Diverse manifestations of acute sickle cell hepatopathy in pediatric patients with sickle cell disease: A case series. Issue 8 (18th April 2018)

• Record Type: Journal Article
• Authors: Pecker, Lydia H.; Patel, Nidhi; Creary, Susan; Darbari, Anil; Meier, Emily Riehm; Darbari, Deepika S.; Fasano, Ross M.
• Is Part Of: Pediatric blood & cancer. Volume 65:Issue 8(2018)
• Page Start: n/a
• Page End: n/a
• Publication Date: 2018-04-18
• Subjects: sickle cell anemia -- sickle cell disease -- sickle hepatopathy; Tumors in children -- Periodicals; Blood -- Diseases -- Periodicals; Cancer in children -- Periodicals; 618.92
• DOI: 10.1002/pbc.27060
• Languages: English
• ISSNs: 1545-5009

Abstract

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.