The pattern of hepatobiliary complications among Egyptian sickle cell disease children

Background: Hepatobiliary complications are frequent among sickle cell disease patients. Sickle cell disease has been extensively studied. However, data about hepatobiliary abnormalities among the pediatric age group are limited.Objective: This study aimed at describing the pattern of hepatobiliary complications among sickle cell disease patients as detected clinically, by laboratory testing and by abdominal ultrasonography.Methods: Seventy sickle cell disease patients, aged 2–8 years, of both SS and Sβ types were enrolled in this cross-sectional study. Thorough history taking, full clinical examination, hematological and biochemical parameters assessment and abdominal ultrasonographic studies were performed to all patients.Result: The median age of the patients was 12.5 years (mean: 11.81 ± 5.1). Hepatomegaly was detected ultrasonographically in 24 patients (34.3%) and was more encountered among patients with increasing age (P = 0.003), patients with HbSβ type (P = 0.002) and high serum ferritin levels (P = 0.047). Gallbladder diseases were encountered in 19 patients (27.1%), and manifested as isolated stones in (7.1%), isolated mud in (5.7%), stones with mud in 2 patients (2.9%), and surgically removed stones in (11.4%). Cholecystectomy was done in 8 patients (11.4%). Gallbladder disease was more frequent among patients with older age (P = 0.041), more severe sickle cell disease attacks (P = 0.035), and longer duration of blood transfusion therapy (P = 0.041). Other hepatobiliary complications were chronic hepatitis C (12.9%), benign hyperbilirubinemia (1.4%), intrahepatic cholestasis (1.4%), hepatic sequestration(1.4%), hepatic crisis (1.4%), and liver cirrhosis (1.4%).Conclusion: Hepatobiliary complications are frequent among sickle cell disease children especially gallbladder diseases. Repeated clinical and ultrasound screening is needed for early detection and treatment of these complications