management of PSC, cholangiocarcinoma, and liver transplantation. The authors conclude that PSC is a chronic disorder, often of unknown etiology, and is commonly associated with inflammatory bowel disease (IBD). The disease presents with abdominal pain, elevated liver enzymes, jaundice, and cholangitis. Ultimately, most patients with PSC will require liver transplantation. The chapter includes a summary of objectives, a list of suggested readings, extensive references, and a set of self-test questions that focus on the material covered in the chapter. 6 figures. 10 tables. 254 references.

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Diagnosis and Current Therapy of Wilson's Disease. Alimentary Pharmacology and Therapeutics. 19(2): 157-165. January 2004.

Wilson's disease is an autosomal recessive inherited disorder of liver (hepatic) copper metabolism resulting in liver disease and or neuropsychiatric disease. This article reviews the diagnosis and current therapeutic recommendations for Wilson's disease. The diagnosis of neurological disease is straightforward if the following symptoms are present: Kayser-Fleischer rings, typical neurological symptoms, and low serum ceruloplasmin levels. The diagnosis is more complex in patients presenting with liver diseases. None of the commonly used parameters alone allows a diagnosis with certainty. A combination of various laboratory parameters is needed to firmly establish the diagnosis. Recently, a group of international experts has proposed a score based on a variety of tests and clinical symptoms. The validity of this score needs to be assessed prospectively. Treatment of Wilson's disease requires life-long administration of copper chelators (D-penicillamine, trientine, zinc). None of these treatments has been tested by prospective randomized controlled studies. Liver transplantation is reserved for severe or treatment-resistant cases with advanced liver disease, whilst experience with refractory neuropsychiatric disease is limited. 3 figures. 1 table. 57 references.

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Action Plan for Liver Disease Research: A Report of the Liver Disease Subcommittee of the Digestive Diseases Interagency Coordinating Committee. Bethesda, MD: National Digestive Diseases Information Clearinghouse. 2004. 196 p.

This Action Plan for Liver Disease Research was developed to advance research on liver and biliary diseases. The Action Plan was undertaken to identify areas of scientific opportunity to help direct research resources at the National Institutes of Health (NIH) toward practical goals in the prevention, diagnosis, and management of liver and biliary diseases. After introductory sections on cell and molecular biology of the liver as well as liver physiology and pathology, this Action Plan Report reviews the different types of liver disease, including viral hepatitis, HIV and liver disease, fatty liver disease, drug- and toxicant-induced liver disease, autoimmune liver disease, pediatric liver disease, genetic liver disease, liver transplantation, complications of liver disease, liver cancer, gallbladder and biliary disease, and liver imaging and biotechnology. Each of these chapters includes sections on current understanding of the topic, recent research advances, research goals, and steps to achieve those research goals. The document identifies 214 research goals in liver and biliary research and ten representative benchmarks that will be used to gauge progress toward meeting the overall aims of the Action Plan. The document concludes with three appendices: a list of Action Plan participants, a list of references for the Epidemiologic data, and a list of acronyms; a subject index is also provided. The document includes two-color charts and illustrations.

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Complications of Liver Disease. IN: U.S. Department of Health and Human Services. Action Plan for Liver Disease Research. Bethesda, MD: National Digestive Diseases Information Clearinghouse. 2004. pp. 129-136.

Chronic liver disease and cirrhosis account for approximately 27,000 deaths in the United States each year. The majority of patients who die of cirrhosis succumb ultimately to a complication of portal hypertension, such as variceal hemorrhage, ascites, spontaneous bacterial peritonitis, hepatic encephalopathy, hepatopulmonary syndrome, or hepatorenal syndrome. These complications are also the most common proximal causes of death in patients awaiting liver transplantation. This chapter on the complications of liver disease is from the Action Plan for Liver Disease Research that was developed to advance research on liver and biliary diseases. The Action Plan was undertaken to identify areas of scientific opportunity to help direct research resources at the National Institutes of Health (NIH) toward practical goals in the prevention, diagnosis, and management of liver and biliary diseases. In this chapter, the authors first review the types of complications that patients with chronic liver disease encounter, including portal hypertension, ascites, esophageal varices, and kidney and pulmonary complications. Other complications of liver disease include symptoms such as fatigue, weakness, jaundice, and pruritus (itching). The chapter then outlines recent research advances in the areas of portal hypertension, the complications of portal hypertension, and the complications of cirrhosis. The authors provide specific research goals in these same areas, also focusing on patient care management and the prevention and therapy of acute liver failure. A final section considers the steps that would assist in achieving these research goals. One chart summarizes the short (0 to 3 years), intermediate (4 to 6 years), and long-term (7 to 10 years) goals of research on these topics. 1 figure. 1 table.

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FYI: Focus on Urea Cycle Disorders. C.L.A.S.S. Notes. Issue 3: 1. 2004.

The normal urea cycle in the human body involves a series of biochemical steps taking place in the liver in which ammonia (waste nitrogen), generated from the breakdown of dietary protein, is converted into a less toxic form and removed from the blood stream. This brief article outlines urea cycle disorders (UCDs), genetic disorders caused by a deficiency of one of the six enzymes in the urea cycle. In UCDs, the highly toxic ammonia remains in the blood and reaches the brain, where it causes irreversible brain damage and death. UCDs are considered part of the category of inborn errors of metabolism. The author reviews the incidence of UCDs, common misdiagnoses for the condition, the six disorders of the urea cycle, symptoms of severe and mild UCDs, the causes of childhood episodes of high ammonia levels, adults with mild urea cycle enzyme deficiency, treatment options (including dietary protein restriction and use of medications that remove ammonia from the blood), the use of liver transplantation in severe cases, and the role of neonatal screening programs. Readers are referred to the National Urea Cycle Disorders Foundation (800-38-NUCDF or www.nucdf.org) for more information.

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Budd-Chiari Syndrome. In: PDxMD. PDxMD Gastroenterology. St. Louis, MO: Elsevier Science. 2003. p. 15-41.

Budd-Chiari syndrome is characterized by an impediment of venous blood flow from the liver, caused by thrombosis (clotting), external compression, or venous malformation. The consequences of this syndrome include portal hypertension, cirrhosis (scarring of the liver), and liver failure. Treatment includes thrombolytic therapy, re-establishing venous flow by shunting or stenting, and in fulminant cases, orthotopic liver transplantation. This chapter on Budd-Chiari syndrome is from a book on gastroenterology that offers concise, action-oriented recommendations for primary care medicine. The chapter covers summary information and background on the condition, and comprehensive information on diagnosis, treatment, outcomes, and prevention. Specific topics covered include the ICD9 code, urgent action, synonyms, cardinal features, causes (etiology), epidemiology, differential diagnosis, signs and symptoms, associated disorders, investigation of the patient, appropriate referrals and consultations, diagnostic considerations, clinical tips, treatment options, patient management issues, drug therapies, prognosis, complications, and how to prevent recurrence. The information is provided in outline and bulleted format for ease of accessibility. The final section of the chapter offers resources, including related associations, key references, and the answers to frequently asked questions (FAQs). 5 references.

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Liver Transplantation. IN: U.S. Department of Health and Human Services. Action Plan for Liver Disease Research. Bethesda, MD: National Digestive Diseases Information Clearinghouse. 2004. pp. 119-128.

Liver transplantation is now considered the standard of care for patients with end-stage liver disease or acute liver failure. Orthotopic liver transplantation involves removal of the diseased organ and replacement with a liver from a deceased donor. In some situations, particularly in pediatric liver transplantation, a portion of the donor liver is used or a single liver is shared between two recipients; partial liver grafts can also be used from a living donor. This chapter on liver transplantation is from the Action Plan for Liver Disease Research that was developed to advance research on liver and biliary diseases. The Action Plan was undertaken to identify areas of scientific opportunity to help direct research resources at the National Institutes of Health (NIH) toward practical goals in the prevention, diagnosis, and management of liver and biliary diseases. In this chapter, the authors first review the indications, success rate, and supply and demand situation of liver transplantation, then outline recent research advances in the areas of immunosuppression, patient care management, improved surgical techniques, and living donor liver transplantation. The authors provide specific research goals in the areas of basic research on allograft tolerance and rejection, immunosuppression, optimizing liver regeneration, clinical investigation and monitoring, and drug therapy (immunosuppressive agents). A final section considers the steps that would assist in achieving these research goals. One chart summarizes the short (0 to 3 years), intermediate (4 to 6 years), and long-term (7 to 10 years) goals of research on these topics. 1 figure. 1 table.

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Mayo Clinic Gastrointestinal Surgery. St. Louis, MO: Elsevier Science. 2004. 1020 p.

This book focuses on the major diseases treated by gastrointestinal surgeons, from the esophagus to the anal canal. The presentation has a definite clinical orientation and a major emphasis on practical applications as they are applied at the Mayo Clinic. Sections on etiology, pathophysiology, pathology, and diagnosis are also included by are purposely not the emphasis of the chapters. The book offers 49 chapters: the experience of being a Mayo Clinic surgeon; gastroesophageal reflux disease (GERD) and esophageal hiatal hernia; achalasia and other esophageal motility disorders; epiphrenic esophageal diverticula; cancer of the esophagus; gastric adenocarcinoma, primary gastric lymphoma; peptic ulcer; disorders of gastrointestinal motility and emptying after gastric operations; morbid obesity; hepatocellular carcinoma and intrahepatic cholangiocarcinoma; hepatic metastases from extrahepatic cancers; benign tumors and cysts of the liver; liver diseases necessitating liver transplantation; biliary stone disease; benign biliary strictures; cancer of the gallbladder; pancreatic and periampullary carcinoma; islet cell tumors; acute and chronic pancreatitis; pancreas transplantation after complications of diabetes mellitus; cystic tumors of the pancreas; thrombocytopenia and other hematologic disorders; malignant tumors of the small intestine; villous tumors of the duodenum; small intestinal diverticula; Crohn's disease; small bowel obstruction; acute mesenteric ischemia; acute mesenteric venous thrombosis; chronic mesenteric ischemia; visceral artery aneurysms; colonic motor disorders (constipation); diverticular disease of the colon; colon cancer; ischemic colitis; appendicitis; chronic ulcerative colitis; colonic volvulus; familial adenomatous polyposis; cancer of the rectum; common anorectal problems; rectal prolapse and solitary rectal ulcer syndrome; abdominal trauma; unclosable abdomen and the dehisced wound; ventral and incisional hernias; open repair of inguinal hernia; endoscopic inguinal hernia repair; and common pediatric gastrointestinal disorders. Each chapter is illustrated with line drawings, black and white photographs, and some color plates. References are provided with each chapter and a detailed subject index concludes the text.

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Approach to the Patient with Ascites. In: Textbook of Gastroenterology. 4th ed. [2-volume set]. Hagerstown, MD: Lippincott Williams and Wilkins. 2003. p. 948-972.

Ascites refers to the condition of pathological fluid accumulation within the abdominal cavity. This chapter on the approach to patients with ascites is from a lengthy, two-volume textbook that integrates the various demands of science, technology, expanding information, good judgment, and common sense into the diagnosis and management of gastrointestinal patients. Topics include the causes of ascites, mechanisms of ascites formation, evaluation of the patient with ascites, complications of ascites, and treatment options. The development of ascites in a patient who has cirrhosis (scarring of the liver) and who is otherwise a good candidate for liver transplantation should lead to evaluation for transplantation. Patients should be rapidly prioritized for liver transplantation once they develop refractory (not responsive to treatment) ascites. Diet education and diuretics are the mainstays of treatment for patients who await transplantation and for those who are not candidates for the procedure. TIPS (transjugular intrahepatic portosystemic stent shunt) is a promising option for treatment of diuretic-resistant ascites, but its final place in the treatment armamentarium remains to be seen. 7 figures. 6 tables. 165 references.

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Approach to the Patient with Fulminant (Acute) Liver Failure. In: Textbook of Gastroenterology. 4th ed. [2-volume set]. Hagerstown, MD: Lippincott Williams and Wilkins. 2003. p. 983-991.

Fulminant (acute) liver failure (FLF) is a relatively uncommon condition characterized by hepatocyte necrosis (liver tissue death) and disruption of liver function in proportion to the degree of liver cell death. With extensive liver cell death comes the development of clinically obvious hepatic encephalopathy, which defines the clinical condition of FLF. This chapter on the approach to patients with FLF is from a lengthy, two-volume textbook that integrates the various demands of science, technology, expanding information, good judgment, and common sense into the diagnosis and management of gastrointestinal patients. The chapter first discusses the definition, pathogenesis, and etiology of FLF. Thereafter, the authors discuss the clinical management of these patients, divided into pre-intensive care unit (ICU) and ICU management of patients with FLF. Subsequently, liver transplantation and developments in transplantation are discussed, as well as the available bioreactors for use in patients with FLF. Supportive therapy allows both functional and structural regeneration of the liver after FLF, without the development of chronic liver disease. However, in certain cases, the prognosis is grave and is greatly improved by emergency liver transplantation. 2 tables. 58 references.

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Pediatric Liver Disease. IN: U.S. Department of Health and Human Services. Action Plan for Liver Disease Research. Bethesda, MD: National Digestive Diseases Information Clearinghouse. 2004. pp. 101-109.

Pediatric liver diseases include biliary atresia, metabolic disorders, intrahepatic cholestatic disorders, alpha-1 antitrypsin deficiency liver disease, nonalcoholic steatohepatitis (NASH), autoimmune hepatitis, sclerosing cholangitis, parenteral nutrition-induced liver injury, drug-induced liver injury, Wilson disease, cystic fibrosis, and various forms of viral hepatitis. Although liver disease is uncommon in children, those liver diseases that occur tend to be severe and progressive. This chapter on pediatric liver disease is from the Action Plan for Liver Disease Research that was developed to advance research on liver and biliary diseases. The Action Plan was undertaken to identify areas of scientific opportunity to help direct research resources at the National Institutes of Health (NIH) toward practical goals in the prevention, diagnosis, and management of liver and biliary diseases. In this chapter, the authors first review the types of liver disease that occur in children and their symptoms, complications, etiology, and mechanisms of injury, then outline recent research advances in the areas of pathogenesis, and prevention and therapy. The authors then provide specific research goals in the areas of pathogenesis and management of biliary atresia, the molecular pathogenesis and treatment of neonatal cholestatic syndromes, the characterization and treatment of pediatric liver diseases beyond the neonatal period, and the optimization of liver transplantation in children. A final section considers the steps that would assist in achieving these research goals. One chart summarizes the short (0 to 3 years), intermediate (4 to 6 years), and long-term (7 to 10 years) goals of research on these topics. 1 figure. 1 table.

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Update on Current Standards of Care in the Diagnosis and Management of Non-Alcoholic Fatty Liver Disease (NAFLD) and Non-Alcoholic Steatohepatis (NASH): Diagnosis: Part 1. Practical Gastroenterology. 28(9): 70-88. September 2004.

Steatosis (fatty infiltration of the liver) also called non-alcoholic fatty liver disease (NAFLD), is increasingly recognized as a common cause of chronic liver disease. This disease ranges from the presence of fatty liver without inflammation to steatosis accompanied by inflammation and fibrosis. NAFLD is associated with obesity, hypertension (high blood pressure), hyperlipidemia (increased levels of blood fats), diabetes mellitus, and insulin resistance. These disorders are collectively known as the Metabolic Syndrome. This article reviews recommendations and the standard of care for the medical treatment of patients with NAFLD. The authors note that NAFLD is progressive and may lead to non-alcoholic steatohepatitis (NASH). NASH can, in turn, lead to cirrhosis (liver scarring) and end stage liver disease ultimately requiring liver transplantation. The authors review diagnostic tests, including liver chemistries, imaging studies, and the role of liver biopsy. NASH can be silent without any physical symptoms or lab abnormalities and may be associated with end stage 'cryptogenic' cirrhosis as well as hepatic (liver) cancer. For that reason, physicians must be alert in recognizing the clinical features of NASH so that earlier diagnosis, treatment, or at least monitoring, can be provided. The authors note that there is still no evidence-based definitive medical treatment of NASH. 5 tables. 125 references.

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Advice to Travelers. In: Textbook of Gastroenterology. 4th ed. [2-volume set]. Hagerstown, MD: Lippincott Williams and Wilkins. 2003. p. 1120-1134.

This chapter on providing advice to travelers is from a lengthy, two-volume textbook that integrates the various demands of science, technology, expanding information, good judgment, and common sense into the diagnosis and management of gastrointestinal patients. The author stresses that the goal of the physician should be to minimize the traveler's risk for illness. To accomplish this goal, physicians must rely on educating travelers about specific risks and methods to reduce them, appropriate vaccinations and prophylactic medications, and the provision of medicines to treat certain illnesses that may arise during travel. The key to providing these services is the pretravel office visit, a visit that optimally should take place at least 6 weeks before the trip. The author reviews the important components of the pretravel history, discusses noninfectious risks to travelers, provides a detailed description of the vaccination options available to travelers, discusses the problem of malaria prevention, describes current approaches to preventing travelers' diarrhea, and concludes with a brief synopsis of how to approach the problem of diarrhea in the returning traveler. Issues that may be of special interest to the gastroenterologist include the problem of prevention of travelers' diarrhea in persons with altered gastric acidity, the diagnostic approach to diarrhea in the returned traveler, and the use of vaccines and prophylactic medications in the immunocompromised patient (such as patients who have undergone liver transplantation). 2 figures. 4 tables. 82 references.

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Functional Outcomes of Pediatric Liver Transplantation. Journal of Pediatric Gastroenterology and Nutrition. 37(2): 155-160. August 2003.

The functional status and health related quality of life (HRQOL) of children who survive liver transplantation (LT) have not been well documented. This article reports on a study undertaken to determine the functional status and HRQOL in this population using a validated measure for children, the Child Health Questionnaire-Parent Form 50 (CHQ-PF50). The CHQ-PF50 instrument was completed by the parents of 55 children; subscale scores for the sample were compared with those of a published normal population (n = 391). Study sample characteristics were: 87 percent Caucasian, 54.5 percent female, mean age at survey was 9.6 years (range 5 to 17 years). Responding caregivers were 95 percent biologic parents and 93 percent female. Compared with the normal population, LT recipients had lower subscale scores for general health perceptions, emotional impact on parents, and disruption of family activities. The mean physical summary score of the LT recipients was lower than that of the normal population, but the mean psychosocial summary score was similar. Within the LT population, the original diagnosis, type of LT, age at LT, z score for height, and hospital days did not significantly influence any of the subscale scores. 5 tables. 19 references.

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Hepatocellular Carcinoma. In: Textbook of Gastroenterology. 4th ed. [2-volume set]. Hagerstown, MD: Lippincott Williams and Wilkins. 2003. p. 2491-2512.

Hepatocellular carcinoma (HCC, liver cancer) is the fifth most common cancer and the third most frequent cause of cancer death worldwide, with an estimated 560,000 new cases per year. There are strong etiologic (causative) associations with chronic hepatitis B virus, chronic hepatitis C virus, alcoholic cirrhosis, other causes of chronic liver disease, and dietary aflatoxin exposure. This chapter on HCC is from a comprehensive gastroenterology textbook that provides an encyclopedic discussion of virtually all the disease states encountered in a gastroenterology practice. In this chapter, the authors cover epidemiology, etiology and risk factors, pathogenesis, surveillance, differential diagnosis, diagnosis, staging, locoregional therapies, and systemic therapy. The authors conclude that the selection of an appropriate treatment strategy for patients with HCC depends on careful tumor staging and assessment of the underlying liver disease. All patients with localized HCC should be evaluated for the potentially curative therapy options of partial hepatectomy or liver transplantation. Given the lack of efficacy data, there are no proven systemic chemotherapy regimens, immunotherapy approaches, or hormonal therapies that can be recommended at this time. The chapter is illustrated with black-and-white graphs and drawings. 9 figures. 4 tables. 165 references.

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Inflammatory Bowel Disease After Liver Transplantation: The Effect of Different Immunosuppressive Regimens. Alimentary Pharmacology and Therapeutics. 18(1): 33-44. July 2003.

Seemingly conflicting results have been reported on the prevalence and severity of inflammatory bowel disease (IBD) after liver transplantation. Regimens with different combinations of drugs can be used for immunosuppression after transplantation. This article reports on a retrospective study of the prevalence of IBD after liver transplantation and the possible relationship with maintenance immunosuppressive regimens. The study included 78 patients with end stage primary sclerosing cholangitis (48 patients) or autoimmune cirrhosis (30 patients), transplanted between 1979 and July 2001, and with a follow up of at least 1 year. The median follow up after transplantation was 7.2 years. Nine of 25 patients with pre-transplant IBD experienced flare-ups after transplantation. Six of 53 patients without pre-transplant IBD developed de novo IBD after transplantation. The IBD-free survival was significantly higher in patients not receiving tacrolimus versus those receiving tacrolimus, in those receiving azathioprine versus those not receiving azathioprine, and in patients taking the regimen prednisolone-azathioprine-cyclosporin A versus those tacking tacrolimus-prednisolone. Pretransplant IBD and the use of tacrolimus were found to be independent predictors for IBD after transplantation. Azathioprine appears to have a protective effect. 2 figures. 4 tables. 20 references.

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Liver Transplantation for Hepatic and Neurological Wilson's Disease. Transplantation Proceedings. 35(4): 1445-1446. June 2003.

Wilson's disease (WD) is an autosomal recessive inherited disorder of copper metabolism characterized by excessive deposition of copper throughout the body. If medical treatment fails in cases of fulminant hepatic (liver) failure and progressive hepatic dysfunction due to advanced cirrhosis, liver transplantation (OLTx) has been demonstrated to be a valuable treatment option. This article reports on a subset from a series of 225 OLTxs in 198 patients that were performed in the authors' institution between 1993 and 2002. In this consecutive series, six patients ( 3 female, 3 male) were liver grafted for WD. The follow up ranged from 3 to 7 years. All patients are alive with well-functioning grafts at present. The ceruloplasmin levels increased after transplantation and remained normal. The Kayser-Fleischer ring disappeared in all patients, and urinary copper excretion normalized. The neurological manifestations in the 2 patients with severe neurological symptoms showed after 2 to 5 years a downward tendency; in one, the ataxic movements disappeared completely. The psychiatric disorder in one patient disappeared as did the mild neurological symptoms in the patient with CHILD A cirrhosis. These two patients are fully recovered and returned to work. The authors conclude that OLTx should be considered as a treatment option in patients with severe progressive neurological deficits, even in cases with stable liver function, since early liver grafting definitely cures the underlying biochemical defect. In such cases an early decision for liver transplantation is justified because neurological deficits may become irreversible. 4 references.

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Liver Transplantation. In: Textbook of Gastroenterology. 4th ed. [2-volume set]. Hagerstown, MD: Lippincott Williams and Wilkins. 2003. p. 2468-2490.

In the fourth decade since the inception of human liver transplantation, this procedure has become a highly successful treatment for patients with life-threatening, advanced liver disease. Many liver transplant centers have now achieved 1-year patient survival rates of about 90 percent and 5-year survival rates of 70 to 80 percent. The success of liver transplantation is, however, limited by the serious crisis of donor shortage. This chapter on liver transplantation is from a comprehensive gastroenterology textbook that provides an encyclopedic discussion of virtually all the disease states encountered in a gastroenterology practice. In this chapter, the authors cover surgical techniques and preservation, indications for liver transplantation, evaluation for liver transplantation, and posttransplant management. The chapter is illustrated with black-and-white graphs and drawings. 4 figures. 7 tables. 276 references.

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Non-alcoholic Fatty Liver Disease. Alimentary Pharmacology and Therapeutics. 17(8): 977-986. April 2003.

This article reviews non-alcoholic fatty liver disease (NAFLD), a clinicopathological condition that comprises a wide spectrum of liver damage, ranging from simple steatosis to steatohepatitis, advanced fibrosis, and cirrhosis (scarring). Non-alcoholic steatohepatitis represents only a stage within the spectrum of NAFLD and is defined pathologically by the presence of steatosis together with necro-inflammatory activity. The true prevalence of NAFLD is unknown, but it is estimated that it affects 10 to 24 percent of the general population in different countries. The diagnosis of NAFLD is based upon convincing evidence of absent or minimal alcohol consumption, compatible histological changes in liver biopsy, and the exclusion of other liver diseases. The natural history of NAFLD remains to be defined. Patients with pure steatosis on liver biopsy follow a relatively benign course, whereas patients with histological necro-inflammatory changes or fibrosis may progress to end-stage liver disease. An initial step in the treatment of NAFLD is the management of associated conditions, such as obesity, diabetes mellitus, and hyperlipidemia. NAFLD patients with steatohepatitis or fibrosis on liver biopsy may benefit from investigational pharmacological therapy. Patients with decompensated cirrhosis from NAFLD may be candidates for liver transplantation. 3 tables. 80 references.

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Pathogenesis and Management of Alcoholic Hepatitis. Review. Journal of Gastroenterology and Hepatology. 18(12): 1332-1344. December 2003.

Alcoholic hepatitis is a potentially life-threatening complication of alcoholic abuse, typically presenting with symptoms and signs of hepatitis in the presence of an alcohol use disorder. This article reviews the pathogenesis and management of alcoholic hepatitis. The definitive diagnosis requires liver biopsy, but this is not generally required. The pathogenesis is uncertain, but relevant factors include metabolism of alcohol to toxic products, oxidant stress, acetaldehyde adducts, the action of endotoxin on Kupffer cells, and impaired hepatic (liver) regeneration. Mild alcoholic hepatitis recovers with abstinence and the long-term prognosis is determined by the underlying disorder of alcohol use. Severe alcoholic hepatitis requires abstinence from alcohol and supportive care. Corticosteroids have been shown to be beneficial in a subset of severely ill patients with concomitant hepatic encephalopathy, but their use remains controversial. Nutritional supplementation with available high-calorie, high-protein diets is beneficial, but does not improve mortality. Orthotopic liver transplantation is not indicated for patients presenting with alcoholic hepatitis who have been drinking until the time of admission, but may be considered in those who achieve stable abstinence if liver function fails to recover. 2 figures. 3 tables. 131 references.

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Survey of Liver Transplantation from Living Adult Donors in the United States. New England Journal of Medicine. NEJM. 348(9): 818-825. February 27, 2003.

The transplantation of the right lobe of a liver from a living adult donor into an adult recipient has been performed increasingly frequently in the United States. Although the use of grafts from living donors is standard practice in transplantation in children, their use in adults remains controversial. This article reports on a study that used a 24 item questionnaire, sent to all liver transplantation programs in the U.S., to collect data on the indications, evaluation, and outcomes of the use of liver transplantation from a living donor. Questionnaires were returned by 84 of the 122 programs (69 percent) describing the results of 449 adult-to-adult transplantations of partial livers from living donors that were performed in 42 centers. A total of 45 percent of potential donors who were evaluated eventually donated a lobe of their liver; 99 percent of these donors were genetically or emotionally related to the recipient. Complications in the donor were more frequent in the centers performing the fewest transplantations from living donors in adults and included biliary complications requiring intervention (6.0 percent), reoperation (4.5 percent), and death (in one donor). Among the recipients, 1.6 percent did not meet criteria for receipt of a cadaveric transplant; cancer, retransplantation, and acute liver failure were uncommon indications for transplantation from a living donor. Biliary complications occurred in 22.0 percent of recipient, and vascular complications occurred in 9.8 percent. The authors conclude that adult-to-adult liver transplantation from a living donor is increasingly performed in the United States but is concentrated in a few large-volume centers. Mortality among donors is low, but complications in the donor are relatively common. 2 figures. 3 tables. 26 references.

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What I Need to Know About Liver Transplantation. Bethesda, MD: National Digestive Disease Information Clearinghouse (NDDIC), National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK), National Institutes of Health. 2003. 24 p.

This booklet explains liver transplantation in clear, nontechnical language. Topics include the role of the liver, the signs of liver problems, a description of liver transplantation, the waiting period to get a new liver, where donated livers come from, what one can expect in the hospital before and after the transplant, rejection and how it can be treated, other problems that can damage the transplant, postoperative care, recovery time at home, and daily activities, including work, diet, exercise, and sexuality. The booklet includes simple line drawings to illustrate the concepts discussed. A glossary of terms is included, as is a list of three resource organizations that provide readers with additional information. The booklet concludes with a brief description of the National Digestive Diseases Information Clearinghouse (NDDIC), an informational services of the National Institutes of Health. 10 figures.

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Acute Liver Failure. In: Sherlock, S.; Dooley, J. Diseases of the Liver and Biliary System. Malden, MA: Blackwell Science, Inc. 2002. p.111-126.

Acute liver failure describes the clinical syndrome of severe impairment of liver function (encephalopathy, coagulopathy and jaundice) within 6 months of the onset of symptoms. Although usually due to an acute insult (most frequently virus or drug) in a previously healthy person, acute liver failure may be the presenting feature of chronic liver disease, in particularly Wilson's disease, autoimmune chronic hepatitis, or delta superinfection in a patient with chronic hepatitis B. This chapter on acute liver failure is from a textbook that presents a comprehensive and up-to-date account of diseases of the liver and biliary system. Topics include a definition, causes, clinical features, investigations, associations, prognosis, and treatment. The authors conclude that liver transplantation cannot be accepted as the perfect and ideal treatment for fulminant hepatic failure, but it gives survival to many patients who otherwise would have died. Early referral of patients to a specialist center is crucial. 6 figures. 11 tables. 93 references.

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Acute Liver Failure. In: Feldman, M.; Friedman, L.S.; Sleisenger, M.H. Sleisenger and Fordtran's Gastrointestinal and Liver Disease: Pathophysiology/Diagnosis/Management. 7th ed. [2-volume set]. St. Louis, MO: Saunders. 2002. p. 1567-1576.

Acute liver failure (ALF), also known as fulminant hepatic failure, is a rare manifestation of liver disease and constitutes a medical emergency. Despite advances in medical management and the availability of liver transplantation, mortality rates in patients with ALF remain substantial. This chapter on ALF is from a comprehensive and authoritative textbook that covers disorders of the gastrointestinal tract, biliary tree, pancreas, and liver, as well as the related topics of nutrition and peritoneal disorders. Topics include definition; causes, including drugs, hepatotropic viruses, and ALF of unknown etiology; clinical presentation (symptoms), including hepatocellular dysfunction, hepatic encephalopathy and cerebral edema (fluid on the brain), infection, gastrointestinal bleeding, and multiple organ failure syndrome; differential diagnosis; predictors of outcome; and management considerations, including issues in medical management, liver transplantation, and experimental therapy. The chapter includes a mini-outline with page citations, full-color illustrations, and extensive references. 1 figure. 4 tables. 87 references.

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Adult-to-Adult Right Hepatic Lobe Living Donor Liver Transplantation. Alimentary Pharmacology and Therapeutics. 16(11): 1833-1841. November 2002.

Spurred on by the critical shortage of cadaveric livers, adult-to-adult right hepatic lobe living donor liver transplantation has grown rapidly as a therapeutic option for selected patients. This review covers a range of related topics, including recipient and donor selection and outcomes, donor risk, controversies, and future issues. In the United States alone, the number of living donor liver transplantations has increased six-fold in the last 4 years. The therapy can be complex, bringing together a variety of disciplines, including transplantation medicine and surgery, hepatology, psychiatry, and medical ethics. Moreover, living donor liver transplantation is still defining itself in the adult-to-adult application. Uniform standards, guidelines and long-term outcomes are yet to be determined. Nevertheless, initial success has been remarkable, and a basic understanding of this field is essential to any physician contemplating options for their liver failure patients. 3 figures. 2 tables. 44 references.

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Alagille Syndrome. Birmingham, England: Children's Liver Disease Foundation. 2002. 8 p.

Alagille syndrome is a type of liver condition characterized by biliary hypoplasia (lack of development of some of the bile ducts) in association with at least two other signs in other parts of the body. These other signs can include typical facial features, cardiovascular abnormalities, eye abnormalities, and abnormal shape to some of the bones in the spine. This brochure helps parents and caregivers of children recently diagnosed with Alagille syndrome. Causes of Alagille syndrome, the role of genetic testing in families who already have one child with Alagille syndrome, diagnostic tests that confirm the presence of Alagille syndrome, how liver disease affects the child, and treatment options, including the possibility of liver transplantation are discussed. The author stresses that while Alagille syndrome is a serious condition, in the majority of cases many of the symptoms will improve between the age of 5 and 10 years. The brochure also describes the work of the England-based Children’s Liver Disease Foundation, an organization started in 1980 by a group of parents who wanted to support research for children’s liver diseases and to help families coping with liver disease. The brochure encourages parents to contact the Children’s Liver Disease Foundation for publications and support. A form with which readers can donate to the Children’s Liver Disease Foundation is provided.

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Alcoholic Liver Disease. In: Feldman, M.; Friedman, L.S.; Sleisenger, M.H. Sleisenger and Fordtran's Gastrointestinal and Liver Disease: Pathophysiology/Diagnosis/Management. 7th ed. [2-volume set]. St. Louis, MO: Saunders. 2002. p. 1375-1391.

This chapter on alcoholic liver disease is from a comprehensive and authoritative textbook that covers disorders of the gastrointestinal tract, biliary tree, pancreas, and liver, as well as the related topics of nutrition and peritoneal disorders. Topics include epidemiology, ethanol metabolism (both hepatic, i.e., liver and gastric, i.e., stomach metabolism), the pathogenesis of alcoholic liver injury, cofactors in the development of alcoholic liver disease (heritable factors, gender, diet and nutrition, coexistent viral hepatitis), diagnostic considerations, complications, treatment options, and prognosis. Treatments discussed include abstinence from alcohol intake, nutritional supplements, anti-inflammatory drugs, antioxidants, drugs with unconfirmed benefit, and liver transplantation. The chapter includes a mini-outline with page citations, full-color illustrations, and extensive references. 7 figures. 6 tables. 188 references.

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Assessment of the Reversibility and Treatments of Alcoholic Liver Disease. In: Tsuji, T., et al, eds. Molecular Biology and Immunology in Hepatology. St. Louis, MO: Elsevier Science. 2002. p. 183-203.

The results of many epidemiologic studies clearly demonstrate that excessive use of alcohol remains the most important cause of cirrhosis (liver scarring) in the Western world and a leading cause of mortality during mid-life, especially in males. This chapter on the reversibility and treatments of alcoholic liver disease (ALD) is from a text book on the pathogenesis and treatment of intractable liver diseases. This chapter reviews general measures for the treatment of ALD, including abstinence from alcohol, nutritive support, relief of vitamin and trace element deficiencies, and, in more detail, specific pharmacotherapy. Topics covered include the spectrum of alcoholic liver disease and these general treatment measures. The chapter concludes with a brief discussion of liver transplantation and the controversy of transplantation in patients with ALD. 2 figures. 2 tables. 119 references.

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Benign Stricture of the Bile Ducts. In: Sherlock, S.; Dooley, J. Diseases of the Liver and Biliary System. Malden, MA: Blackwell Science, Inc. 2002. p.629-637.

Benign strictures of the biliary system are uncommon and usually follow surgery, in particular cholecystectomy, laparoscopic or open. They may also complicate liver transplantation. Cystic lesions of the liver and bile ducts are increasingly being diagnosed. This chapter on benign stricture of the bile ducts is from a textbook that presents a comprehensive and up-to-date account of diseases of the liver and biliary system. The authors note that other causes are primary sclerosing cholangitis, chronic pancreatitis, and abdominal trauma. Clinical features are cholestasis with or without sepsis and pain. Diagnosis is by cholangiography. In most cases, the underlying cause is clear from the clinical data. The authors conclude that in all benign strictures of the bile duct, the outcome depends on the experience and judgment of the team of surgeon, endoscopist and radiologist, in selecting and performing the most suitable corrective procedure tailored to the individual patient. 7 figures. 1 table. 33 references.

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Autoimmune Liver Disease. Birmingham, England: Children's Liver Disease Foundation. 2002. 8 p.

Autoimmune diseases are caused by the body’s defense mechanism (the immune system) malfunctioning and attacking part of itself. This brochure provides information for parents of children diagnosed with autoimmune liver disease, including autoimmune hepatitis and autoimmune sclerosing cholangitis. Autoimmune sclerosing cholangitis is very similar to autoimmune hepatitis, except that the former attacks not only the liver cells, but also the bile ducts inside and outside the liver. The brochure covers the symptoms, diagnosis and treatments for these diseases, including the use of immunosuppressive agents and their side effects, as well as the indications for liver transplantation. The most common symptoms are tiredness and lack of appetite; other symptoms can include weight loss, nausea, itching, fever, nose bleeds, diarrhea, jaundice, and abdominal pain. Late symptoms include swollen abdomen (ascites), puffiness or swelling of the legs (edema), and irritability or confusion. A diagnosis is often made by excluding other causes of liver disease; liver biopsy and liver function tests are also used. Most treatments feature immunosuppressive drugs used to slow the action of the immune system. The brochure describes the work of the England-based Children’s Liver Disease Foundation, an organization started in 1980 by a group of parents who wanted to support research for children’s liver diseases and to help families coping with liver disease. A form with which readers can donate to the Children’s Liver Disease Foundation is provided.

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Burden of Hepatitis C in the United States. Hepatology. 36(5 Supplemental 1): S30-S34. November 2002.

This article on the burden of hepatitis C in the United States is from a special supplemental issue of Hepatology journal on the National Institutes of Health (NIH) Consensus Development Conference (June 2002) on the management of hepatitis C. According to the third National Health and Nutrition Examination Survey (NHANES), 3.9 million of the United States civilian population have been infected with hepatitis C virus (HCV), of whom 2.7 million (74 percent) have chronic infection. HCV infection is most common among non-Caucasian men, ages 30 to 49 years. Moreover, the prevalence of antibody to hepatitis C virus in groups not represented in the NHANES sample, such as the homeless or incarcerated, may be as high as 40 percent. The age-adjusted death rate for non-A, non-B viral hepatitis increased from 0.4 to 1.8 deaths per 100,000 persons per year between 1982 and 1999. In 1999, the first year hepatitis C was reported separately, there were 3,759 deaths attributed to HCV, although this is likely an underestimate. There was a 5-fold increase in the annual number of patients with HCV who underwent liver transplantation between 1990 and 2000. Currently, more than one third of liver transplant candidates have HCV. Inpatient care of HCV-related liver disease has also been increasing. In 1998, an estimated 140,000 discharges listed an HCV-related diagnosis, accounting for 2 percent of discharges from non-federal acute care hospitals in the United States. The total direct health care cost associated with HCV is estimated to have exceeded $1 billion in 1998. Future projections predict a 4-fold increase between 1990 and 2015 in persons at risk of chronic liver disease (i.e., those with infection for 20 years or longer), suggesting a continued rise in the burden of HCV in the U.S. in the foreseeable future. 3 figures. 2 tables. 18 references.

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Children with Hepatitis B: A Growing Problem. In: Everson, G.T.; Weinberg, H. Living with Hepatitis B: A Survivor's Guide. Long Island, NY: Hatherleigh Press. 2002. p.229-252.

Chronic hepatitis B can lead to cirrhosis (liver scarring), liver cancer, and the need for liver transplantation. This chapter on children with hepatitis B is from a book that helps readers diagnosed with hepatitis B virus (HBV) infection educate themselves about the disease and its treatment. After a brief overview of transmission issues, the authors discuss diagnostic tests, preventing the infection, pregnancy or breastfeeding in women with hepatitis B, disclosure of the child's disease, chronic disease and the family, the course of the infection in children, dealing with blood draws and biopsies, monitoring children with hepatitis B (including the role of liver biopsy), and treatment considerations, including the indications for interferon, giving injections, nonresponders, lamivudine, and posttreatment follow up. Throughout the chapter the authors include quotes from real people who are parenting children with hepatitis. The authors also include resources for both parents and children that may offer additional support and information for readers. 1 reference.

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Chronic Hepatitis C: Latest Treatment Options. Nurse Practitioner. 27(4): 32-33, 37-40, 42, 47-49. April 2002.

The most common chronic bloodborne infection in the United States, hepatitis C virus (HCV) is the most frequent reason for liver transplantation. Unfortunately, most infected individuals do not realize that they are HCV positive and only discover the disease after severe liver damage has occurred. This article updates nurses on the epidemiology, transmission, risk factors, diagnosis, clinical presentation, and management of chronic HCV. A patient treatment algorithm is provided. The author also focuses on counseling and quality of life issues for infected patients. 6 figures. 52 references.

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Complications. In: Green, W.F. First Year: Hepatitis B. New York, NY: Marlowe and Company. 2002. p. 191-201.

Viral hepatitis B (liver infection) is one of the most preventable medical conditions due to the availability of a hepatitis B vaccine, yet an estimated 100,000 people in the United States are infected each year, and 6,000 die from complications. When the author of this book was diagnosed in 1993, he decided to be proactive in his quest to understand and manage his illness. In this chapter, the author focuses on what readers might be learning about by the eighth month after they receive their diagnosis of hepatitis B virus (HBV) infection, discussing complications of the disease. In nontechnical language, the author discusses how hepatitis B can lead to cirrhosis (scarring of the liver) and the problems that can subsequently arise from the cirrhosis, including fluid build up, hormonal imbalances, changes in the heart or lungs, vulnerability to bruising and bleeding, sensitivity to medications, portal hypertension, encephalopathy (brain swelling), and kidney failure. The second section of the chapter addresses liver transplants, including how to get on a waiting list, how to qualify for a transplant, living donors, success rates for liver transplantation, and co-infection with HIV. The chapter concludes with a list of resource organizations about organ donation for readers wishing more information.

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Current Strategy of Living Related Liver Transplantation: Overview. In: Tsuji, T., et al, eds. Molecular Biology and Immunology in Hepatology. St. Louis, MO: Elsevier Science. 2002. p. 307-317.

Currently, liver transplantation is the successful and fully accepted treatment for end stage liver disease; however, organ donor shortage continues to be a critical problem. To solve the critical shortage of graft organs, living related liver transplantation (LRLT) has been receiving much attention as an option to expand the donor reserves. This chapter on current strategies of LRLT is from a text book on the pathogenesis and treatment of intractable liver diseases. The authors describe their ten years' experience with LRLT at their institute. Topics include donor selection, the expected results of LRLT, graft size matching, right lobe LRLT for adults, and future directions. 67 references.

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Current Treatment of Hepatitis C. In: Gordon, S.C. Management of Chronic Viral Hepatitis. New York, NY: Marcel Dekker Inc. 2002. p. 187-216.

This chapter on the current treatment of hepatitis C is from a monograph on the management of chronic viral hepatitis (liver inflammation), bringing the advances of clinical and basic research into the doctor's office. Two brief case presentations to address the real life intricacies of managing patients who present with viral hepatitis. Approximately 20 to 30 percent of patients infected with hepatitis C virus (HCV) will develop cirrhosis (liver scarring) and are at risk for developing complications of end stage liver disease, including hepatocellular carcinoma (HCC, liver cancer). Chronic hepatitis C is now the most common indication for liver transplantation in the United States. Thus, there is a need for effective therapies to treat HCV infection. For initial treatment of patients with hepatitis C, the most effective therapy in terms of biochemical, virological, and histological response criteria is the combination of interferon (IFN) and ribavirin. The duration of this therapy should be based on HCV genotype, with 48 weeks of therapy for patients with type 1 genotype and 24 weeks of therapy for other genotypes. Patients who are not candidates for IFN plus ribavirin combination therapy can be considered for IFN monotherapy. Side effects and the need for dosage modification or discontinuation are more frequent with IFN plus ribavirin compared to IFN monotherapy, but these can usually be managed with close follow up and careful monitoring. 3 figures. 6 tables. 73 references.

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Cystic Fibrosis and Liver Disease. Birmingham, England: Children's Liver Disease Foundation. 2002. 7 p.

A number of children and young people with cystic fibrosis (CF) develop liver problems later in life. This brochure provides information for parents of children with CF who have been recently diagnosed with liver disease. The author describes how CF can damage the bile ducts and then the liver, in a similar manner to the damage caused to the lungs; the tests used to diagnose liver problems, including physical examination, liver function tests, and abdominal ultrasound; complications including portal hypertension, an enlarged spleen, and disturbance of normal functioning of the liver; medical and nutritional treatments that may be used; and the diagnosis and treatment of varices. The author also notes that liver transplantation can be an effective treatment for children with cystic fibrosis who have severe liver disease. The brochure also describes the work of the England-based Children’s Liver Disease Foundation, an organization started in 1980 by a group of parents who wanted to support research for children’s liver diseases and to help families coping with liver disease. A form with which readers can donate to the Children’s Liver Disease Foundation is provided.

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Diagnosis and Management of Fluid Retention and Refractory Ascites. Practical Gastroenterology. 26(4): 22,24, 25, 29-30, 32. April 2002.

This article discusses the diagnosis and management of fluid retentation and refractory (resistant to treatment) ascites. Ascites is a pathologic accumulation of intraperitoneal fluid that most commonly occurs as a result of underlying cirrhosis (liver scarring). The development of ascites marks an important worsening in the patient with cirrhosis, and has an impact on both morbidity (illness) and mortality (death). The definitive treatment in the majority of cases is orthotopic liver transplantation and appropriate patients should be referred in the early stages. In efforts to bridge the gap to transplantation, medical therapy should be initiated to improve quality of life and to decrease the risk of complications of ascites. The majority of patients with ascites can be successfully managed with sodium (salt) restriction and diuretic therapy. A small percentage of patients are refractory to conventional therapies. These patients are often at the advanced stage of their disease and must be managed cautiously with more invasive measures. 12 references.

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Getting a New Liver: Facts About Liver Transplants. Laurel, NJ: American Society for Transplantation. 2002. 15 p.

This booklet provides information for patients about to undergo liver transplantation. The booklet is designed to help patients understand the process and to become a more informed member of their own health care team. The document first lists facts about the liver and its role in the body, then covers pretransplant evaluation (including diagnostic tests), the waiting period, the transplant admission, living-donor transplantation, and pediatric liver transplantation. Step-by-step details are provided in non-technical language to help readers understand each procedure and what to expect. Throughout the booklet, readers are encouraged to take a proactive role in preventing and treating complications and thus help to ensure a healthy transplant. Simple graphics illustrate the booklet. The contact information for the American Society of Transplantation, Patient Care and Education Committee, is provided (ast@ahint.com). 1 figure.

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Disease Recurrence After Liver Transplantation: Need We Worry? (editorial). Journal of Gastroenterology and Hepatology. 17(7): 733-736. July 2002.

It has long been recognized that in patients transplanted for hepatitis B or C related liver disease, recurrence of hepatitis infection in the graft is common and that this may result in serious and progressive liver injury. There are now varying levels of evidence to suggest that most other forms of chronic liver disease also have the potential to recur in the graft. This editorial stresses that it is important for those involved in the care of liver transplant recipients to recognize the possibility of disease recurrence, so that patients may be adequately counseled, liver biochemistry, radiology and histology correctly interpreted, and to ensure that appropriate therapy is instituted. The authors briefly report on recent studies in this area. The authors conclude that is it likely that as the overall results of liver transplantation continue to improve, recurrence of diseases such as primary sclerosing cholangitis, autoimmune hepatitis, and hepatitis C disease will become increasingly recognized as a major cause of late morbidity and mortality. In contrast, the problems of recurrence of hepatitis B and hepatocellular cancer have now largely been overcome, resulting in an increased demand for transplantation for these indications, at a time when overall organ donor rates are falling. This has major implications for both patient selection and donor organ allocation.

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Haemochromatosis. Alimentary Pharmacology and Therapeutics. 16(12): 1963-1975. December 2002.

This article reviews hereditary hemochromatosis, the prototype disease for primary iron overload. The disorder is very common, especially amongst subjects of Northern European extraction. The disorder is characterized by an autosomal recessive mode of inheritance. Hemochromatosis is now recognized to be a complex genetic disease with probable significant environmental and genetic modifying factors. The early diagnosis of individuals at risk for the development of hemochromatosis is important, because survival and morbidity (related illness) are improved if phlebotomy (blood-letting) therapy is instituted before the development of cirrhosis (liver scarring). The cost-effectiveness and utility of large-scale screening for hemochromatosis have been questioned, given that many individuals with the genetic mutation do not have iron overload or end-organ damage. Liver biopsy remains important in management to determine the presence or absence of cirrhosis. Patients with end stage liver disease may develop liver failure or primary liver cancer, and liver transplantation may be required. Liver transplantation for hemochromatosis is associated with a poorer outcome compared with other indications because of infections and cardiac complications. 2 figures. 1 table. 102 references.

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Hepatic Transplantation. In: Sherlock, S.; Dooley, J. Diseases of the Liver and Biliary System. Malden, MA: Blackwell Science, Inc. 2002. p.657-679.

Elective liver transplantation in low-risk patients has a 90 percent 1-year survival rate. Improved results can be related to more careful patient selection, to better surgical techniques and postoperative care, better immunosuppression, and to greater willingness to re-transplant after rejection. This chapter on hepatic (liver) transplantation is from a textbook that presents a comprehensive and up-to-date account of diseases of the liver and biliary system. Topics include patient selection; the anticipated outcome in candidates with cirrhosis (scarring), autoimmune chronic hepatitis, chronic viral hepatitis, neonatal hepatitis, alcoholic liver disease, cholestatic liver disease, primary metabolic disease, acute liver failure, and malignant disease; absolute and relative contraindications to liver transplantation; general preparation of the patient; donor selection and operation; the recipient operation, including segmental (split liver) transplantation, auxiliary liver transplantation, xeno-transplantation, domino liver transplantation, hepatocyte transplantation, and liver transplantation in pediatrics; immunosuppression, including tolerance; postoperative course; and post-transplantation complications, including rejection, infections, malignancies (cancer), drug-related toxicity, disease recurrence, central nervous system toxicity, bone disease, and ectopic soft-tissue calcification. 16 figures. 11 tables. 124 references.

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Hepatitis C Virus. In: Sherlock, S.; Dooley, J. Diseases of the Liver and Biliary System. Malden, MA: Blackwell Science, Inc. 2002. p.305-319.

This chapter on hepatitis C virus (HCV) is from a textbook that presents a comprehensive and up-to-date account of diseases of the liver and biliary system. The chapter covers molecular virology of this disease, serological (blood) tests, immune response, epidemiology, natural history, clinical course, hepatic histology, hepatitis C and serum autoantibodies, associated diseases, diagnosis, prognosis, prevention (including vaccines), treatment strategies, and hepatic (liver) transplantation. Combination therapy with interferon alpha and ribavirin is the current standard of care for chronic hepatitis C. This offers a 30 to 65 percent change of sustained virological response, depending on various factors in particular the HCV genotype. However, the cost and availability makes all the present treatments out of reach of millions of people with HCV worldwide. 9 figures. 7 tables. 129 references.

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Hepatitis C: Understanding Chronic HCV Infection. Yardley, PA: The StayWell Company: KRAMES Health and Safety Education. 2002. 16 p.

Hepatitis C is an infectious disease that damages the liver. This booklet helps readers understand chronic hepatitis C, a common occurrence after infection with the hepatitis C virus. The booklet describes chronic hepatitis C, the importance of maintaining one's health when hepatitis C is present, how to prevent others from becoming infected, the physiology of the healthy liver, the consequences of hepatitis C virus (HCV) infection, how liver damage happens, how the disease is transmitted, diagnostic approaches, how liver health is assessed, treatment options and how to decide amongst them, how to protect the liver from further damage (avoid alcohol, watch medications and supplements, eat well), how to prevent transmission of the infection (dispose of needles safely, practice safer sex, do not share toiletries), how to cope with liver damage (including the indications for liver transplantation). The brochure concludes with the answers to some common questions, a glossary of related terms, and the contact information for four resource organizations that can provide additional information about hepatitis C. The brochure is illustrated with full-color line drawings. 25 figures.

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Hepatorenal Syndrome in Cirrhosis: Pathogenesis and Treatment. Gastroenterology. 122(6): 1658-1676. 2002.

Hepatorenal syndrome (HRS) is a major complication in cirrhosis (liver scarring), with an annual incidence in patients with ascites (fluid accumulation) of approximately 8 percent. HRS develops at the latest phase of the disease and, although initially considered not to have an impact on the patient's prognosis (patients would die with and not by the kidney failure), there is now evidence that HRS is an important determinant in survival. This article reviews the publications on HRS, highlighting those aspects of HRS that are important to understand the pathogenesis (development of the disease) and the rational basis of the modern therapy of this syndrome. The most characteristic feature of HRS is a functional renal (kidney) failure caused by an intense renal vasoconstriction, the syndrome is also a more generalized process affecting the heart, brain, and the splanchnic organs (the internal organs). Long term administration of IV albumin and vasoconstrictors or the correction of portal hypertension with a TIPS (transjugular intrahepatic portacaval shunt) are effective treatments of HRS, improve the survival rate, and may serve as a bridge to liver transplantation, which is the treatment of choice in these patients. 11 figures. 2 tables. 187 references.

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Hepatorenal Syndrome in Patients with Cirrhosis. Journal of Gastroenterology and Hepatology. 17(7): 739-747. July 2002.

This article reviews type 1 hepatorenal syndrome (HRS), a severe complication of end-stage cirrhosis (liver scarring). Type 1 HRS is an acute functional renal failure (i.e. glomerular hypofiltration) with no other explanation than the presence of the circulatory and neurohumoral alterations associated with severe chronic liver disease. Plasma volume expansion does not improve renal (kidney) function. In contrast, administration of the vasopressin analog terlipressin, a splanchnic and systemic vasoconstrictor, may improve renal function and be used while awaiting liver transplantation. 3 figures. 3 tables. 63 references.

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HIV/AIDS, Hepatitis C, and Delta Co-Infection: Triple Trouble. In: Everson, G.T.; Weinberg, H. Living with Hepatitis B: A Survivor's Guide. Long Island, NY: Hatherleigh Press. 2002. p.210- 228.

Chronic hepatitis B can lead to cirrhosis (liver scarring), liver cancer, and the need for liver transplantation. This chapter on the viral co-infections is from a book that helps readers diagnosed with hepatitis B virus (HBV) infection educate themselves about the disease and its treatment. The authors discuss several different types of viral co-infections: HIV, hepatitis C and hepatitis D. Topics include definitions of HIV and AIDS, testing problems, population trends, the current treatment of HIV infection (including with highly active anti-retroviral therapies or HAART), complications of HAART therapy, how HIV co-infection affects the rate of progression of liver disease, antiviral therapy of hepatitis B for people co-infected with HIV, co-infection with hepatitis C, co-infection with hepatitis D (delta), and liver transplantation (before and after HAART). Throughout the chapter the authors include quotes from real people who are living with hepatitis. 1 reference.

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Learning About Your Liver: Your Body's Chemical Factory: Liver Facts and Liver Disease Symptoms. In: Everson, G.T.; Weinberg, H. Living with Hepatitis B: A Survivor's Guide. Long Island, NY: Hatherleigh Press. 2002. p.53-77.

Chronic hepatitis B can lead to cirrhosis (liver scarring), liver cancer, and the need for liver transplantation. This chapter on the physiology and anatomy of the liver is from a book that helps readers diagnosed with hepatitis B virus (HBV) infection educate themselves about the disease and its treatment. The authors discuss what the liver looks like, how it functions, and what happens to the liver when it is infected with hepatitis B, including ten warning signals that demonstrate that liver function is compromised. Topics include the history of medical understanding of the liver and its functions; the gross and microscopic anatomy of the liver; how the liver works, including blood, bile, lymph, immune system, chemical factory, bilirubin, ALT, AST, GGT, alkaline phosphatase, albumin, clotting factors, and hormones; the phases of hepatitis B, i.e., infection, inflammation, fibrosis, and cirrhosis; the ten danger signs of liver disease, including early symptoms, changes in liver functions, compensated cirrhosis, decompensated cirrhosis, changes in the appearance of the skin, fluid buildup (ascites), bleeding (variceal hemorrhage), mental confusion (encephalopathy), weight loss, thinning of bones and fractures, blood clotting problems, itching (pruritus); and extra-liver conditions associated with hepatitis B, including kidney damage, inflammation of the arteries (polyarteritis nodosa), hives, arthritis, inflammation of skeletal muscle, Sjogren syndrome, carditis, and neuritis. Throughout the chapter the authors include quotes from real people who are living with hepatitis. 1 figure. 2 references.

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Liver Cancer: Are You at Risk?. In: Everson, G.T.; Weinberg, H. Living with Hepatitis B: A Survivor's Guide. Long Island, NY: Hatherleigh Press. 2002. p.198- 209.

Chronic hepatitis B can lead to cirrhosis (liver scarring), liver cancer, and the need for liver transplantation. This chapter on liver cancer is from a book that helps readers diagnosed with hepatitis B virus (HBV) infection educate themselves about the disease and its treatment. Primary liver cancer is an extremely serious, life threatening complication of chronic hepatitis B. The authors discuss risk factors, warning signs, screening and diagnostic tests for liver cancer, and results of current treatment. Specific topics include primary liver cancer, secondary liver cancer, stage of hepatitis B as a risk factor, duration of infection as a risk factor, other liver disease, warning signs (asymptomatic but with underlying cirrhosis, deterioration in liver function, pain, sudden development of portal hypertension), testing, early screening guidelines, diagnostic tests, treatment options, cancer staging, hepatic (liver) resection, transplantation, chemoembolization, high frequency radio waves (radiofrequency tumor ablation), alcohol injection, cryosurgery, and chemotherapy. Throughout the chapter the authors include quotes from real people who are living with hepatitis.

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Liver Disease in Cystic Fibrosis: A Prospective Study on Incidence, Risk Factors, and Outcome. Hepatology. 36(6): 1374-1382. December 2002.

Incidence of liver disease (LD) associated with cystic fibrosis (CF) and its clinical characterization still is unsettled. The authors of this article assessed prospectively the incidence and risk factors of this complication, and its impact of the clinical course of CF. Between 1980 and 1990, the authors enrolled 177 CF patients without LD in a systematic clinical, laboratory, ultrasonography screening program of at least a 10 year duration. During a 14 year median follow (2,432 patient-years), 48 patients developed LD, with cirrhosis already present in 5 patients. Incidence rate (number of cases per 100 patient-years) was 1.8 percent, with sharp decline after the age of 10 years and higher risk in patients with a history of meconium ileus, male sex, or severe mutations. Incidence of cirrhosis (liver scarring) was 4.5 percent during a median period of 5 years from diagnosis of liver disease. Among the 17 cirrhotic patients, 13 developed portal hypertension, 4 developed esophageal varices, and 1 developed liver decompensation requiring liver transplantation. Development of LD did not condition different mortality or higher incidence of other clinically relevant outcomes. The authors conclude that LD is a relatively frequent and early complication of CF, whose detection should be focused at the first life decade in patients with history of meconium ileus, male sex, or severe genotype. Although LD does not condition a different clinical course of CF, in some patients it may progress rapidly and require liver transplantation. 2 figures. 4 tables. 46 references.

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Liver in Infancy and Childhood. In: Sherlock, S.; Dooley, J. Diseases of the Liver and Biliary System. Malden, MA: Blackwell Science, Inc. 2002. p.453-470.

This chapter on the liver in infancy and childhood is from a textbook that presents a comprehensive and up-to-date account of diseases of the liver and biliary system. The chapter covers neonatal hyperbilirubinemia, including unconjugated hyperbilirubinemia and hemolytic disease of the newborn; hepatitis and cholestatic syndromes (conjugated hyperbilirubinemia), including viral hepatitis, non-viral causes of hepatitis, urinary tract infections (UTIs), and neonatal hepatitis syndrome; infantile cholangiopathies, including biliary atresia, extra-hepatic (outside the liver) biliary atresia, Alagille's syndrome, prolonged parenteral nutrition, abnormal bile acid synthesis, genetic cholestatic syndromes, and symptomatic treatment of cholestatic syndromes; other causes of cholestatic jaundice; Reye's syndrome (and Reye-like syndrome); cirrhosis (liver scarring) in infancy and childhood, including Indian childhood cirrhosis and copper-associated liver disease); hepatic steatosis, including fetal alcohol syndrome and idiopathic steatohepatitis; and tumor of the liver, including hamartomas, mesenchymal hamartoma, malignant mesenchymoma (undifferentiated sarcoma), adenomas, hepato-cellular carcinoma (HCC), hepatoblastoma, infantile hemangio-endothelioma, nodular regenerative hyperplasia, and hepatic (liver) transplantation. 8 figures. 6 tables. 87 references.

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Liver Transplantation. In: Feldman, M.; Friedman, L.S.; Sleisenger, M.H. Sleisenger and Fordtran's Gastrointestinal and Liver Disease: Pathophysiology/Diagnosis/Management. 7th ed. [2-volume set]. St. Louis, MO: Saunders. 2002. p. 1620-1639.

Orthotopic liver transplantation (OLT) has evolved rapidly in the past two decades; the focus of management has shifted from prevention of rejection to the threat of disease recurrence, with potentially serious consequences for the allograft and patient. This chapter on OLT is from a comprehensive and authoritative textbook that covers disorders of the gastrointestinal tract, biliary tree, pancreas, and liver, as well as the related topics of nutrition and peritoneal disorders. Topics include indications; listing criteria and policies of the United Network for Organ Sharing (UNOS); absolute and relative contraindications; transplant evaluation and listing; disease-specific indications, for alcoholic liver disease, hepatitis B, hepatitis C, acute liver failure, cholestatic liver disease, hepatic malignancy (liver cancer), metabolic disorders, vascular disorders, and autoimmune hepatitis; surgical aspects of liver transplantation, including living donor liver transplantation; immunosuppression; postoperative course; and long-term patient care management, including general preventive medicine, immunizations and bacterial prophylaxis, when to contact the transplant center, and hepatic retransplantation. The chapter includes a mini-outline with page citations, full-color illustrations, and extensive references. 9 figures. 13 tables. 75 references.

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Liver Transplantation. Toronto, Ontario: Canadian Liver Foundation. 2000. 4 p.

A liver transplant is a life-giving operation that replaces a diseased liver with either a whole or portion of a healthy, donated liver. Livers are donated, either from individuals who are brain dead or from a living donor such as a relative or close friend. This brochure describes liver transplantation. Written in a question and answer format, the brochure discusses the diseases that are most commonly treated by liver transplant, patient selection considerations (patients with liver cancer, patients with alcohol-related liver disease), at what stage transplantation becomes appropriate, risk factors, success rates, postoperative care, the side effects of the anti-rejection medications, rejection, living liver donation, lifestyle changes after a liver transplantation, and problems with recurrence of the original disease in the transplanted liver. The brochure concludes with the contact information for the Canadian Liver Foundation (www.liver.ca).

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Liver Transplants: A Miracle of Modern Medicine. In: Everson, G.T.; Weinberg, H. Living with Hepatitis B: A Survivor's Guide. Long Island, NY: Hatherleigh Press. 2002. p.175-197.

Chronic hepatitis B can lead to cirrhosis (liver scarring), liver cancer, and the need for liver transplantation. This chapter on liver transplants is from a book that helps readers diagnosed with hepatitis B virus (HBV) infection educate themselves about the disease and its treatment. The authors first offer a brief history of liver transplantation, then discuss the indications for transplantation, denial of transplants, paying for transplants, the transplant team, waiting for a liver, the evaluation process, the waiting list, transplant support groups, liver transplant surgery, donor livers, the living donor liver transplant, the surgical procedure, the hospital stay, living with a new liver, medications to prevent rejection, managing complications, psychological transformation, survival rates, and how organs are allocated. Throughout the chapter the authors include quotes from real people who have gone through this process. The authors also include resource organizations that may offer additional support and information for readers. 1 reference.

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Living with Hepatitis B: A Survivor's Guide. Long Island, NY: Hatherleigh Press. 2002. 303 p.

Chronic hepatitis B can lead to cirrhosis (liver scarring), liver cancer, and the need for liver transplantation. This book helps readers diagnosed with hepatitis B virus (HBV) infection educate themselves about the disease and its treatment. Topics include the nature of the disease and its effects on the body, how to recognize early warning symptoms, what steps to take after learning one has been infected, the HBV vaccine (including safety and effectiveness), coping with the emotional effects of hepatitis B, how to avoid infecting others, how to understand blood test results, nutrition tips for a healthier liver, children with hepatitis B, treatment options, cutting medical costs, and research trends. Much of the information is presented in outline format for ease of understanding. Throughout the book are quotes from real people living with HBV infection. The book concludes with an extensive list of references and a detailed subject index.

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Management of Bleeding in the Cirrhotic Patient. Journal of Gastroenterology and Hepatology. 17(4): 355-360. April 2002.

Important advances have been made in the management of variceal (enlarged veins or arteries) bleeding. Despite these advances, bleeding in the patient with cirrhosis (liver scarring) remains one of the most demanding clinical challenges that a gastroenterologist or gastrointestinal surgeon may face. This article addresses the management of bleeding in the patient with cirrhosis. The aim of management is to identify the source of bleeding, control active bleeding, and prevent rebleeding. This requires a multidisciplinary team, and the optimal management algorithm depends on the clinical circumstance of the patient and the local availability of endoscopic, radiological, and surgical expertise. Injection sclerotherapy is effective in stopping acute variceal bleeding, but has the drawback of a high incidence of complications. Endoscopic variceal ligation is just as effective, and is associated with fewer complications. To prevent rebleeding, beta-blockers are recommended for all patients with large varices (including those which have never bled). Injection sclerotherapy or band ligation, conducted at weekly intervals after the initial control of bleeding, is equally effective at obliterating varices and decreasing the risk of further hemorrhage; band ligation results in fewer complications. Other newer treatment options for variceal bleeding, such as somatostatin analogs, transjugular intrahepatic portosystemic shunt and liver transplantation, offer more optimal approaches to control bleeding and prevent rebleeding, but may be prohibitively expensive. Even for the most affluent communities, affordability, cost-effectiveness, and resource rationing are important considerations in management of patients with cirrhosis complicated by gastrointestinal bleeding. 3 figures. 59 references.

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Management of Refractory Ascites and Hepatorenal Syndrome. Journal of Gastroenterology and Hepatology. 17(4): 456-461. April 2002.

Refractory ascites (fluid accumulation in the abdominal cavity) and hepatorenal syndrome (HRS, a type of kidney failure associated with hepatitis or cirrhosis of the liver) are the late complications of the terminal stages of cirrhosis (liver scarring). This article discusses the management of refractory (resistant to treatment) ascites and HRS. The definitions of refractory ascites and HRS proposed by the International Ascites Club in 1996 are now widely accepted, and are useful in diagnosis, treatment and research in this field. In both conditions, the only treatment of proven value for improved survival is liver transplantation. However, because of better understanding about the pathophysiology of HRS, including the roles of portal hypertension, ascites formation, and hemodynamic derangements, treatments such as transjugular intrahepatic portosystemic shunt (TIPS) and new pharmacological agents may be considered to alleviate the problem prior to transplantation. Symptomatic treatment of refractory ascites includes TIPS and repeated large volume paracentesis (fluid removal). TIPS can improve survival while waiting for liver transplantation. Practical management guidelines for TIPS and large volume paracentesis, including the prevention and management of further complications, are considered in this review. 30 references.

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Nonalcoholic Fatty Liver Disease. New England Journal of Medicine. 346(16): 1221-1231. April 18, 2002.

Nonalcoholic fatty liver disease (NAFL) is an increasingly recognized condition that may progress to end stage liver disease. This review article considers the epidemiology, clinical manifestations (symptoms), pathogenesis (development), diagnosis, natural history, and management of NAFL. Insulin resistance and oxidative stress have critical roles in the pathogenesis of NAFL. Liver biopsy remains the most sensitive and specific means of providing important prognostic information. Simple steatosis may have the best prognosis within the spectrum of NALF, but it has the potential to progress to Steatohepatitis, fibrosis, and even cirrhosis (scarring of the liver). No effective medical therapy is currently available for all patients with nonalcoholic fatty liver disease. Weight reduction, when achieved and sustained, may improve the liver disease. Drug therapy aimed at the underlying liver disease holds promise, however, questions remain regarding the use of drug therapy and the effect of recommended dietary measures. Liver transplantation is a therapeutic alternative for some patients with decompensated, end stage NALF disease, but NALF may recur or develop after liver transplantation. 5 figures. 3 tables. 95 references.

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Obteniendo un Nuevo Higado: Informacion Acerca del Transplante de Higado. [Getting a New Liver: Facts About Liver Transplants). Laurel, NJ: American Society for Transplantation. 2002. 15 p.

This Spanish language booklet provides information for patients about to undergo liver transplantation. The booklet is designed to help patients understand the process and to become a more informed member of their own health care team. The document first lists facts about the liver and its role in the body, then covers pretransplant evaluation (including diagnostic tests), the waiting period, the transplant admission, living-donor transplantation, and pediatric liver transplantation. Step-by-step details are provided in non-technical language to help readers understand each procedure and what to expect. Throughout the booklet, readers are encouraged to take a proactive role in preventing and treating complications and thus help to ensure a healthy transplant. Simple graphics illustrate the booklet. The contact information for the American Society of Transplantation, Patient Care and Education Committee, is provided (ast@ahint.com). 1 figure.

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Molecular Biology and Immunology in Hepatology: Advances in the Treatment of Intractable Liver Diseases. St. Louis, MO: Elsevier Science. 2002. 362 p.

This text book offers 25 chapters on intractable liver diseases. Based on the editor's choice of important topics in hepatology, the book ranges from clinical research to basic research, including animal models. Topics include molecular biology and immunology; mechanisms of liver injury in hepatitis B virus (HBV) infection; genetic diversity and pathophysiology of HBV; immunopathogenesis of hepatitis C; interferon therapy for hepatitis C; new therapeutic strategies for chronic hepatitis C; transgenic mouse models for viral hepatitis and the role of hepatitis viruses in the development of liver cancer; gene therapy of viral hepatitis; the reversibility of liver cirrhosis; treatment of liver cancer (hepatocellular carcinoma); gene expression profiles in liver cancer; gene therapy for liver cancer; new immunological treatments for liver cancer; assessment of the reversibility and treatments of alcoholic liver disease; molecular mechanisms of autoimmune hepatitis; roles of hepatitis C virus (HCV) in autoimmune hepatitis; molecular mechanisms of T cell responses of autoimmune hepatitis; primary biliary cirrhosis; overlap syndrome; etiology and pathophysiology of fulminant hepatic failure; cytokines and fulminant hepatic failure; treatment and prognosis of fulminant hepatic failure; living related liver transplantation; viral cirrhosis and liver cancer in relation to living donor liver transplants. Each chapter concludes with references; the text concludes with a subject index.

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Non-alcoholic Fatty Liver Disease. Journal of Gastroenterology and Hepatology. 17 (Supplement): S186-S190. February 2002.

This review article considers nonalcoholic fatty liver disease (NAFLD), a chronic liver disease that affects a high proportion of the world's population. Insulin resistance and oxidative stress play a critical role in the pathogenesis (development) of NAFLD. Clinical, biochemical and imaging studies are of value in the diagnostic evaluation of patients with NAFLD, but liver biopsy remains the most sensitive and specific means of providing important diagnostic and prognostic information. Simple steatosis (fatty liver) has the best prognosis within the spectrum of NAFLD, but NAFLD has the potential to progress to steatohepatitis, fibrosis, and even cirrhosis (liver scarring). No effective medical therapy is currently available for all patients with NAFLD. In patients with diabetes mellitus and hyperlipidemia (high levels of blood fats), appropriate metabolic control is always recommended, but rarely effective in resolving the liver disease. Weight reduction, when achieved and sustained, may improve the liver disease, although the results with weight loss have been inconsistent. Drug therapy aimed at the underlying liver disease holds promise. Several medications with different mechanisms of action and potential benefit are currently being evaluated in clinical trials. Liver transplantation is a life-extending treatment choice for patients with end stage NAFLD, but NAFLD may recur after liver transplantation. 43 references.

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Primary Biliary Cirrhosis. In: Feldman, M.; Friedman, L.S.; Sleisenger, M.H. Sleisenger and Fordtran's Gastrointestinal and Liver Disease: Pathophysiology/Diagnosis/Management. 7th ed. [2-volume set]. St. Louis, MO: Saunders. 2002. p. 1474-1485.

Primary biliary cirrhosis (PBC) is an autoimmune liver disease that generally affects middle-aged women and that is characterized by ongoing inflammatory destruction of the bile ducts. This destruction leads to chronic cholestasis (lack of bile flow) and biliary cirrhosis, with consequent complications such as portal hypertension and liver failure. This chapter on PBC is from a comprehensive and authoritative textbook that covers disorders of the gastrointestinal tract, biliary tree, pancreas, and liver, as well as the related topics of nutrition and peritoneal disorders. Topics include epidemiology; pathogenesis; clinical features (symptoms); diagnosis; natural history; medical treatment; complications of chronic cholestasis and their management, including bone disease, fat-soluble vitamin deficiency, hypercholesterolemia, hyperlipidemia, pruritus (itching), and steatorrhea; liver transplantation; and autoimmune cholangitis or antimitochondrial antibody-negative PBC. The chapter includes a mini-outline with page citations, full-color illustrations, and extensive references. 2 figures. 4 tables. 95 references.

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Research Trends: Hope for the Future. In: Everson, G.T.; Weinberg, H. Living with Hepatitis B: A Survivor's Guide. Long Island, NY: Hatherleigh Press. 2002. p.253-265.

Chronic hepatitis B can lead to cirrhosis (liver scarring), liver cancer, and the need for liver transplantation. This chapter on research trends is from a book that helps readers diagnosed with hepatitis B virus (HBV) infection educate themselves about the disease and its treatment. The authors first describe the differences between clinical research and basic research, then discuss the phases of clinical trials, testing new drugs, deciding whether or not to sign up for a clinical drug study, current clinical research for the treatment of hepatitis B (including interferon alfa, long acting interferons, and thymosin-alpha1), potential new therapies, standard vaccines, vaccines for the treatment of chronic hepatitis B, nucleoside and nucleotide analogues (adefovir, entecavir, emtricitabine), ribozymes, research funding, and basic research in the areas of molecular virology, cell biology, liver cell transplantation, stem cells, pathophysiology, pharmacology, and the bioartificial liver.

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Treatment and Prognosis of Fulminant Hepatic Failure. In: Tsuji, T., et al, eds. Molecular Biology and Immunology in Hepatology. St. Louis, MO: Elsevier Science. 2002. p. 293-304.

The term fulminant hepatic failure (FHF) is commonly used in Western countries to indicate acute severe liver disease. This term implies acute liver diseases complicated by coma within 8 weeks after onset of the first symptoms. The term fulminant hepatitis (FH) is more often used in Japan. This chapter on the treatment and prognosis of fulminant liver failure is from a text book on the pathogenesis and treatment of intractable liver diseases. The author first outlines the differences between FHF and FH, noting that recognizing these differences in pathogenesis (how the disease develops) is of primary importance in the development of a reliable medical treatment. Topics include symptomatic treatment of FHF, the research into the artificial liver, treatment of underlying liver diseases, liver transplantation, and prognostic considerations. 2 figures. 3 tables. 33 references.

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Sleisenger and Fordtran's Gastrointestinal and Liver Disease: Pathophysiology/Diagnosis/Management. 7th ed. St. Louis, MO: Saunders. 2002. (CD-ROM).

This CD-ROM is an interactive version of a definitive compendium of current clinical knowledge in gastroenterology and hepatology. The authors offer a balanced, detailed account of the basic science of the digestive system, as well as complete coverage of current diagnosis and management. Readers will find up-to-date discussions of the cell biology and molecular biology that determine the digestive system's function, in addition to descriptions of organ physiology and the pathophysiology of the signs, symptoms and laboratory abnormalities of organ disease. The program contains an expanded section on the liver, integrates the latest endoscopic scanning and therapeutic techniques, discusses the latest perspective in possible roles of H.pylori in dyspepsia and gastric cancer, contains information on possible causes of non-ulcer dyspepsia, presents expanded coverage of GI bleeding, with description of the latest techniques for diagnosis and treatment, contains new information on genetic markers for cancer of the colon, post liver transplantation for hepatitis and diagnostic tests for celiac disease, offers a complete description of effective responses for inflammatory bowel disease and Crohn's disease, and discusses relevant histological criteria for the diagnosis of Barrett's esophagus, gastritis, IBD and celiac disease. The program features a separate section on problems involving multiple organs such as AIDS, systemic manifestation of GI disease and abdominal pain and bleeding. The program is illustrated with full-color photographs and drawings.

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Split Liver Transplantation and Risk to the Adult Recipient: Analysis Using Matched Pairs. Journal of the American College of Surgeons. 195(5): 648-657. November 2002.

The technique of liver splitting is an effective way of increasing the donor pool and reducing pediatric waiting list mortality (death rates). But the procedure is still not fully accepted because of concerns that it may cause complications in adult recipients. This article reports on a study of 59 adult recipients of primary extended right split liver transplantations (SLTs) who were matched to recipients of whole liver transplantations (WLTs). Fifteen percent of the recipients in the study were highly urgent cases (UNOS 1) and 85 percent were UNOS status 3 to 4. The 3 month and 12 month patient survival rates after SLT and WLT were 82.5 percent and 77.1 percent, and 92.5 percent and 87.5 percent, respectively. The 3 month and 12 month graft survival rates showed no significant difference in either group. The rates of primary nonfunction, primary poor function, biliary and vascular complications, intra and postoperative blood transfusion, and intensive care stay were comparable for SLT and WLT. The authors conclude that SLT, using the extended right hepatic (liver) lobe, does not notably differ from WLT with regard to initial graft function, postoperative complications, or patient and graft survival. Based on this, the liver can be considered a paired organ, and mandatory splitting of good quality livers can be recommended. 4 figures. 5 tables. 42 references.

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Taking Care of Yourself Emotionally: Emotional Challenges of Chronic Illness. In: Everson, G.T.; Weinberg, H. Living with Hepatitis B: A Survivor's Guide. Long Island, NY: Hatherleigh Press. 2002. p.99-117.

Chronic hepatitis B can lead to cirrhosis (liver scarring), liver cancer, and the need for liver transplantation. This chapter on the emotional challenges of chronic illness is from a book that helps readers diagnosed with hepatitis B virus (HBV) infection educate themselves about the disease and its treatment. The authors draw on the expertise of mental health professionals who work with hepatitis patients, as well as on the experiences of the patients themselves. The authors discuss diagnosis, attitudes and expectations, reorganization, healing versus curing, the warning signs of depression, understanding family systems, and some practical suggestions for wellness, including using medical care and psychological help, including exercise and nutrition, feeling useful, having fun, and exploring creative and spiritual sides of oneself. Throughout the chapter the authors include quotes from real people who are living with hepatitis. The authors also include resource organizations that may offer additional support and information for readers. 1 reference.

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Taking Care of Yourself Financially: An Overview. In: Everson, G.T.; Weinberg, H. Living with Hepatitis B: A Survivor's Guide. Long Island, NY: Hatherleigh Press. 2002. p.118-138.

Chronic hepatitis B can lead to cirrhosis (liver scarring), liver cancer, and the need for liver transplantation. This chapter on the financial challenges of chronic illness is from a book that helps readers diagnosed with hepatitis B virus (HBV) infection educate themselves about the disease and its treatment. The authors present a general overview of financial issues and include resources to help patients find their own solutions. Topics include costs of treatment, such as ongoing medical care, antiviral treatment, and liver transplantation; private health insurance, including selecting health insurance and the different types of private insurance; government health insurance, including Medicare, Medicaid, and Veterans Administration programs; applying for disability; and disability insurance, including Social Security Disability Insurance (SSDI) and Supplemental Security Income (SSI). Throughout the chapter the authors include quotes from real people who are living with hepatitis. The authors also include resource organizations that may offer additional support and information for readers.

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Taking Care of Yourself Nutritionally: Guidelines for Healthy Nutrition in Liver Disease. In: Everson, G.T.; Weinberg, H. Living with Hepatitis B: A Survivor's Guide. Long sland, NY: Hatherleigh Press. 2002. p.78-98.

Chronic hepatitis B can lead to cirrhosis (liver scarring), liver cancer, and the need for liver transplantation. This chapter on nutrition is from a book that helps readers diagnosed with hepatitis B virus (HBV) infection educate themselves about the disease and its treatment. The authors discuss some general nutritional concepts, outline what happens with liver disease affects nutrition, and address some specific deficiencies and their treatments. Topics include ideal body weight, a normal diet, carbohydrate metabolism, protein metabolism, fat metabolism, bile, vitamins, and nutritional needs for hepatitis B patients with and without cirrhosis. Tips are offered in the areas of caloric requirements, vitamin supplements, nutritional (herbal) therapies, herbs that are harmful to the liver, and salt and fluid restriction. Throughout the chapter the authors include quotes from real people who are living with hepatitis. 1 figure. 2 tables. 7 references.

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Treatment for Hepatitis B: Interferon, Lamivudine, and Adefovir. In: Everson, G.T.; Weinberg, H. Living with Hepatitis B: A Survivor's Guide. Long Island, NY: Hatherleigh Press. 2002. p.139-174.

Chronic hepatitis B can lead to cirrhosis (liver scarring), liver cancer, and the need for liver transplantation. This chapter on drug therapy is from a book that helps readers diagnosed with hepatitis B virus (HBV) infection educate themselves about the disease and its treatment. The authors first offer an overview of general guidelines for patients with acute hepatitis B, chronic hepatitis B, and monitoring issues for patients with chronic infection. The authors then discuss drug therapy, including interferon, lamivudine, thymosin-alpha1 (Zadazin), famciclovir (Famvir), ganciclovir (Cytovene), and adefovir dipovoxil. For each drug, the authors describe the therapy, consider administration and dosage issues, and address side effects that may be encountered. The chapter concludes with information about nonresponders, the hepatitis B vaccine, the hepatitis A vaccine, and hepatitis B immune globulin (HBIG). Throughout the chapter the authors include quotes from real people who are living with hepatitis. 6 figures. 1 reference.

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Treatment of Chronic Hepatitis B in Transplant Recipients. In: Gordon, S.C. Management of Chronic Viral Hepatitis. New York, NY: Marcel Dekker Inc. 2002. p. 119-157.

This chapter on the treatment of hepatitis B in transplant recipients is from a monograph on the management of chronic viral hepatitis (liver inflammation), bringing the advances of clinical and basic research into the doctor's office. Three clinical case presentations address the real life intricacies of managing patients who present with viral hepatitis. Originally, a high rate of graft infection and early graft failure, as well as frequent patient death, led to skepticism about the use of orthotopic liver transplantation (OLT) for chronic HBV infection. Marked improvements in long term survival have resulted from the use of low dose immunosuppressive therapy and administration of indefinite hepatitis B immune globulin. The goals of treatment of HBV infection include decreasing viral load, lessening infectivity, decreasing the level of hepatic inflammation, preventing or slowing the development of cirrhosis (liver scarring) and liver failure, delaying time to liver transplantation, and prevention of liver cancer. Lamivudine alone or in combination with hepatitis B immune globulin (HBIG) has been shown to be effective in the long term management of recipients of liver transplants. The combination of lamivudine and HBIG is probably the best current therapy, since lamivudine monotherapy, especially in the setting of immunosuppression, has a very high mutation rate leading to resistance and active viral replication in more than 40 percent of patients after 2 years of therapy post-transplantation. Patients with severe recurrent HBV infection, including fibrosing cholestatic hepatitis (FCH), can undergo liver retransplantation but costs are extremely high and long term survival is inferior to that of patients undergoing a first liver transplantation. 2 figures. 3 tables. 165 references.

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Treatment of Chronic Hepatitis C: Transplant Recipients. In: Gordon, S.C. Management of Chronic Viral Hepatitis. New York, NY: Marcel Dekker Inc. 2002. p. 263-293.

This chapter on the treatment of chronic hepatitis C in transplant recipients is from a monograph on the management of chronic viral hepatitis (liver inflammation), bringing the advances of clinical and basic research into the doctor's office. This chapter uses a clinical case presentation in order to address the real life intricacies of managing patients who present with viral hepatitis. The case patient was a 45 year old female who presented with chronic hepatitis C with cirrhosis (liver scarring). She was referred for liver transplantation 1 year after diagnosis, due to deterioration of her condition. Although her hepatitis recurred after transplant and contributed to three serious episodes of rejection, the patient is now clinically stable. Recurrent hepatitis occurs in 40 to 80 percent of patients; it is usually found during the first year posttransplant. Recurrence of HCV infection leads to cirrhosis in 10 percent of patients 5 years after transplant. The clinical presentation of HCV recurrence and acute graft infection after transplantation varies from asymptomatic or minimal liver damage to an influenza like syndrome with or without jaundice. Risk factors involved in a more severe recurrence are still being studied. Immunosuppressive medications result in increased viral proliferation and a worse outcome of hepatitis C recurrence. Histological features are important, but can be confusing in differentiating HCV recurrence from other post transplantation complications such as acute rejection. Combination therapy with inferon and ribavirin may lead to an improved liver histology in patients with severe recurrence, but few patients have a sustained viral response and further advances in the management of this group of patients are required. 1 figure. 4 tables. 107 references.

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Treatment of Hepatitis D. In: Gordon, S.C. Management of Chronic Viral Hepatitis. New York, NY: Marcel Dekker Inc. 2002. p. 319-345.

This chapter on the treatment of hepatitis D is from a monograph on the management of chronic viral hepatitis (liver inflammation), bringing the advances of clinical and basic research into the doctor's office. Three brief clinical case presentations in order to address the real life intricacies of managing patients who present with viral hepatitis. The hepatitis delta agent (hepatitis D virus, HDV) is an incomplete defective RNA virus with a replicative cycle dependent on a helper function of another virus (hepatitis B virus, HBV). The issue of clinically effective etiological therapy for HDV hepatitis is still open. Patients with HDV may have severe liver disease with the early appearance of cirrhosis (liver scarring) but they often have a disease course indistinguishable from that of chronic HBV infection. HDV infection is acquired by two different modalities: as a co infection (simultaneous exposure of an HBV nonimmune subject to HBV and HDV) and as a superinfection (exposure to HDV of a chronic HBV carrier). Chronic HDV infection causes a severe chronic hepatitis with a rapid and relentless progressive course to cirrhosis and liver failure. Treatment of chronic hepatitis D has been disappointing thus far. Treatments can include nonantiviral treatments, interferons, nucleoside analogues (e.g., lamivudine), and liver transplantation. 61 references.

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Treatment of Patients with Hepatitis C and Cirrhosis. Hepatology. 36(5 Supplemental 1): S185-S194. November 2002.

This article offers guidelines for the treatment of patients with hepatitis C and cirrhosis (scarring of the liver). The author notes that determining recommendations for this patient population is difficult. Few prospective studies have focused on the treatment of patients with advanced disease, and response rates appear to be lower and serious side effects more frequent in patients with cirrhosis. In patients with compensated cirrhosis, combination therapy with interferon alfa and ribavirin results in a sustained virological response (SVR) in 33 to 41 percent of patients. Responses to combination therapy are not significantly higher using peginterferon alfa 1a or 2b, compared with standard interferon. In using peginterferon in combination therapy, the benefits of once weekly dosing need to be weighed against the higher risks of cytopenias and greater costs with the pegylated formulations. Combination therapy results in some degree of histological improvement even in patients who are virological non-responders. These findings provide the scientific basis for ongoing studies of maintenance therapy with peginterferon to prevent complications of cirrhosis in nonresponders patients with hepatitis C. Recommendations for the management of decompensated cirrhosis and of recurrent hepatitis C after liver transplantation are difficult because of limitations of data, most of which are derived from uncontrolled case series. Combination therapy is poorly tolerated in both groups and rates of response are low. Thus, while the medical need is great, treatment of patients with decompensated cirrhosis or with recurrent hepatitis C after transplantation should be undertaken cautiously and only within the confines of prospective clinical trials. 2 figures. 1 table. 16 references.

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Viral Hepatitis. In: Edmundowicz, S.A., ed. 20 Common Problems in Gastroenterology. New York, NY: McGraw-Hill, Inc. 2002. p. 219-232.

Viral hepatitis (inflammation of the liver) is the most common cause of chronic liver disease, cirrhosis (scarring), and hepatocellular carcinoma (HCC, liver cancer) in the United States. This chapter on viral hepatitis is from a book that focuses on the most common gastroenterological problems encountered in a primary practice setting. The chapter is organized to support rapid access to the information necessary to evaluate and treat most patients with this problems. Topics include incidence and background; an overview of the hepatotropic viruses (hepatitis A, B, C, D and E) and the clinical syndromes, including acute viral hepatitis, fulminant hepatic failure, chronic viral hepatitis, end stage liver disease, and HCC; key history, notably risk factors for the subvarieties of hepatitis; the physical examination and ancillary tests, including routine blood tests, serologic evaluation, imaging, and liver biopsy; treatment options, including prevention of the hepatitis A, B, and C viruses, prophylaxis of exposed individuals, vaccination, antiviral therapy, and liver transplantation; patient education; common errors in diagnosis and treatment; and controversies, including HIV coinfection. The chapter includes an outline for quick reference, the text itself, a diagnostic and treatment algorithm, and selected references. 5 figures. 2 tables. 21 references.

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What is Autoimmune Hepatitis?. Cedar Grove, NJ: American Liver Foundation. 2002. [2 p.].

This brochure describes autoimmune hepatitis, a disease of young women that is associated with increased gamma globulin in the blood and chronic hepatitis (liver inflammation) on liver biopsy. The brochure summarizes the symptoms of autoimmune hepatitis, which can include fatigue, abdominal discomfort, aching joints, itching, jaundice, enlarged liver, and spider angiomas (blood vessels) on the skin. A liver biopsy is important to confirm the diagnosis and provide a prognosis. The remainder of the brochure outlines treatment options, including anticipated prognosis. The treatment of autoimmune hepatitis is immunosuppression with prednisone alone or prednisone and azathioprine. However, the majority of patients relapse within six months after therapy is ended. Therefore, most patients need long-term maintenance therapy. For patients who do not respond to medical therapy, liver transplantation can be considered. The brochure concludes with a description of the work of the American Liver Foundation (ALF) and its contact information (www.liverfoundation.org).

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What is Hepatitis B?: An Introduction. In: Everson, G.T.; Weinberg, H. Living with Hepatitis B: A Survivor's Guide. Long Island, NY: Hatherleigh Press. 2002. p.1-17.

Chronic hepatitis B can lead to cirrhosis (liver scarring), liver cancer, and the need for liver transplantation. This introductory chapter is from a book that helps readers diagnosed with hepatitis B virus (HBV) infection educate themselves about the disease and its treatment. The authors discuss some basic facts and statistics about hepatitis B, the history and discovery of the hepatitis B virus and vaccines, and information about viruses in general and other forms of viral hepatitis. Throughout the chapter the authors include quotes from real people who are living with hepatitis. 1 figure. 1 reference.

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When You Have Hepatitis B: Understanding the Diagnosis: Blood Tests and Biopsies. In: Everson, G.T.; Weinberg, H. Living with Hepatitis B: A Survivor's Guide. Long Island, NY: Hatherleigh Press. 2002. p.18-37.

Chronic hepatitis B can lead to cirrhosis (liver scarring), liver cancer, and the need for liver transplantation. This chapter on diagnostic tests is from a book that helps readers diagnosed with hepatitis B virus (HBV) infection educate themselves about the disease and its treatment. The authors answer questions about the testing process for hepatitis B, from diagnosis through monitoring during the years of ongoing care. The chapter covers hepatitis B virus tests, including proteins, antigens, and antibodies; liver imaging tests, including ultrasound, computed tomography, and magnetic resonance imaging (MRI); liver biopsy, including the procedure used and how to interpret the results obtained; liver blood tests, including liver enzymes, bilirubin, albumin, clotting factors, alpha-fetoprotein, and complete blood count (CBC); and patterns of hepatitis B tests in patients, including for acute and chronic disease, and for chronic carriers. Throughout the chapter the authors include quotes from real people who are living with hepatitis. 6 figures. 3 tables.

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Why Me? What About Them?: How You Got Infected and How to Avoid Infecting Others. In: Everson, G.T.; Weinberg, H. Living with Hepatitis B: A Survivor's Guide. Long Island, NY: Hatherleigh Press. 2002. p.38-52.

Chronic hepatitis B can lead to cirrhosis (liver scarring), liver cancer, and the need for liver transplantation. This chapter on transmission is from a book that helps readers diagnosed with hepatitis B virus (HBV) infection educate themselves about the disease and its treatment. The authors first offer an overview of how infectious the hepatitis B virus is, then summarize documented ways that hepatitis B is transmitted, including: intravenous drug abuse, sexual transmission, childbirth and delivery, immigrants and travelers from countries with high rates of HBV, transfusion of blood or blood products (for surgery or medical treatment and for hemophilia), needle-stick accidents, tattooing and body piercing, household contact, institutional contact, and organ transplantation. The chapter concludes with a section on how to prevent transmitting the disease. Throughout the chapter the authors include quotes from real people who are living with hepatitis. 1 figure. 1 reference.

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Acute Liver Failure. Journal of Clinical Gastroenterology. 33(3): 191-198. 2001.

Acute liver failure is defined as hepatic encephalopathy (a brain manifestation of extensive liver damage) complicating acute liver injury. This article reviews the definitions, etiologies, prognostic factors, and issues in the management of patients with acute liver failure (ALF). The most common etiologies (causes) are acute viral hepatitis A and B, medication overdose (e.g., acetaminophen), idiosyncratic drugs reactions, ingestion of other toxins (e.g., amanita mushroom poisoning), and metabolic disorders (e.g., Reye's syndrome). Despite advances in intensive care management, mortality (death) continues to be high (40 to 80 percent) and is partly related to ALF's complications, such as cerebral edema (fluid accumulation), sepsis, hypoglycemia (low blood glucose), gastrointestinal bleeding, and acute renal (kidney) failure. Several prognostic models have been developed to determine which patients will spontaneously recover. Treatment is directed at early recognition of the complications and general supportive measures. The only proven therapy for those who are unlikely to recover is liver transplantation. Therefore, recognition of ALF is paramount, and urgent referral to a transplant center is critical to assess transplantation status. 2 figures. 5 tables. 62 references.

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Biliary Atresia. Toronto, Ontario: Canadian Liver Foundation. 2001. 2 p.

This brochure describes biliary atresia, a condition in infants in which the bile duct outside the liver that carries bile from the liver to the small intestine is damaged. This prevents bile from leaving the liver so it accumulates and causes progressive damage to the liver tissue. In addition, there is ongoing damage to smaller bile ducts inside the liver. Unless bile flow can be established, liver function is gradually lost and affected children rarely survive beyond two years. The brochure describes the role of bile, the causes of the disease, the typical symptoms, treatment strategies (a surgery called the Kasai procedure is usually the first option), secondary treatment options, complications of the disease, and the indications for liver transplantation in children with biliary atresia. The brochure concludes with the contact information of the Canadian Liver Foundation (www.liver.ca).

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Bleeding Esophageal Varices: How to Treat This Dreaded Complication of Portal Hypertension. Postgraduate Medicine. 109(2): 75-76, 81-86, 89. February 2001.

Bleeding esophageal varices, one of the most feared complications of portal hypertension (high blood pressure in the liver venous system), contribute to the estimated 32,000 deaths annually attributed to cirrhosis (liver scarring). This article describes the care of patients with this complication. The authors stress that successful control requires knowledge of the pertinent anatomy, underlying pathophysiology of portal hypertension, and natural history of gastroesophageal varices. The authors discuss the various prophylactic (preventive) and therapeutic approaches to management, including pharmacologic agents (drug therapy), endoscopic sclerotherapy, and transjugular intrahepatic portosystemic shunt (TIPS). Nonselective beta blockers are the treatment of choice for prevention of the first bleeding episode. Active bleeding is managed with octreotide and endoscopic sclerotherapy. Goals in the management of active bleeding are hemodynamic resuscitation, prevention and treatment of complications, and control of bleeding. Complications related to bleeding or its treatment can substantially increase the risk of death in each episode. TIPS and shunt surgery are reserved for those in whom octreotide and endoscopic surgery have failed. Endoscopic band ligation (tying off) should be used for prevention of recurrent bleeding. If endoscopic band ligation fails, patients can be offered TIPS or surgical therapy; they should be evaluated for liver transplantation. 4 figures. 4 tables. 11 references.

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Current Therapy of Chronic Hepatitis C Infection. Practical Gastroenterology. 25(7): 14, 16, 19-21, 25. July 2001.

Hepatitis C virus (HCV) is a major cause of end stage liver disease worldwide and a leading indication for liver transplantation. This review article considers the current treatments for chronic CHV which can eradicate the virus and improve liver histology in certain patients. The current most efficacious treatment for patients with HCV is interferon combined with ribavirin, which eradicates the virus long term in 35 to 40 percent of treated patients. For those in whom ribavirin is contraindicated, interferon alone may be used with successful viral eradication in approximately 15 percent of patients. The authors discuss the current recommended initial treatment, indications and contraindications to therapy, workup and monitoring of patients during and after therapy, the side effects associated with treatment, the predictors of response, and the therapy response. The authors conclude by discussing potential future treatment regimens that may lead to improved efficacy in the management of chronic HCV patients. 1 figure. 4 tables. 25 references.

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Cholangiocarcinoma in the Inflammatory Bowel Disease Patient. In: Bayless, T.M. and Hanauer, S.B. Advanced Therapy of Inflammatory Bowel Disease. Hamilton, Ontario: B.C. Decker Inc. 2001. p. 311-315.

This chapter on cholangiocarcinoma (biliary tract cancer) is from the second edition of a book devoted to the details of medical, surgical, and supportive management of patients with Crohn's disease (CD) and ulcerative colitis (UC), together known as inflammatory bowel disease (IBD). Cholangiocarcinoma (CC) is a dreaded complication of PSC due to its poor prognosis (median survival of 5 months) and limited treatment options. In the general population, CCs are rare tumors but occur in 5 to 15 percent of patients with primary sclerosing cholangitis (PSC). The major risk factor for CC development is chronicity of PSC. This duration of disease is somewhat difficult to prove as the precise onset of PSC is often uncertain. Most CC patients have already developed cirrhosis and have longstanding Ulcerative Colitis (UC). Its detection in the setting of PSC necessitates a high index of clinical suspicion in addition to serial assess-ments of serum biliary biochemistry and possible imaging with CT scan of the abdomen and ERCP with tissue sampling of dominant strictures. Early detection of potentially curable CC remains the challenge, and no proven technique exists. Serum IL-6 concentration and PET scanning may prove to be helpful in this regard. The only definitive treatment is surgical resection as the prognosis following liver transplantation remains unacceptably poor. Neoadjuvant therapy with chemoradiation may offer some benefit in reducing tumor bulk and increasing the proportion of patients suitable for curative resection. Palliative therapy aims to relieve the symptoms of biliary obstruction and optimize quality of life. Endoscopic stricture dilation and biliary stent placement is possible in the majority of patients and highly successful in effecting biliary decompression. A multidisciplinary approach optimizes the treatment strategy in the management of patients with CC.

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Cholestatic Liver Disease. Practical Gastroenterology. 25(1): 28, 30, 32-35. January 2001.

Cholestatic liver disease remains a rare but increasingly recognized cause of illness affecting adults worldwide. Primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) are the two major cholestatic liver diseases. This article provides an overview of adult cholestatic liver disease and the options available to clinicians in the management of these patients. The author discusses epidemiology, pathologic features, clinical features, diagnosis, medical therapy, management of complications, and prognosis of each disease. While there is much current evidence supporting an underlying immunologic process for both PBC and PSC, no unifying hypothesis exists to allow for the development of target specific therapies. Because of advances in technology and the refinement of diagnostic modalities, an increasing number of individuals are being identified at earlier stages of disease who may benefit from existing treatment options. In the majority of patients with PBC, a progressive clinical course resulting in fibrosis and eventual cirrhosis (scarring) is observed. Pruritus (itching) as a frequently complication creates difficult management options as significant impairments in quality of life are observed. Individuals undergoing orthotopic liver transplantation (OLT) for PBC have the highest rates of graft and patient survival over 5 years. Disease progression in PSC as a result of chronic bile duct obliteration eventually leads to biliary cirrhosis, hepatic (liver) failure, and complications from portal hypertension. Despite improvements in the management of complications from end stage liver disease and excellent long term results from liver transplantation, a continued understanding of the clinicopathologic processes responsible for cholestatic liver disease remains important. 23 references.

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Your Liver, Your Health. Toronto, Ontario: Canadian Liver Foundation. 2001. 4 p.

Virtually all the blood returning from the intestinal tract to the heart passes through the liver. This means that all foods and compounds that are swallowed that are absorbed into the bloodstream pass through the liver. This brochure describes the role of the liver and the common diseases that can affect the liver. Topics include the physiology of the liver; who is affected by liver disease; common liver diseases including gallstones, viral hepatitis, hepatitis A, hepatitis B, hepatitis C, cirrhosis (scarring of the liver), liver cancer, liver disease in children, autoimmune hepatitis, alcohol-related liver disease, hemochromatosis, and primary biliary cirrhosis (PBC); the symptoms and signs of liver disease; strategies for taking care of one's liver; and the use of liver transplantation. The brochure concludes with a brief description of the goals and activities of the Canadian Liver Foundation (www.liver.ca).

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Severe Liver Injury After Treatment with the Leukotriene Receptor Antagonist Zafirlukast. Annals of Internal Medicine. 133(12): 964-968. December 19, 2000.

In registration trials, zafirlukast (Accolate, Zeneca Pharmaceuticals), an asthma medication, caused asymptomatic elevated aminotransferase levels in up to 5 percent of participants. However, no cases of severe hepatitis attributed to zafirlukast have been reported to date. This article reports the clinical characteristics of three patients with severe hepatitis due to zafirlukast. The three middle aged women patients were taking zafirlukast, 20 milligrams twice per day. The interventions consisted of discontinuation of zafirkulast therapy in all three patients, steroid therapy in two patients, and orthotopic liver transplantation in one patient. Patient 1 recovered spontaneously, had a severe relapse after inadvertent rechallenge with the medication, and ultimately made a complete recovery. Patient 2 developed subfulminant hepatic failure and required liver transplantation. Patient 3 developed severe hepatitis that improved after treatment with corticosteroids. Liver tissue was available from two patients and showed histologic changes commonly associated with drug reactions. The authors conclude that patients receiving zafirkulast may develop severe liver injury and should be observed for signs and symptoms of hepatitis. 1 figure. 1 table. 2 references.

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Chronic Hepatitis C: Implications for Health-Related Quality of Life. Gastroenterology Nursing. 24(4): 169-175. 2001.

Hepatitis C viral (HCV) infection with its sequelae is a significant health care problem. Hepatitis C infects nearly 4 million Americans with almost half of these people unaware of their infection. Many of those individuals infected with hepatitis C develop chronic hepatitis C and in 15 percent of these patients, the infection will progress to cirrhosis (liver scarring) within 20 years. Several cross sectional and longitudinal studies have demonstrated the negative impact of chronic hepatitis C on health related quality of life (HRQOL). This review article describes what is currently known about the impact of chronic hepatitis C on health related quality of life during pharmacologic treatment and after liver transplantation. It is important to note that few studies have prospectively followed patients over time with respect to quality of life or examined other factors including symptoms, markers of disease progression, or host immune function. Studies suggest that patients with chronic hepatitis C, even without major disease related complications, perceive themselves to be unwell and have significant changes in their physical and mental well being. Such results have important implications for nursing care and management, including in such areas as alcohol consumption, fatigue reduction, and depression and psychological distress. The authors conclude that intervention studies focused on self care management with an emphasis on symptom reduction are warranted. 1 figure. 43 references.

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Current and Novel Immunosuppressive Therapy for Autoimmune Hepatitis. Hepatology. 35(1): 7-13. January 2002.

Corticosteroids alone or in conjunction with azathioprine represent the treatment of choice in patients with autoimmune hepatitis (AIH) and results in remission induction in over 80 percent of patients. This article reports on immunosuppressive therapy for AIH. Sustained response to this therapy may result in substantial regression of fibrosis even in advanced cases. The result of rapid withdrawal of immunosuppression (stopping the drugs) is disease relapse in many patients. Consequently, the use of 2 milligrams per kilogram per day of azathioprine as a sole agent to maintain remission has been widely accepted in clinical practice. Persistent severe laboratory abnormalities or histologic abnormalities such as bridging necrosis (tissue death) or multilobular necrosis are absolute indications for treatment based on controlled clinical trials, but debate exists as to whether all patients with AIH need treatment. Examination of liver tissue (by biopsy) remains the best method of evaluating both treatment response and need for treatment in patients who have little biochemical activity. Alternative strategies in patients who have failed to achieve remission on 'standard therapy' of corticosteroids with or without azathioprine or patients with drug toxicity include the use of cyclosporine, tacrolimus, or mycophenolate mofetil. Liver transplantation is the treatment of choice in managing decompensated disease. 1 figure. 2 tables. 50 references.

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Endoscopic Retrograde Cholangiopancreatography. Gastrointestinal Endoscopy Clinics of North America. 11(4): 585-601. October 2001.

Endoscopic retrograde cholangiopancreatography (ERCP) is an established procedure in the evaluation and treatment of adult patients with suspected disorders of the pancreas and the biliary tract. This article defines the technique, indications, complications, and diagnostic and therapeutic applications of ERCP in children. The use of this technique in children has been limited. This may be caused by multiple factors including the relatively low incidence of pancreatic and biliary diseases in childhood and a low index of clinical suspicion; limited availability of pediatric duodenoscopes; lack of pediatric gastroenterologists adequately trained to perform ERCP; the impression that ERCP in children is technically difficult to accomplish; difficulty in the effective evaluation of a therapeutic result; and lack of well-defined indications and safety of ERCP in children. The article reviews biliary findings, including bile plug syndrome, primary sclerosing cholangitis, biliary obstruction due to parasitic infestation, cholelithiasis (gallstones) and choledocholithiasis (bile tract stones), benign and malignant biliary strictures, biliary obstruction or leaks after liver transplantation, and common bile duct complications after laparoscopic cholecystectomy (removal of the gallbladder). The authors also discuss pancreatic findings, including recurrent pancreatitis, congenital disorders, pancreatic anomalies, duodenal anomalies, acquired disorders, chronic pancreatitis, and pseudocysts. 8 figures. 4 tables. 67 references.

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Surgery for Adult Polycystic Liver Disease. Journal of Gastroenterology and Hepatology. 15(11): 1239-1242. November 2000.

Adult polycystic liver disease, commonly associated with polycystic kidney disease, can result in massive hepatomegaly (enlarged liver) and debilitating symptoms. This article reviews the use of surgery for adult polycystic liver disease (APLD). Surgical intervention for symptomatic APLD, such as cyst fenestration (the creation of an opening in the cyst) or liver resection has been associated with significant morbidity (illness and complications) and inconsistent long term palliation. However, selected patients with severe symptoms benefit from liver resection and extensive fenestration with acceptable morbidity and mortality (death). These procedures can allow the excision of most prominent cysts with minimal resection of liver tissue; liver volume is preserved despite the polycystic disease. Total hepatectomy (removal of the liver) and orthotopic liver transplantation may need to be considered for patients with severe APLD. The author of the review describes the surgical results of various types of operations to help surgeons conceptualize which operation to offer patients with APLD. 1 table. 50 references.

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Features of Recurrent Primary Sclerosing Cholangitis in Two Consecutive Liver Allografts After Liver Transplantation. Journal of Clinical Gastroenterology. 32(2): 151-154. February 2001.

Primary sclerosing cholangitis (PSC) is a disease of unknown etiology (cause) that is characterized by inflammation and fibrosis (scarring) of the biliary tree, which results in stricture formation and, eventually, in liver failure. Recurrence of PSC after liver transplantation is very uncommon. The true incidence of recurrence is unknown, mainly because of the difficulty in differentiating ischemic strictures from that of recurrent disease. PSC and ischemic strictures have identical histopathologic and cholangiographic (a type of diagnostic test) features. In this article, the authors report the case of a young man who had recurrence of PSC in two allografts (transplants) and also report their experience with 32 patients who had liver transplantation for PSC. Six patients (18 percent) had evidence of non anastomotic strictures and, of these, only one patient (reported here) had unequivocal evidence of true recurrence. The strictures in the other five patients happened because of ischemia (lack of blood flow to the organ involved). The authors conclude that the recurrence of the disease in two allografts in an immunosuppressed patient (taking drugs to avoid rejection of the transplant), in the absence of ischemia, chronic rejection, or any known pathogen, raises the question of the role of an unidentified infectious agent in the cause of PSC. 2 figures. 2 tables. 14 references.

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Genetic Testing in Hemochromatosis. Practical Gastroenterology. 25(9): 44, 47-48, 50, 52, 54, 56. September 2001.

Hereditary hemochromatosis (HH) is an inherited disorder in which the regulation of intestinal iron absorption is lost. Over several decades, iron accumulates and damages multiple organs such as the liver, pancreas, and heart. The identification of the gene responsible for HH (called HFE) has placed this disorder on the cutting edge of molecular medicine. Although this discovery has led to many advances in the understanding of the genetics, pathophysiology and clinical consequences of HH, many questions remain. This article reviews genetic testing in HH. The authors stresses that appropriate management of HH in the modern era requires gastroenterologists and hepatologists (liver specialists) to have an understanding not only of the medical aspects of HH but also of the issues involved in genetic testing. Before embarking on genetic testing, a physician must be willing and able to manage the implications of genetic testing. Despite a normal life expectancy when diagnosed before the development of cirrhosis or diabetes, insurance discrimination remains a concern. Patient confusion about the interpretation of results is common, as are ethical considerations such as the disclosure of positive results to family members. The author also comments on some unresolved issues in HH, in the areas of liver transplantation, hepatitis C, cardiovascular disease, and diabetes mellitus. An algorithm for the diagnosis of HH using genetic testing is provided. 2 figures. 3 tables. 29 references.

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Hepatic Encephalopathy. Practical Gastroenterology. 25(5): 48, 50, 52, 54, 56, 58. May 2001.

The management of hepatic encephalopathy (brain or neuropsychiatric disturbances associated with liver dysfunction) is a largely empiric regimen. This article reviews the care of patients with hepatic encephalopathy. The precise cause for this usually reversible neuropsychiatric syndrome is unknown. Blood ammonia levels are felt to play a major role. Once the diagnosis of hepatic encephalopathy (HE) is suspected, a standardized set of measures are employed for nearly all patients. Supportive care as needed for any patient with altered mental status is instigated. After estimating the degree of severity of mental status changes, precipitating factors for the episode and other potential concurrent causes of encephalopathy are sought and treated. Aggressive gut cleansing with primarily nonabsorbable disaccharides complete the largely effective management for HE. Second line measures include antibiotic and dietary manipulation. In cases that are truly resistant to treatment, elective portosystemic shunt closures and liver transplantation are becoming more commonplace. 6 tables. 10 references.

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Hepatitis C and Minorities. Practical Gastroenterology. 25(11): 14, 16, 19-20, 23. November 2001.

Hepatitis C virus (HCV) is a major cause of chronic liver disease in the United States and the leading indication for liver transplantation. This review article summarizes the impact of racial and ethnic factors on different aspects of HCV infection. Most of the published data relates to blacks and very little information is available on other minorities. HCV infection rate is higher in blacks and Hispanics than in whites. Blacks have a higher rate of cirrhosis, hepatocellular carcinoma and death due to HCV related liver disease. There are no racial differences with respect to baseline liver test abnormalities and HCV RNA titers. However, blacks have a higher infection rate with HCV genotype 1 that responds less well to treatment compared to other genotypes. The overall response to anti-viral therapy is lower in blacks compared to whites. Finally, blacks are less likely to get a liver transplant, and those who get transplanted have lower survival rates than whites. 29 references.

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Liver Hanging Maneuver: A Safe Approach to Right Hepatectomy Without Liver Mobilization. Journal of the American College of Surgeons. 193(1): 109-111. July 2001.

In right hepatectomy (removal of the right side of the liver), the complete mobilization of the liver before parenchymal (body of the organ) transection is considered as a basic maneuver for a safe procedure. This article discusses a safe approach to right hepatectomy that does not involve liver mobilization. The authors propose a new technique of hanging the liver after lifting it with a tape passed between the anterior surface of the (inferior vena cava) IVC and the liver parenchyma. The authors describe the technique and then discuss its indications. Mobilization (movement or rotation of the liver) can result in tumor dissemination (spread) or compression of the remnant liver. Avoiding this movement with the liver hanging maneuver avoids some of these complications. The absence of compression of the remnant liver is a key factor especially in patients with cirrhosis (liver scarring) and in liver resection in adult living related liver transplantation. 3 figures. 5 references.

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Hepatic Encephalopathy: Metabolic Consequence of Cirrhosis Often is Reversible. Postgraduate Medicine. 109(2): 52-54, 57-60, 63-65, 69-70. February 2001.

This article discusses hepatic encephalopathy, a condition characterized by neuropsychiatric manifestations ranging from a slightly altered mental status to coma, and neuromuscular symptoms may be present. This complication of chronic or acute liver disease is a result of the failure of the liver to detoxify toxins originating in the intestine. The pathogenesis (how it occurs) probably is multifactorial, although the predominant causative agent appears to be ammonia. About 30 percent of patients with cirrhosis (scarring of the liver) die in hepatic coma. The molecular basis of neurotoxicity of ammonia or other agents implicated in the condition is poorly understood. Therapy includes timely recognition and correction of precipitating factors. Once the condition is manifested, standard therapy is acute administration of lactulose, a disaccharide that is undigested in the small intestine. The beneficial action of lactulose is not fully understood. The use of oral antibiotics and BCAAs (branched chain amino acids) is of some benefit in patients who do not respond to lactulose. Limitation of protein in the diet may be useful for short periods but is not recommended for long term use because of potential worsening of already poor nutrition. The ultimate therapy for hepatic encephalopathy is orthotopic liver transplantation. Future research will likely focus on the correction of alterations in neurotransmission. 2 figures. 4 tables. 20 references.

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Hepatitis C Virus Infection. New England Journal of Medicine. 345(1): 41-52. July 5, 2001.

Hepatitis C virus (HCV) infects an estimated 170 million persons worldwide and thus represents a viral pandemic, one that is five times as widespread as infection with HIV. This article reviews recent advances in understanding the pathogenesis (development) of the infection and improvements in treatment options. The author notes that the institution of blood screening measures in developed countries has decreased the risk of transfusion associated hepatitis to a negligible level, but new cases continue to occur mainly as a result of injection drug use and, to a lesser degree, through other means of percutaneous or mucous membrane exposure. Progression to chronic disease occurs in the majority of HCV infected persons, and infection with the virus has become the main indication for liver transplantation. HCV infection also increased the number of complications in persons who are coinfected with HIV. The authors conclude that the best hope for a solution to the epidemic of HCV infection is the development of an effective vaccine. Although the recent demonstration of apparent immunologic clearance of virus in some persons with acute infection provides hope that a vaccine may someday be developed, it is not likely to be available soon. For those who are already infected with HCV, new therapeutic approaches can be expected in the future. Persons who have no response to therapy and who have a high risk of imminent progression to decompensated liver disease might benefit from therapies (such as interleukin 10) that halt disease progression until better therapies become available. 3 figures. 3 tables. 106 references.

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Liver Transplantation for Primary Sclerosing Cholangitis. In: Bayless, T.M. and Hanauer, S.B. Advanced Therapy of Inflammatory Bowel Disease. Hamilton, Ontario: B.C. Decker Inc. 2001. p. 305-310.

Primary sclerosing cholangitis (PSC) is a chronic, usually progressive, liver disease characterized by intrahepatic or extrahepatic (inside or outside the liver, respectively) duct stricturing (narrowing) and dilatation ('beaded' appearance). This chapter on liver transplantation for PSC is from the second edition of a book devoted to the details of medical, surgical, and supportive management of patients with Crohn's disease (CD) and ulcerative colitis (UC), together known as inflammatory bowel disease (IBD). About 70 percent of the patients with PSC have IBD, with UC being more common than CD (by a ratio of 9 to 1). Dominant, symptomatic strictures can be managed endoscopically. Reconstructive biliary surgery has no role in the management of PSC. Liver transplantation is the treatment of choice. Five-year survival after liver transplantation is approximately 80 to 90 percent. Actuarial patient survival after liver transplantation is significantly better than the survival rate of patients treated with nontransplantation biliary surgery or the predicted survival from prognostic models. Ten to 15 percent of patients with PSC develop cholangiocarcinoma (biliary tract cancer), which is difficult to diagnose and rarely curable; however, in patients with small incidental cholangiocarcinomas, recognized only at liver exploration (in the absence of lymph node spread), the prognosis is more encouraging. It is recommended that patients with PSC are transplanted earlier than patients with other liver diseases. The high risk and poor prognosis for cholangiocarcinoma in longstanding PSC are an argument in favor of performing liver transplantation in an earlier phase of the disease. Colonoscopic surveillance for dysplasia (abnormal tissue) is essential. 2 figures. 1 table. 21 references.

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Liver Transplantation. In: Okuda, K., ed.,et al. Hepatobiliary Diseases: Pathophysiology and Imaging. Malden, MA: Blackwell Science, Inc. 2001. p. 599-612.

Liver transplantation is the treatment of choice for patients with irreversible liver failure. This chapter on liver transplantation is from a textbook that familiarizes the reader with various imaging modalities, the information they provide, and the merits of each, in order to facilitate the combined use of different imaging techniques in the diagnosis and management of hepatobiliary (liver and bile tract) diseases. The authors stresses that successful liver transplantation is dependent on accurate radiological imaging. As a member of the transplantation team, the radiologist must be familiar with the surgical anatomy of liver transplantation and the complex imaging of liver transplant patients. The radiological assessment includes the preoperative evaluation of patients with liver failure, accurate hepatic imaging of living donors, and rapid and accurate diagnosis of postoperative complications. The author discusses preoperative imaging, anatomy and normal postoperative findings, and post transplantation complications, including vascular complications, biliary complications, fluid collections, rejection, and post transplant malignancy (cancer). The preoperative assessment includes both technical aspects such as liver volume determination, and accurate diagnosis of conditions affecting transplantation, such as vessel thrombosis and liver cancer. The postoperative evaluation centers of rapid and accurate detection of complications. The author concludes that in many instances, accurate and prompt radiological assessment can play an important role in implementing treatments that prevent graft failure. 26 figures. 44 references.

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Liver Transplantation: Current Status and Novel Approaches to Liver Replacement. Gastroenterology. 120(3): 749-762. February 2001.

This article reviews the current status and novel approaches to liver replacement. The author contends that the major challenge currently facing liver transplantation is the performance of a greater number of liver transplants, which has been fueled by the large and growing disparity between the increasing number of qualified patients listed for transplantation and the relatively static number of available cadaver donor organs. In the past 2 years, approximately 4,500 liver transplants have been performed annually, with 1 year survival rates in the 85 to 90 percent range, while the waiting list has expanded as of November 2000 to more than 16,000 patients, resulting in an increasing death rate among listed patients. In the short term, there will continue to be a major focus on more effective use of available cadaver donor organs to balance the competing principles of justice (patients with most urgent need for transplant and lower probability of posttransplant survival) and medical utility (patients with less urgent need for transplant and higher odds of postoperative survival). Over the long term, there will be an increasing application of novel approaches to liver replacement including cadaver split liver transplantation and adult living donor liver transplantation and possibly, in the more distant future, xenotransplantation (using organs from other mammals) and hepatocyte transplantation. The treatment, and ideally the prevention, of recurrent disease after liver transplantation, notably chronic hepatitis C, is a major priority to optimize the use of liver grafts. Finally, improved immunosuppressive strategies, including movement toward minimal immunosuppression and steroid withdrawal and the development of safer and more effective drugs, is another important factor that has the potential to increase the success of liver transplantation. 2 figures. 11 tables. 104 references.

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Liver Transplantation: What the Non-Hepatologist Should Know. Practical Gastroenterology. 25(4): 42, 47-48, 50, 52, 54, 56-58, 60, 62, 64, 66. April 2001.

Over the last two decades, liver transplantation (LT) has been transformed from an experimental procedure with uncertain outcome to a safe and reliable treatment for hepatic failure that has dramatically changed the natural history of liver diseases. This article familiarizes health care providers (who are not liver specialists) with the present indications for liver transplantation and care of patients undergoing this procedure. More than 3,000 LTs are performed each year in the United States; the number is limited only by the shortage of donor organs. The 5 year survival rate after LT is up to 80 percent in the most successful centers and LT is the only reasonable option for individuals with end stage liver disease. However, because of the reduced number of available organs and the long waiting times on transplant lists, the selection and timing of patient referral to a transplant center is crucial. Equally important is the long term medical followup of individuals with liver transplants. The goals should be to prevent and to recognize at the earliest opportunity any complications that can be managed appropriately. For all this to occur, all the medical personnel involved in the care of an individual with liver disease must be familiar with the option of LT as well as with the pathophysiology of liver failure, its clinical recognition and management. The authors conclude with a discussion of the side effects of immunosuppression, noting that these adverse drug events are common medical problems in allograft recipients. These complications include hypertension (high blood pressure), diabetes mellitus, renal dysfunction (kidney failure), infection (usually by an opportunistic organism), and new onset neoplasia, including cancer. 2 figures. 10 tables. 30 references.

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Primary Biliary Cirrhosis. Toronto, Ontario: Canadian Liver Foundation. 2001. 2 p.

This brochure describes primary biliary cirrhosis (PBC), a chronic liver disease. The brochure reviews the causes of PBC, its symptoms, diagnostic tests used to confirm the condition, prognosis, and treatment options. It is believed that PBC results from an abnormal reaction of the body's immune system. The immune system of PBC patients attacks the liver causing slow, progressive damage to the bile ducts, resulting in the accumulation in the liver of bile and other substances. These retained toxic substances and inflammation can cause further damage. While there is no known specific treatment for PBC, there are various treatments that can help patients cope with the symptoms. Liver transplantation is now a common treatment option for people with advanced PBC. The brochure concludes with a brief description of the goals and activities of the Canadian Liver Foundation (www.liver.ca).

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Long-Term Management of Cirrhosis: Appropriate Supportive Care Is both Critical and Difficult. Postgraduate Medicine. 109(3): 101-118. March 2001.

In the United States, about 26,000 of the 3 million people who have chronic liver disease die each year because of hepatic cirrhosis (liver scarring). Liver transplantation offers the best hope of survival for many of these patients, but the number of patients awaiting transplant far exceeds the number of organs available. This article (the last in a four part series on cirrhosis) offers a guide for primary care physicians who are caring for patients with chronic liver disease. The focus is on strategies to slow the course of liver disease and improve both the quality and length of life for these patients. The authors stress that early consultation with a liver transplant center can be helpful. The transplant hepatologist (liver specialist) and surgeon can help with triage decisions, guide workup, provide advice about patient care, optimize the timing of transplantation, offer specialized diagnostic and therapeutic options, and help the treating physician stay up to date on the continuous changes in this complex field. The authors offer a ten-point program for the comprehensive management of the patient with cirrhosis. The authors conclude that it is often the skill and diligence of the primary care physician in diagnosing liver disease, identifying and treating correctable causes, optimizing the patient's health and nutrition, and anticipating and preventing catastrophic complications that determine whether the patient lives or dies. Appended to the article is a lengthy list of resources on cirrhosis, including organizations, web sites, and publications, separated by those designed for physicians and those specifically for patients. 1 figure. 3 tables. 27 references.

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Minimizing Ascites: Complication of Cirrhosis Signals Clinical Deterioration. Postgraduate Medicine. 109(2): 91-96, 101-103. February 2001.

Ascites, the pathologic accumulation of fluid in the peritoneal cavity, is a common and serious complication of cirrhosis (scarring) of the liver. The development of ascites is associated with a grave prognosis: 50 percent of patients die within 2 years of diagnosis. This article describes strategies to minimize ascites and its sequelae. Proper management with a combination of dietary, medical, and surgical approaches is essential to prolong life and improve its quality. Patients with ascites are at risk for ascitic fluid infections and neurohormonal dysregulation that can lead to hepatorenal syndrome. Early recognition of these complications allows therapeutic interventions that minimize further clinical deterioration in already chronically ill patients. Treatment goals include symptoms relief, correction of underlying pathophysiologic abnormalities (i.e., renal sodium retention, sinusoidal portal hypertension), prevention and treatment of complications of ascites, and improvement of outcome. Treatment options range from bed rest to orthotopic liver transplantation, and can include dietary sodium restriction, diuretic therapy, large volume paracentesis (removal of 5 liters or more of ascitic fluid during a single session), peritoneovenous shunt (to return ascitic fluid directly from the peritoneal cavity to the systemic circulation), transjugular intrahepatic portasystemic shunt (TIPS), and liver transplantation. Potential complications of ascites include refractory ascites, hepatorenal syndrome, and spontaneous bacterial peritonitis. 3 tables. 17 references.

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Outcome of Hospital Care of Liver Disease Associated with Hepatitis C in the United States. Hepatology. 33(1): 201-206. January 2001.

This study describes mortality and resource utilization for inpatient (hospital) care of hepatitis C (HCV) in comparison to alcohol induced liver disease (ALD) in the United States. The study also identifies factors that affect outcomes. The Healthcare Cost and Utilization Project database, a national inpatient sample was used to identify hospitalization records with diagnoses related to liver disease from HCV and ALD. Outcomes of hospitalizations was analyzed in terms of inhospital deaths and health care resource utilization. For 1995, the authors estimate that there were 26,700 hospitalizations and 2,600 deaths in acute, nonfederal hospitals in the United States for liver diseases caused by HCV. Total charges for these hospitalizations were $514 million. In comparison, ALD was associated with 101,200 hospitalizations; 13,400 deaths, and $1.8 billion in charges. Simultaneous HCV and alcohol abuse was associated with younger ages at the time of hospitalization and death compared with HCV or ALD alone. In analyses, alcohol abuse and HIV infection were associated with an increased risk of death among those with HCV. Liver transplantation and patient death were associated with the largest increase in hospitalization charges. Major complications of cirrhosis (such as variceal bleeding, encephalopathy, and hepatorenal syndromes) and sociodemographic factors (such as race and health insurance) were also significantly associated with the risk of death and hospitalization charges, which were similar in HCV and ALD. The authors provide new estimates regarding the public health impact of HCV, for use in health policy decision and cost effectiveness analyses of preventive and therapeutic interventions. 6 tables. 21 references.

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Primary Sclerosing Cholangitis. In: Okuda, K., ed.,et al. Hepatobiliary Diseases: Pathophysiology and Imaging. Malden, MA: Blackwell Science, Inc. 2001. p. 674-681.

Primary sclerosing cholangitis (PSC) is a disease of unknown etiology characterized by progressive inflammation and fibrosis of the liver and bile ducts. The disease typically occurs in patients with inflammatory bowel disease, but it may also occur alone. This chapter on PSC is from a textbook that familiarizes the reader with various imaging modalities, the information they provide, and the merits of each, in order to facilitate the combined use of different imaging techniques in the diagnosis and management of hepatobiliary (liver and bile tract) diseases. Topics include etiology and pathology, clinical features, imaging diagnosis, and treatment of the condition. A gradual onset of progressive fatigue and pruritus (itching), followed by jaundice, is the most frequent complex of symptoms that leads to the diagnosis of PSC. Direct Cholangiography via an endoscopic or percutaneous approach is regarded as the gold standard technique for the diagnosis of PSC. There is no specific therapy for PSC, owing to the uncertainty regarding the cause of the condition. Medical therapy has centered on the symptoms and complications, as well as the underlying disease processes. However, for patients with end stage disease who have symptomatic portal hypertension, liver failure, and recurrent or intractable bacterial cholangitis, liver transplantation is the only effective treatment. 8 figures. 1 table. 17 references.

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Sclerosing Cholangitis. In: Bayless, T.M. and Hanauer, S.B. Advanced Therapy of Inflammatory Bowel Disease. Hamilton, Ontario: B.C. Decker Inc. 2001. p. 299-302.

This chapter on sclerosing cholangitis is from the second edition of a book devoted to the details of medical, surgical, and supportive management of patients with Crohn's disease (CD) and Ulcerative Colitis (UC), together known as inflammatory bowel disease (IBD). Sclerosing cholangitis is a cholestatic liver disorder characterized by diffuse strictures (scar tissue causing a narrowing) of the bile ducts. The disorder may be primary (idiopathic) or secondary due to structural abnormalities of the bile ducts. Primary sclerosing cholangitis (PSC) is clinically indistinguishable from disorders that cause secondary sclerosing cholangitis. There is a strong association of PSC and IBD, particularly ulcerative colitis. Approximately 4 percent of patients with IBD will either have or develop PSC. In this chapter, the authors review the relevant data on the management of PSC. The treatment of PSC has been limited by uncertainty about its cause. The medical management is divided into management of symptoms and complications and specific therapy of the underlying disease process. The author discusses the use of drugs, including corticosteroids and azathioprine, D penicillamine, colchicine, cyclosporine, urodeoxycholic acid, methotrexate, pentoxifylline, and tacrolimus. However, liver transplantation is the only effective treatment and is recommended for patients with end-stage disease who have symptomatic portal hypertension, liver failure, and recurrent or intractable bacterial cholangitis. 10 references.

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Stricture Management in Primary Sclerosing Cholangitis. In: Bayless, T.M. and Hanauer, S.B. Advanced Therapy of Inflammatory Bowel Disease. Hamilton, Ontario: B.C. Decker Inc. 2001. p. 303-304.

This chapter on stricture management in primary sclerosing cholangitis is from the second edition of a book devoted to the details of medical, surgical, and supportive management of patients with Crohn's disease (CD) and Ulcerative Colitis (UC), together known as inflammatory bowel disease (IBD). Sclerosing cholangitis is a cholestatic liver disorder characterized by diffuse strictures (scar tissue causing a narrowing) of the bile ducts. The disorder may be primary (idiopathic) or secondary due to structural abnormalities of the bile ducts. Primary sclerosing cholangitis (PSC) is clinically indistinguishable from disorders that cause secondary sclerosing cholangitis. There is a strong association of PSC and IBD, particularly ulcerative colitis. Approximately 4 percent of patients with IBD will either have or develop PSC. The mainstay of treatment of PSC is liver transplantation with survival rates of greater than 80 percent at 5 years posttransplant. The author cautions that the use of other palliative treatment options such as endoscopic, nontransplant surgical or radiologic interventions should be evaluated in the context of the effectiveness of liver transplantation. The concern of underlying cholangiocarcionoma (biliary tract cancer) should be a priority when evaluating and treating these strictures. 11 references.

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Transplantation of the Liver and Pancreas. In: Beckingham, I.J., ed. ABC of Liver, Pancreas and Gallbladder. London, UK: BMJ Publishing Group. 2001. p.47-49.

Liver transplantation is carried out for many chronic liver diseases and for fulminant hepatic (liver) failure. Activity is limited by availability of donor organs. Transplantation of the pancreas is less well established. The pancreas is usually transplanted together with a kidney in patients with end stage diabetes mellitus and renal (kidney) failure. This chapter on transplantation of the liver and pancreas is from an atlas of the liver, pancreas and gallbladder. The section on liver transplantation covers indications and contraindications, timing and selection of patients for transplantation, acute liver failure and timing of transplantation, the surgical procedures, postoperative management, expected results, and pediatric liver transplantation. The section on pancreatic transplantation covers the goals of transplantation, selection of recipients, and isolated pancreatic islet transplantation. The chapter concludes with summary points of the concepts discussed. 4 figures. 2 tables.

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What is Hemochromatosis?. Cedar Grove, NJ: American Liver Foundation. 2001. [4 p.].

This brochure reviews hemochromatosis, an inherited condition that causes the body to absorb and store too much iron. The brochure answers common questions about hemochromatosis, covering the risk factors for the disease, the symptoms, diagnostic tests for iron overload, treatment options, the prognosis for people with hemochromatosis, the presence of anemia with iron overload, the role of alcohol in accelerating liver damage, the relationship between diet and iron overload, and liver transplantation in patients with hemochromatosis. The brochure concludes with a brief description of the American Liver Foundation (ALF), a nonprofit, national voluntary health organization dedicated to the prevention, treatment, and cure of hepatitis and other liver diseases through research, education, and advocacy.

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Alpha 1-Antitrypsin Deficiency Liver Disease. Minneapolis, MN: Alpha 1 Association. 2000. [4 p.].

This brochure describes Alpha 1 antitrypsin deficiency (A1AD or Alpha 1), a genetic disorder that affects infants, children, and adults. It is the most common metabolic disorder that causes liver disease in infants and children; the disorder also causes cirrhosis and cancer of the liver in adults. The brochure reviews the functions of the liver, the causes of the deficiency, symptoms in children and adults, and treatment options. Alpha 1 antitrypsin (AAT) is a protein primarily manufactured in the liver and then released into the blood. The normal function of AAT is to protect body tissues from being damaged by neutrophil elastase, a protein found in white blood cells. The backup of abnormal AAT in the liver can cause liver damage. Symptoms of A1AD in children includes jaundice, low birth weight, mildly elevated liver enzymes, cholestasis, enlarged liver, abnormal bleeding, feeding difficulties, poor growth (or failure to thrive), and ascites (abnormal accumulation of fluids). In adults, the spectrum of liver disease associated with A1AD deficiency varies from mild to severe. Symptoms include chronic active hepatitis, cryptogenic cirrhosis (liver scarring of unknown cause), portal hypertension (high blood pressure in the portal vein of the liver), and hepatocellular carcinoma (liver cancer). Clinical care for all affected individuals largely involves supportive management for liver dysfunction and prevention of complications. For those who develop severe liver injury, liver transplantation is usually recommended. Proper nutrition is essential for everyone with A1AD. The brochure concludes with contact information for the Alpha 1 Association. 1 figure. 3 references.

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Antiviral Therapy for Chronic Hepatitis B and C: Which Patients are Likely to Benefit from Which Agents?. Postgraduate Medicine. 107(2): 135-138, 141-142, 144. February 2000.

This article is the third in a four article symposium that provides a practical approach to the diagnosis and management of common hepatic disorders encountered by primary care physicians. In this article, the author discusses current therapies for chronic hepatitis B and C and provides insight into future medications. Antiviral therapy in chronic hepatitis can completely eradicate the virus and induce remission of liver disease. To achieve such benefits, however, therapy should be initiated before decompensated liver disease ensues; at that point, liver transplantation is the only available option. The author describes indications for and contraindications to antiviral therapy in chronic hepatitis. For chronic HBV infection, interferon alfa2b requires only a 4 month course. However, it has adverse effects and contraindications and does not produce a universal response. Another option for HBV infection is lamivudine, which is administered orally and causes few side effects. However, relapse may occur when treatment is discontinued, and mutant virus may emerge. For chronic HCV infection, interferon alfa2a, interferon alfa2b, consensus interferon, and interferon combined with ribavirin have been used. The combination alternative is emerging as the method of choice in patients who do not have contraindications to oral ribavirin. Adverse effects are common and durability of response varies according to HCV RNA level and genotype. 5 tables. 21 references.

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Autoimmune Hepatitis. Gastroenterology Nursing. 23(4): 157-159. July-August 2000.

Autoimmune hepatitis (AIH) is a necro inflammatory disease that, untreated, carries a 3 year mortality (death) rate of approximately 50 percent. In this last of a series of three articles, the author reviews current knowledge about AIH. The mode of presentation of AIH is variable: insidious (hidden) onset with few if any symptoms, presenting with symptoms indistinguishable from that of any acute viral hepatitis, or onset with fulminant hepatitis. In AIH, hepatocytes become injured by various agents (such as a viral infection) and become antigenic, leading to a self perpetuating antigen/antibody response with subsequent chronic liver disease. AIH is the only type of hepatitis that is responsive to corticosteroids, but, complete withdrawal of steroids may not be possible. Occasionally, AIH develops after liver transplantation. In patients with AIH, there is a high frequency of other coexisting autoimmune disorders, particularly type 1 diabetes, vitiligo, glomerulonephritis, and autoimmune hemolytic anemia. Neither type of AIH is contagious, and with treatment, the adults and children affected can usually lead a near normal life. Medications metabolized in the liver and alcohol need to be avoided by these patients. 21 references.

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Characteristics of and Current Treatment Options for Hepatitis B and Hepatitis C. Journal of the Canadian Dental Association. 66(10): 537. November 2000.

This brief article reviews the characteristics of and current treatment options for hepatitis B and hepatitis C, focusing on the risks to health care workers, including dental professionals. The author provides information about the natural history, therapeutic choices, and future directions of therapy for these two forms of hepatitis. Not all patients who test positive for hepatitis B surface antigen (HbsAg) require antiviral therapy; many people have circulating HBsAg without evidence of significant hepatitis. These people are chronic carriers and account for the majority of hepatitis B cases. Treatment options include alpha interferon or oral lamivudine; however, conversion to negative status is achieved in only approximately 20 percent of cases with either therapy. Hepatitis C is a common condition affecting approximately 2 percent of Canadians; of patients with chronic hepatitis C, approximately 20 percent experience serious liver complications. These complications can include cirrhosis, need for liver transplantation, hepatocellular carcinoma (liver cancer), or death. Standard treatment for hepatitis C is alpha interferon and ribavirin; sustained remission occurs in approximately 40 percent of cases. The article concludes with the website for the Canadian Association for the Study of the Liver (www.lhsc.on.ca/casl/summ.htm ).

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Case of Sclerosing Cholangitis Managed by a Percutaneous Approach. Journal of Clinical Gastroenterology. 30(2): 205-209. March 2000.

This article reports on a case in which, in 1992, a 61 year old man who complained of recurrent episodes of fever and jaundice was diagnosed as having sclerosing cholangitis. In the three years that followed, the clinical picture progressively worsened; and, in 1995, the patient was hospitalized again for biliary obstruction. A liver transplantation was excluded because of concomitant severe coronary heart disease. A percutaneous transhepatic cholangiogram showed several critical strictures of the intrahepatic biliary tree and a temporary internal external biliary drainage was placed to relieve the obstruction. After 40 days, a two step percutaneous biliary balloon dilation was performed followed by topical steroid treatment through the catheter. After 45 days, the catheter was removed and steroid treatment tapered orally. In the three years that followed, the patient was well. He experienced only about 1 to 2 episodes of ascending cholangitis per year requiring antimicrobial therapy. Laboratory analysis showed a gradual improvement in hepatic chemistry, serum bilirubin, and erythrocyte sedimentation rate (ESR). In the patient, the association of percutaneous balloon dilation and topical steroid treatment improved both the clinical and radiological picture, without significant side effects. This approach should be considered a valuable and cost effective option in primary sclerosing cholangitis (PSC), mainly for patients not eligible for liver transplantation.

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Combination of Interferon-Alpha and Ribavirin Therapy for Recurrent Hepatitis C Virus Infection After Liver Transplantation. Transplantation Proceedings. 32(4): 714-716. June 2000.

Liver transplantation (LT) used as treatment for hepatitis C virus (HCV) infection is almost universally associated with a recurrence of infection. More than 60 percent of patients show clinical and histological signs of hepatitis within 1 year of transplantation, and in several studies a rapid development of fibrosis and cirrhosis was reported. This study was undertaken to examine the efficacy, safety, and tolerability of the combination of interferon (IFN) and ribavirin for recurrent HCV infection after LT. Five patients (3 men and 2 women, age range 43 to 63 years) were included in the study; the median time between LT and initiation of treatment was 20 months (range, 10 to 24 months). Only one patient completed the 6 months of combination therapy. He had a normal serum ALT level at the end of the course, but remained serum positive for HCV, and 3 months after completing therapy, his serum ALT increased again. In the other four patients, therapy was discontinued after 1 to 3 months. All four had severe symptomatic hemolysis (breakdown of red blood cells); two patients required blood transfusions. Decreasing the ribavirin dose did not yield an increase in serum hemoglobin level, and ribavirin had to be withdrawn in all 4 patients. No episodes of rejection were recorded. All patients retained stable graft function 3 to 5 years after transplantation. The authors conclude that, despite the small sample size, the study suggests that the combination therapy with IFN and ribavirin after LT for recurrent HCV infection is associated with a high rate of severe side effects necessitating withdrawal of therapy. 1 table. 9 references.

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Facts on Liver Transplantation. Cedar Grove, NJ: American Liver Foundation. 2000. [4 p.].

Many diseases are capable of interfering with the liver's functions sufficiently to threaten the life of the patient; many of these diseases are potentially treatable by liver transplantation. This brochure answers 26 commonly asked questions about liver transplantation. Cirrhosis, the death of liver cells due to a variety of causes, is one of the most common reasons for liver transplantation in adults. In children, the disease most often treated by liver transplantation is biliary atresia, a failure of the bile ducts to develop normally to drain bile from the liver. Most cancers of the liver begin somewhere else in the body and spread to the liver; these are not curable with a liver transplant. Before surgery, the risks are mainly the development of some acute complication of the disease that might render the patient unacceptable for surgery. With transplantation, there are risks common to all forms of major surgery, as well as technical difficulties in removing the diseased liver and implanting the donor liver. Immediately after surgery, bleeding, poor function of the grafted liver, and infections are major risks. The brochure describes immediate postoperative and acute recovery, as well as long term recovery, including concerns regarding rejection of the transplanted organ. All the drugs used to prevent rejection increase the person's susceptibility to infections (and possibly to the development of tumors). Routine follow up consists of monthly blood tests, measuring blood pressure by a local physician, and annual or semi-annual checkups at the transplant center. Most patients are able to return to a normal or near normal existence 6 to 12 months after a successful liver transplant. The brochure includes a discussion of organ donation and costs. The brochure concludes with a description of the American Liver Foundation (ALF) and its activities and contact information.

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Hepatitis C: A Viral Mystery. Boston, MA: Fanlight Productions. 2000. (videocassette).

Hepatitis C is a viral disease of the liver which affects nearly four million Americans. The virus is primarily spread through blood contamination. There is no vaccine and no definite cure for the infection. This documentary videotape profiles several individuals who are living with this serious, chronic illness. In addition to discussing the available medical treatments, the program explores alternative options for treatment, including dietary changes, herbal therapies, meditation, guided imagery, and Qi Gong. The program reviews the modes of transmission of hepatitis C virus (HCV), including through blood transfusions, IV drug use, unsafe sex practices or multiple sexual partners, and tattooing and body piercing. The program interviews Dr. Stephen Steady, a hepatologist (liver specialist) who reminds viewers that many people with HCV infections are asymptomatic, but complications can be rampant, affecting other organ systems and overall quality of life (primarily through fatigue). The program reviews the drug therapy options (interferon and ribavirin, predominantly), noting that these treatments are effective only in 40 percent of the patients with hepatitis C, yet they can cause many side effects of their own. The program concludes with a look at the need for liver transplantation for some patients with hepatitis C, the important role of hepatitis C education for prevention, and support groups for patients with the illness.

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Hepatitis C: An Update on the Silent Epidemic. Journal of Clinical Gastroenterology. 30(2): 125-143. March 2000.

Hepatitis C virus (HCV) currently infects an estimated 2 to 3 million people in the United States and 175 million people globally. Over 80 percent of infected patients go on to develop chronic disease. This article offers an update on this 'silent epidemic,' noting that most patients remain asymptomatic despite insidious progression of the disease. The sequelae of HCV induced chronic liver disease accounts for 8,000 to 10,000 deaths annually in the United States and is currently the leading indication for liver transplantation. The cost of this epidemic to the United States was estimated in 1991 at $600 million in terms of medical expenses (excluding costs related to liver transplantation) and work lost. Over the last decade, since the viral genome of HCV was first sequenced in 1989, there has been a great increase in understanding of this infection. The authors discuss epidemiology of infection, viral characteristics, risk factors for disease, diagnostic testing, clinical manifestations, and therapeutic options. With modification of risk factors, aided by availability of accurate diagnostic tests and educational campaigns to target groups at risk, a decline in the incidence of acute HCV has already occurred. Therapeutic results are improving with combination interferon alpha and ribavirin, with sustained virologic response rates of 30 to 40 percent. The authors conclude that several disease modifiers may affect the natural history of HCV infection, leading to an accelerated clinical course and poorer therapeutic outcomes. Most important of these factors is continued alcohol use, which, even with modest consumption, may profoundly affect progression of liver disease in HCV infected patients. 5 figures. 4 tables. 176 references.

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Hepatitis Report: A Critical Review of the Research and Treatment of Hepatitis C Virus (HCV) and Hepatitis and HIV Coinfection. New York, NY: Treatment Action Group. 2000. 134 p.

This report is designed to bring clinicians, allied health care workers, and patients up to date on hepatitis C virus (HCV), including epidemiology, natural history, diagnosis, pathogenesis, and treatment. After an analysis of peer reviewed articles, over 40 researchers, clinicians, primary care physicians, government health administrators, industry representatives, and patients with viral hepatitis were interviewed for this report. Eleven chapters cover epidemiology, modes of transmission, and risk factors; pathogenesis, viral dynamics, and immunologic response; natural history, clinical manifestations, and prognostic indicators of disease progression and survival of HCV infection; diagnostic considerations; the use of interferon to treat HCV infection; the mechanism of HCV resistance to interferon; experimental treatments and new areas of research; hepatitis and HIV coinfection; current opinions and controversies in HCV infection; research and policy recommendations; and clinician's response. The natural history chapter reminds readers that not all patients with HCV inexorably deteriorate to end stage liver disease, liver transplantation, or death. The diagnosis of HCV is often complex with multiple tests and the confusion of liver biopsy evaluation. The role of HCV in response to HIV therapy is largely unknown. Approximately only half of HCV patients respond to current therapies, but the report considers whether perhaps only half may actually need therapy in the long term. Unfortunately, clinicians cannot determine which patients will progress to fibrosis and end stage liver disease and so most patients are treated, especially if they have some scarring without cirrhosis. Interferon remains the mainstay of therapy, with pegylated IFNs providing new advances. Each chapter includes illustrations and a list of references.

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Liver Disorders Sourcebook. Detroit, MI: Omnigraphics. 2000. 591 p.

This Sourcebook provides basic health care information about liver functions, guidelines for liver health, and tests that assess liver distress. The book also presents the symptoms, treatments, and preventive measures available for liver cancer; hepatitis A, B, C, D and E; genetically based liver diseases; and other liver diseases. The liver transplantation process is explained. Specific topics include strategies for protecting the liver, risk factors, common laboratory tests in liver disease, liver biopsy, cancer tumor markers, cirrhosis (scarring of the liver), infectious agents and parasites, pregnancy and the liver, jaundice in the healthy newborn, the liver's response to drugs, alcohol and the liver, acetaminophen, herbs and alternative medicine, galactosemia, Gaucher disease, hereditary hemochromatosis, Niemann-Pick disease, Wilson's disease, biliary atresia, cystic disease of the liver, fatty liver, gallstones, primary biliary cirrhosis, primary sclerosing cholangitis, organ donation, and the bioartificial liver. A glossary, a directory of organizations and support groups with up to date contact information (including websites and email addresses), a listing of transplant centers, and a subject index conclude the volume.

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Hepatitis: Still a Concern?. SCD. Special Care in Dentistry. 20(5): 209-212. 2000.

Although some forms of viral hepatitis were identified more than 50 years ago, hepatitis continues to have an impact on the practice of dentistry. This article reviews hepatitis, discussing possible transmission in the dental setting, management of the chronically ill, and legal issues related to treatment of infectious patients. Currently, seven viral forms of hepatitis are recognized. Those with predominantly enteral modes of transmission are of minor concern in the dental environment. Hepatitis B virus (HBV), the most infectious blood borne pathogen, has been largely controlled in this country by vaccination and the use of universal precautions. Hepatitis D virus (HDV) is an incomplete virus that has HBV infection as a prerequisite. Hepatitis C virus (HCV) is of great concern today for several reasons. A high percentage of HCV infections results in chronic disease. Most cases of HCV remain asymptomatic for an extended period of time, and many have no identifiable risk factors. Currently, no vaccination is available for HCV. Patients infected with HCV present a management challenge, because they may ultimately develop serious liver dysfunction. In fact, HCV infection is presently the most common reason for liver transplantation. By understanding the various forms of viral hepatitis and following recommended infection control and vaccination protocols, the dental health care worker can treat infected patients in a manner that is safe for both patients and dental health care workers. 1 table. 12 references.

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Infected and Unaware: Unmasking the Silent Epidemic of Hepatitis. Digestive Health and Nutrition. p. 22-25. March-April 2000.

An estimated 4 million people in the United States have chronic hepatitis and many of them are not even aware of their condition. This article familiarizes readers with the hidden epidemic of hepatitis, predominately hepatitis B and C viruses (HBC and HCV, respectively) that have become chronic infections. The author stresses that early diagnosis and treatment are curing some people with hepatitis and improving and prolonging the lives of others. Chronic hepatitis differs from the acute variety in the length of time an individual is affected. Treatment advances in the past few years for both HBV and HCV have produced drugs that reduce or eliminate evidence of the viruses from infected patients. Most hepatitis patients are referred by their primary care doctor. The patients most commonly are experiencing fatigue, but some may have more advanced symptoms such as jaundice and fluid retention. Only when the disease has done significant damage to the liver do the most serious symptoms appear, at which point a minority of patients seek medical help for the first time. Treatment options include lifestyle changes (particularly the avoidance of alcohol use), drug therapy, and liver transplantation. The author uses two case examples to show how chronic hepatitis can affect the lives of these patients. One sidebar reviews the physiology of the liver and its functions. The article concludes with a list of websites through which readers can obtain additional information.

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Liver Disease. In: King, J.E., ed. Mayo Clinic on Digestive Health. Rochester, MN: Mayo Clinic. 2000. p. 151-166.

This chapter on liver disease is from a comprehensive guidebook from the Mayo Clinic that focuses on a variety of digestive symptoms, including heartburn, abdominal pain, constipation, and diarrhea, and the common conditions that are often responsible for these symptoms. Written in nontechnical language, the book includes practical information on how the digestive system works, factors that can interfere with its normal functioning, and how to prevent digestive problems. The first section of the chapter focuses on hepatitis, including the key signs and symptoms of hepatitis, notably fatigue, loss of appetite, nausea, unexplained weight loss, and yellowing of skin and eyes (jaundice). The authors describe the different types of hepatitis (alcohol or drug induced, hepatitis A, hepatitis B, hepatitis C, hepatitis D and E, autoimmune hepatitis, and nonalcoholic steatohepatitis); review the blood tests (liver function tests) that may be used to help diagnose or monitor hepatitis; and discuss treatment options, including corticosteroids, interferon, lamivudine, and liver transplantation. A final section reviews healthy lifestyle approaches for living with hepatitis, and strategies for preventing the disease. The next section of the chapter addresses hemochromatosis (a genetic abnormality that causes the intestines to absorb too much iron), noting that the symptoms can include fatigue, joint pain, impotence (erectile dysfunction) or loss of sex drive, increased skin pigmentation (bronzing), and increased thirst and urination. This section also reviews diagnosis, determining whether screening is necessary for family members of patients with hemochromatosis, and the use of diet therapy (reduced iron intake) to help treat the disease. One sidebar mentions Wilson's disease and alpha 1 antitrypsin deficiency as other inherited liver disease. The last section of the chapter addresses cirrhosis, a condition in which scar tissue forms in the liver and keeps it from functioning normally. 1 figure.

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Liver Transplantation and Quality of Life. Canadian Journal of Gastroenterology. 14(8): 693-699. September 2000.

To date, more than 50,000 liver transplantations have been performed around the world; the survival rate five years after transplantation ranges from 60 to 65 percent. One of the fundamental objectives for liver transplantation teams is to achieve the best possible quality of life (QOL) for the patients. This article reports on a study of liver transplantation and QOL. The authors describe a concise analysis of the methodology used (15 questionnaires) to measure QOL of patients with liver transplants. The authors suggest that now is the time to establish a database so that a validated instrument will be available to compare QOL results from all the liver transplantation programs. Liver transplantation is believed to improve QOL, although there are several problems with some of the papers published on this subject. Some studies are retrospective, whereas in others, patients in bad physical conditions are excluded from the study; in quite a few of the prospective studies, deaths are not included in the data analysis. The authors conclude with a brief, concise analysis of late complications and of recurrence of the disease after liver transplantation, which influence QOL. One of the symptoms that most often worsens after transplantation is pain, which undoubtedly may result in a worse QOL. Other factors that can result in a decreased QOL include complications that appear in the long term, especially vascular and biliary diseases; neurological alterations, arterial hypertension and hepatic (liver) osteodystrophy; and the absence of family support, income, and employment. 56 references.

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Management of Primary Biliary Cirrhosis. Hepatology. 31(4): 1005-1013. April 2000.

Primary biliary cirrhosis (PBC) is a presumed autoimmune disease of the liver, which predominantly affects women once over the age of 20 years. Most cases are diagnosed when asymptomatic (60 percent). This article offers guidelines to assist physicians in the recognition, diagnosis, and management of patients with PBC. The antimitochondrial antibody is present in serum (blood) in most, but not all, patients with PBC. The disease generally progresses slowly, but survival is less than an age and gender matched general population. The symptomatic patients may have fatigue, generalized pruritus (itching), portal hypertension (high blood pressure), osteoporosis, skin xanthomata (yellowish nodules), fat soluble vitamin deficiencies, and or recurrent asymptomatic urinary tract infections (UTI). Many non liver autoimmune diseases are found in association with PBC and may prompt initial presentation. To date, immunosuppressive therapy has not been shown to prolong survival in PBC. The use of urodeoxycholic acid (UDCA) can delay the time to liver transplantation or death (typically given in a dose of 13 to 15 mg per kg daily). This therapy also causes a significant improvement of all the biochemical markers of cholestasis but has no beneficial effects on any of the symptoms or associated disorders. Treatment with UDCA does not obviate the need for liver transplantation. Therapies to prevent complications arising from malabsorption, portal hypertension, and or osteoporosis are required as well. Good control of pruritus can be achieved in most patients. PBC is diagnosed with increasing frequency, but the agent(s) responsible for this slowly progressive destruction of the bile ducts remains elusive and hence a specific therapy remains unavailable. 3 figures. 1 table. 105 references.

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Meeting the Challenge of Alcoholic Liver Disease. Patient Care. 34(11): 112-118, 124-126, 129-130. June 15, 2000.

Effective treatment options for alcoholic liver disease are limited, and most alcoholics have difficulty conquering their addiction. This article reviews the problem and management of alcoholic liver disease (ALD), focusing on long term supportive therapy that emphasizes permanent abstinence. Complications associated with liver cirrhosis (scarring of the liver, most often caused by excessive alcohol consumption) include portal hypertension, including bleeding esophageal varices (EV); ascites, which can lead to spontaneous bacterial peritonitis (SBP); and hepatic encephalopathy. The authors discuss risk factors, noting that for reasons that remain unclear, ALD develops in patients with widely varying levels of alcohol consumption. Factors contributing to the pathogenesis of ALD including amount and duration of alcohol consumption, gender, genetics, liver infection, and diet. ALD can be characterized as fatty liver, alcoholic hepatitis, and alcoholic cirrhosis. Ascites (accumulation of fluid in the abdomen) develops in about 50 percent of patients with compensated liver cirrhosis. Abstinence and long term supportive care are essential ingredients for effective patient management. Using corticosteroids to treat alcoholic hepatitis remains controversial. Liver transplantation is the only effective therapy for end stage disease. One sidebar reviews therapy options for emergent variceal bleeding; another outlines red flags for alcoholism and possible liver damage; a third provides a rationale for permitting liver transplantation in patients with ALD. 4 tables. 14 references.

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Pediatric Liver: Helping Adults by Treating Children. Clinics in Liver Disease. 4(4): 753-963. November 2000.

This issue of Clinics in Liver Disease presents indepth reviews of selected topics afflicting the pediatric (child) liver. The text begins with definition and treatment strategies for chronic cholestasis (stoppage or suppression of the flow of bile) syndromes. Topics in this section include progressive familial intrahepatic cholestatis (PFIC), Alagille syndrome (arteriohepatic dysplasia), and the surgical options used to treat biliary atresia. The next section contains extended reviews on selected metabolic disorders amenable to nontransplant treatment options. Articles in this section cover the nontransplant treatment of tyrosinemia, urea cycle disorders, and liver disease caused by disorders of bile acid synthesis. The final section covers two issues outside the spectrum of genetic and metabolic liver disease: viral hepatitis and liver transplantation. The authors recognize the highly successful use of liver transplantation to save lives of children with end stage or metabolic liver disease and discusses ongoing challenges that are encountered in the pre and perioperative periods and long term care of children with transplants. The text concludes with a subject index.

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Outcomes Following Liver Transplantation for Patients with Alcohol-Versus Nonalcohol-Induced Liver Disease. Canadian Journal of Gastroenterology. 14(10): 851-855. November 2000.

This article reports on a study undertaken to document and compare the outcomes of adult patients who received liver transplants for alcohol and nonalcohol induced liver diseases. These patients also attended a liver transplantation followup clinic in an urban, nontransplantation center at a time when no formal alcohol abuse program for transplant candidates or recipients was offered. The study population included 10 alcoholic patients and 48 nonalcoholic patients followed for an average of 41 months (range 5 to 79 months) and 46 months (range 2 to 116 months), respectively. Primary outcome variables included rates of recidivism (going back to drinking), duration of abstinence after transplantation, and compliance with posttransplant medical followup visits. Secondary outcome variables included time to discharge after transplantation, episodes of graft rejection, liver and renal biochemical abnormalities, diabetes, hypertension, sepsis, strictures, complications unrelated to transplantation, and changes in psychosocial status. Significant differences were found with respect to a higher incidence of recidivism (50 percent for alcoholic patients compared with 2 percent for nonalcoholic patients), a shorter period of abstinence after transplantation (mean of 14.7 months for alcoholic patients compared with a mean of 26.3 months for nonalcoholic patients), and more missed office visits (2.7 for alcoholic patients compared with 1.0 for nonalcoholic patients) in the alcoholic group. The alcoholic group also had a lower incidence of rejection episodes, but higher rates of posttransplantation diabetes, more nontrasplantation related complications, and higher serum creatinine but lower bilirubin and cyclosporine A levels. Marital separations were also more common in the alcoholic group. The authors conclude that, in the absence of formal alcohol abuse programs, the posttransplantation outcome in alcoholic patients generally does not compare well with that of patients who undergo transplantation for nonalcoholic related liver diseases. 3 tables. 13 references.

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Primary Sclerosing Cholangitis. Canadian Journal of Gastroenterology. 14(4): 311-315. April 2000.

This article reviews the treatment of primary sclerosing cholangitis (PSC), a chronic cholestatic liver disease, characterized by fibrosing inflammation and obliteration of intra and or extrahepatic bile ducts. The disease is one of the most common cholestatic diseases in adults and is diagnosed with increasing frequency. It is very often associated with ulcerative colitis (UC). Patients with PSC have an increased incidence of bile duct carcinomas (cancer), and those with UC also have an increased incidence of colonic carcinomas. In end stage disease, liver transplantation is the treatment of choice. Immunosuppressive treatment has little effect. Ursodeoxycholic acid (UDCA), which has been shown to improve liver histology and survival in patients with primary biliary cirrhosis, has a beneficial effect in PSC, provided that patients who develop major duct stenoses (narrowing) are treated endoscopically. The aim is to treat patients as early as possible to prevent progression to the advanced stages of the disease. During treatment with UDCA, stenoses of major ducts may develop, and early endoscopic dilation is highly effective. Because UDCA treatment improves but does not cure cholestatic liver diseases, permanent treatment seems to be necessary. Such prolonged treatment with UDCA may be recommended because, until now, no side effects have been reported. In patients with end stage disease, UDCA is not effective and liver transplantation is indicated. 3 tables. 43 references.

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Treatment of Chronic Hepatitis B Virus Infection in Special Groups of Patients: Decompensated Cirrhosis, Immunosuppressed and Paediatric Patients. Journal of Gastroenterology and Hepatology. 15(Supplement): E71-E78. May 2000.

Treatment of special groups of patients (i.e., patients with decompensated cirrhosis, immunocompromised patients, and children) is challenging and requires different treatment strategies. This article explores the management of chronic hepatitis B in these special populations. Patients with decompensated liver disease have a poor prognosis and are difficult to treat. Chances of survival for this group are limited without liver transplantation. Interferon alpha (IFN alpha) is presently the recommended treatment for patients with clinically stable chronic hepatitis B. The aim of treatment is to permanently suppress or eliminate HBV infection and thereby induce remission of liver disease. The author notes that there is increasing interest in the use of nucleoside analogues in the treatment of decompensated liver disease and those going for liver transplantation. The author discusses the use of thymosin alpha 1 and lamivudine as treatment options. Chronic hepatitis B is common in immunosuppressed patients, including antiHIV positive patients, patients with chronic renal failure, and patients undergoing organ transplantation. Unfortunately, their response to IFN therapy is poor, mostly because of high level viraemia (levels of virus in the blood) and depressed cell mediated immunity. The prevalence of hepatitis B in Asian children is probably similar to that in adults. Infection acquired early in life may not progress to liver disease until later in childhood or early adulthood. However, both cirrhosis and liver cancer (hepatocellular carcinoma, or HCC) have been documented in children. It is therefore important to consider effective therapy for children with chronic HBV infection and to monitor these children closely for HCC. 64 references.

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Update on Primary Biliary Cirrhosis. Canadian Journal of Gastroenterology. 14(1): 43-48. January 2000.

This article offers an update on primary biliary cirrhosis (PBC), a chronic inflammatory condition of the liver characterized by generalized pruritus (itching), enlargement and hardening of the liver, fatigue, weight loss, and diarrhea with pale, bulky stools. The diagnosis of PBC is most often made in the asymptomatic phase, sometimes before the development of abnormal liver biochemistry. The antimitochondrial antibody remains the predominant hallmark. The etiology (cause) of PBC remains elusive; studies suggest that the interlobular bile duct destruction is immune based, and associated autoimmune diseases are common. There are no markers that predict outcome in asymptomatic patients, whose chance of survival is less than that of age and sex matched populations, but much better than the median survival of eight years in patients with symptomatic PBC. Symptoms common in this disease include fatigue, pruritus, and xanthelasma (soft yellow spots or plaque occurring on the eyelids), as well as complications of portal hypertension (high blood pressure) and osteoporosis. Treatment includes symptomatic and preventive measures, as well as specific therapeutic measures. Immunosuppressive therapy has yielded disappointing results in the long term management of PBC, and the only therapy shown to improve survival in the hydrophobic dihydroxy bile acid, urosdeoxycholic acid. Treatment at a dose of 13 to 15 milligrams per kilogram of body weight per day is optimal, given in separate doses or as a single dose at least 4 hours from giving the oral anion exchange resin cholestyramine, which may be used to control pruritus. However, liver transplantation remains the only cure for this disease. Recurrence after transplantation takes place but is rarely symptomatic and does not deter from the benefits of transplantation. 55 references.

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Wilson's Disease. Birmingham, England: Children's Liver Disease Foundation. 2000. 8 p.

Wilson’s disease is an inherited condition in which copper is not excreted properly from the body. The excess copper can build up in the liver, the brain, or collect in other parts of the body including the eyes and the kidneys. This brochure helps parents and caregivers of children with Wilson’s disease understand the disease and the care needed for these children. The brochure explains how copper is used in the body; the signs and symptoms of Wilson’s disease; the kinds of liver disease that are caused; neurological problems that can be caused; diagnostic approaches; treatment options, such as drug therapy and liver transplantation; administration and dosage considerations; dietary therapy; lifestyle modifications; the indications for a liver transplant; genetic tests, indications for screening family members; and pregnancy in women with Wilson’s disease. The brochure also explains the work of the England-based Children’s Liver Disease Foundation, and reports on current advances in the prevention and understanding of hepatitis A. A form with which readers can donate to the Children’s Liver Disease Foundation is provided.

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Wilson's Disease. Birmingham, England: Children's Liver Disease Foundation. 2000. 8 p.

Wilson’s disease is an inherited condition in which copper is not excreted properly from the body. The excess copper can build up in the liver, the brain, or other parts of the body including the eyes and the kidneys. This brochure helps parents and caregivers of children with Wilson’s disease to understand the disease and the care for these children. The brochure explains how copper is used in the body, the signs and symptoms of Wilson’s disease, the kinds of liver disease that are caused by Wilson’s disease, neurological problems that can be caused by Wilson’s disease, diagnostic approaches, treatment options (drug therapy and liver transplantation), administration and dosage considerations, dietary therapy, lifestyle modifications, the indications for a liver transplant, genetic tests for Wilson’s disease, indications for screening family members for Wilson’s disease, and pregnancy in women with Wilson’s disease. The brochure also explains the work of the England-based Children’s Liver Disease Foundation, and reports on current advances in the prevention and understanding of hepatitis A. A form with which readers can donate to the Children’s Liver Disease Foundation is provided.

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