Intrahepatic cholestasis in sickle cell disease: A review of diagnostic criteria, treatments, and case reports

Christopher L. Edwards, Sharena Scott, Michaela Boggan, Jordan Meek, Kiera Alston, Aiden Pearson, Alexandria McDougald, McKenzie Broadnax, Mary Wood, Camela S. Barker, Jessica Miller, Elaine Whitworth, Osaffo James, John J. Sollers  III, W. Jeff Bryson, Roland Thorpe, Goldie Byrd, Keith E. Whitfield, Shiv Sudhakar, Debra O. ParkerJonathan Livingston, Nirmish Shah, Kenyon Railey

Research output: Contribution to journalReview articlepeer-review


Objective To delineate the etiology, symptomatology, and treatment of sickle cell intrahepatic cholestasis (SCIC). Sickle cell disease (SCD) is the most frequently inherited hematologic disease, and SCIC is one rare and often fatal complication and comorbid disease. The literature contains only a small number of case reports involving SCIC and hence limited guidance can be obtained. Methods We reviewed the scientific literature to evaluate the science of SCIC to determine if there were consistencies in presentation, evaluation, treatment, and clinical outcomes. Results We reviewed 6 case reports and a limited number of clinical papers on SCIC. We reported consistencies in clinical presentation and treatment outcomes among cases as well as serological and hematological finding. Conclusions While there is some consistency in the symptom presentation of individuals with SCIC, reliable evaluation and clinical procedures were not demonstrated in what we reviewed. Further research is needed to delineate the attributes of this complicated disease that occurs within SCD.

Original languageEnglish (US)
Pages (from-to)26-37
Number of pages12
JournalJournal of the National Medical Association
Issue number1
StatePublished - Feb 2023


  • Intrahepatic cholestasis
  • Rare
  • Sickle cell disease
  • Thalassemia

ASJC Scopus subject areas

  • General Medicine


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