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Mild hereditary spherocytosis: decision analysis assesses surgery.

Marchetti M, Barosi G, Quaglini S; International Society of Technology Assessment in Health Care. Meeting.

Annu Meet Int Soc Technol Assess Health Care Int Soc Technol Assess Health Care Meet. 1997; 13: 121.

Laboratory of Medical Informatics, I.R.C.S.S. Policlinico S. Matteo, Pavia, Italy.

PURPOSE: The appropriate management of patients with mild hereditary spherocytosis (HS) is still a debatable matter: splenectomy is widely recommended for patietns with HS, but no consensus was achieved for the 20-30% patients with mild features of the disease. Splenectomy highly reduces the occurence of pigment gallstones and is curative for hemolytic and aplastic crisis, but it carries a high risk for sepsis. Cholecystectomy is mandatory at splenectomy if the patient carries gallstones. METHODS: Evidence was collected to frame a decision-analystic model facing the competing risks of prophylactic surgery, gallstones, sepsis and hemolysis. Four strategies were analized: cholecystectomy alone, splenectomy alone, contomitant cholecystectomy during splenectomy and none. Prophylaxis for sepsis was also modelled: effectiveness of a complex strategy including vacination for both Pneumococcus and H. influenzae and oral penicillin was included along with compliance. The clinical course of patients was modelled by a Markov tree. Three scenarios were scrutinized: patients without gallstones, patients with asymptomatic gallstones and symptomatic patients. Original meta-analysis were performed to gain the incidence of post-splenectomy sepsis and the prevalence of gallstones in HS. Life expectancy was adjusted for the quality of life and future life years were discounted at a 3% yearly rate. The quality of life was evaluated by both adjusting factors and by tolls. RESUTLS: Cholecystectomy is appropriate for both patients with asymptomatic gallstones and for patients with no evidence of gallstones, if aged less than 44. Lower mortality rates, such as those carried by laparoscopic procedures, can amplify the marginal benefit of cholecystectomy and splenectomy in respect to the strategy of not performing any surgical act. CONCLUSIONS: The decision model proposed, by framing both time and compliance, showed that the immediate risks of cholecystectomy are outweighted by the future benefits and thus supports the appropriateness of the surgical intervention in a patient with a diagnosis of mild hereditary spherocytosis. Splenectomy, on converse, carries too high risks. Critical parameters were identified which allow to shape the decision on the individual patient.

Publication Types:
  • Meeting Abstracts
Keywords:
  • Cholecystectomy
  • Cholelithiasis
  • Decision Support Techniques
  • Evaluation Studies
  • Hemolysis
  • Humans
  • Incidence
  • Laparoscopy
  • Life Expectancy
  • Spherocytosis, Hereditary
  • Splenectomy
  • immunology
  • methods
  • surgery
  • hsrmtgs
Other ID:
  • HTX/98601688
UI: 102233233

From Meeting Abstracts




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