Thesis (M.Sc.) - Cairo University - Faculty of Medicine - Department of Pediatrics

Background: Hepatobiliary involvement in patients with chronic hemolytic anemia is multifactorial and ranges from benign hyperbilirubinemia to potentially hepatic failure and cirrhosis. Aim & methods: a cross- sectional observational study aimed at the assessment of the prevalence of different forms of hepatobiliary disease among 100 Egyptian children with hereditary chronic hemolytic anemia (45 B-thalassemia, 40 sickle cell anemia, 15 hereditary spherocytosis), and to clarify its features and its possible underlying risk factors. Results: symptoms of hepatobiliry dysfunction included abdominal pain in 53%, hepatic symptoms in 58%, and biliary symptoms in 41%. Sixty-six percent patients had elevated AST level, 47% had elevated ALT and 58% has elevated total bilirubin. Fifty-two patients had abnormal coagulation profile and 21% of 61 subjects were HCV seropositive with hepatomegaly being the only predictor of HCV seropositivity. The prevalence of colelithiasis was 21% and 10% had biliary sludge. Cholithiasis correlated significantly with frequency and duration of transfusion. Multivariate logistic regression analysis showed that abdominal symptoms, hepatomegaly, and prolonged PT/PTT could predict biliary stones. Hepatomegaly detected in 73% patients and multivariate logistic regression analysis showed that abdominal and hepatic symptoms, prolonged PT/PTT and biliary stones could be predictors of hepatomegaly

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