The pattern of hepatobiliary complications among Egyptian sickle cell disease children

BackgroundHepatobiliary 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.ObjectiveThis study aimed at describing the pattern of hepatobiliary complications among sickle cell disease patients as detected clinically, by laboratory testing and by abdominal ultrasonography.MethodsSeventy 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.ResultThe 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%).ConclusionHepatobiliary 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.