Objective: to describe clinical, laboratory and ultrasonographic abnormalities in the hepatobiliary system of pediatric patients with sickle cell disease in the city of Salvador, Brazil.

Material And Methods: pediatric patients with sickle cell disease were clinically evaluated, their charts were reviewed and findings of supplementary tests were examined to identify hepatobiliary abnormalities.

Results: a total of 134 patients were evaluated, 65 of whom (48.9%) presented hepatomegalia. Elevated transaminases were present in 42.2% and 11.4% presented cholelithiasis. There was a statistically significant association between the presence of hepatomegalia and SS homozygotes and between cholelithiasis and patients over 10 years of age (p = 0.01 and p = 0.00, respectively).

Conclusion: hepatobiliary abnormalities in patients with sickle cell disease were common, particularly in patients with hemoglobin SS and in adolescent patients.

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