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4 Simple or exchange transfusion for sepsis, acidosis, or hypoxia Admission (consideration of outpatient management in stable patients with isolated fever) Management/Diagnostic Testing If the patient describes pain typical in location and quality to a vasoocclusive crisis, begin fluids, provide analgesia, and observe the patient for improvement Elevated aspartate aminotransferase (AST) and hyperbilirubinemia may be present in infarction as well as biliary disease Abdominal ultrasound can assess the anatomy of the liver, gall bladder, and biliary tree Cholelithiasis is the most common hepatic and biliary tract complication in children with sickle cell disease, with an incidence of 12% in 2- to 5-year olds and approximately 40% by the age of 15 to 18 years Patients can present with acute right upper quadrant pain and tenderness, hyperbilirubinemia, and elevated liver enzyme levels Typically, surgery is delayed until acute inflammation has subsided to reduce the risk of complications The optimal treatment includes elective laparoscopic cholecystectomy after adequate preparation for surgery (e.g., transfusions) Rarely, acute intrahepatic sickling or viral hepatitis can result in a similar clinical presentation with massive hyperbilirubinemia and elevated enzyme levels Fulminant hepatic failure with hepatic encephalopathy and shock can also occur as a rare, often fatal, syndrome that may be amenable to exchange transfusion Priapism Initial Assessment Priapism is a painful vasoocclusive crisis causing a tender and engorged penis that may persist for hours Urination may be difficult Management/Diagnostic Testing Treatment is similar to other painful crises with management directed at hydration and analgesia A number of systemic therapies can also be utilized Pseudoephedrine can be given orally, while terbutaline delivery is either oral or subcutaneous Terbutaline has been studied for the treatment of priapism in nonsickle cell disease patients and has some demonstrated efficacy in the sickle cell disease group Consider early consultation with a pediatric hematologist and urologist Infection/Sepsis

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