Hyperbilirubinemia in Gilbert syndrome and hepatitis B infection: Two confusing clinical cases accompanied by hereditary spherocytosis

Gilbert’s syndrome (GS) is a benign disorder that causes chronic jaundice and unconjugated hyperbilirubinemia. When it occurs in conjunction with hereditary spherocytosis (HS), it can lead to an elevated risk of severe hyperbilirubinemia and associated complications. However, acute pancreatitis is a rare complication in this setting. This study explores complex etiologies in severe hyperbilirubinemia and pancreatitis through two acute pancreatitis cases. One case involves a patient with HS and hepatitis B, and the other is of GS with HS contributing to the condition. Two pediatric patients with severe hyperbilirubinemia and acute pancreatitis, managed at different time points in our pediatric gastroenterology clinic, were presented. GS in one case and hepatitis B infection in the other were considered contributing factors but did not fully explain the disease’s severity. A systematic review of clinical, laboratory, and imaging findings identified hereditary spherocytosis as the common underlying condition. A 16-year-old boy with consanguineous parents presented with recurrent abdominal pain and jaundice. Laboratory and imaging findings indicated acute pancreatitis due to gallstones, extreme hyperbilirubinemia, and HS with GS. Genetic testing confirmed GS (UGT1A polymorphism). ERCP relieved the obstruction, and the patient was referred for cholecystectomy and splenectomy. The other boy, a 14-year-old with chronic hepatitis B, presented with acute abdominal pain. Imaging revealed gallstones, splenomegaly, and acute pancreatitis. Laboratory findings suggested HS, confirmed by osmotic fragility testing. MRCP excluded bile duct obstruction. Following stabilization, laparotomic cholecystectomy and splenectomy were performed without complications. We present two pediatric cases with distinct etiologies who developed severe hyperbilirubinemia and acute pancreatitis in the presence of hereditary spherocytosis. These cases highlight the complex interplay of underlying conditions contributing to disease severity. By adding to the limited literature on this association, we aim to enhance understanding of the diverse etiologies of severe hyperbilirubinemia and acute pancreatitis.

Keywords: Acute pancreatitis, Gilbert syndrome, hepatitis, hereditary spherocytosis, hyperbilirubinemia.