RESUMO
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.