Diverse manifestations of acute sickle cell hepatopathy in pediatric patients with sickle cell disease: A case series

Lydia H. Pecker, Nidhi Patel, Susan Creary, Anil Darbari, Emily Riehm Meier, Deepika S. Darbari, Ross M. Fasano

Research output: Contribution to journalArticle

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.

Original languageEnglish (US)
JournalPediatric Blood and Cancer
DOIs
StateAccepted/In press - Jan 1 2018

Keywords

  • Sickle cell anemia
  • Sickle cell disease
  • Sickle hepatopathy

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Hematology
  • Oncology

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