Sickle cell intrahepatic cholestasis with cholelithiasis

Karina Irizarry, Hans Christof Rossbach, Joseph R.A. Ignacio, Michele P. Winesett, Greg C. Kaiser, Mudra Kumar, Enid Gilbert-Barness, Michael J. Wilsey

Research output: Contribution to journalArticlepeer-review

6 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)95-102
Number of pages8
JournalPediatric Hematology and Oncology
Issue number2
StatePublished - Mar 1 2006
Externally publishedYes


  • Cholecystitis
  • Cholelithiasis
  • Hepatic crisis
  • Hepatitis
  • Intrahepatic cholestasis
  • Sickle cell disease

ASJC Scopus subject areas

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


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