Sickle cell intrahepatic cholestasis (SCIC) is a rare complication seen in sickle cell patients who present with sudden onset of RUQ pain, progressive hepatomegaly, mild elevation of transaminases, coagulopathy, and extreme hyperbilirubinemia. Early recognition of this entity is essential to avoid life-threatening complications. Diagnosis can be challenging given the overlap in clinical presentation with other conditions affecting the hepatobiliary biliary system in sickle cell anemia such as hepatitis, cholecystitis, and hepatic crisis. Treatment is currently limited to exchange transfusion. The authors present two patients with SCIC and cholelithiasis; the clinical picture of one is complicated by choledocholithiasis.

Cytomegalovirus (CMV) is the most common congenitally acquired viral infection in the United States and is associated with significant morbidity and mortality. Primary CMV enterocolitis is well documented in immunocompromised patients, but remains rare in congenitally acquired infections. There are no universally accepted recommendations for the treatment of CMV enterocolitis in the pediatric population. Case reports show varied dosing and length of treatment of either intravenously administered ganciclovir, orally administered valganciclovir, or a combination of both. We present a congenitally infected infant with primary CMV enterocolitis who was successfully treated with orally administered valganciclovir.