Diverse manifestations of acute sickle cell hepatopathy in pediatric patients with sickle cell disease: A case series |
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| Authors: | Lydia H. Pecker Nidhi Patel Susan Creary Anil Darbari Emily Riehm Meier Deepika S. Darbari Ross M. Fasano |
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| Affiliation: | 1. Division of Hematology, Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, Maryland;2. Department of Pediatrics, Children's Hospital of Los Angeles, Los Angeles, California;3. Center for Innovation in Pediatric Practice, Division of Hematology/Oncology/BMT, Nationwide Children's Hospital, Columbus, Ohio;4. Division of Gastroenterology, Hepatology, and Nutrition, Children's National Health System, Washington, District of Columbia;5. Indiana Hemophilia and Thrombosis Center, Indianapolis, Indiana;6. Division of Hematology, Children's National, Department of Pediatrics, George Washington University School of Medicine and Health Sciences, Washington, District of Columbia;7. Center for Transfusion and Cellular Therapies, Department of Pathology and Laboratory Medicine, Emory University School of Medicine, Atlanta, Georgia |
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| Abstract: | The hepatic complications of sickle cell disease (SCD) are associated with increased morbidity and mortality in adults; children usually survive but may suffer significant sequelae. Few diagnostic tools differentiate the various hepatic manifestations of SCD. Why patients exhibit one hepatic pathology versus another is unclear. We report four pediatric patients with hemoglobin SS disease with diverse manifestations of acute hepatic involvement including acute sickle hepatic crisis, hepatic sequestration, sickle cell intrahepatic cholestasis, and a non‐SCD cause of hepatopathy in a patient with viral hepatitis. These complications require a systematic approach to extensive evaluation and coordinated multidisciplinary care. |
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| Keywords: | sickle cell anemia sickle cell disease sickle hepatopathy |
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