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Hepatobiliary system in sickle cell disease.

T T Schubert

    Gastroenterology
    |June 1, 1986
    PubMed
    Summary

    Sickle cell disease (SCD) can lead to progressive liver damage, cirrhosis, and acute hepatic crisis. Differentiating SCD liver complications from viral hepatitis and gallstones requires careful diagnosis.

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    Area of Science:

    • Hepatology
    • Hematology
    • Internal Medicine

    Background:

    • Sickle cell disease (SCD) is a genetic blood disorder with significant implications for the hepatobiliary system.
    • Patients with SCD often develop progressive liver injury, fibrosis, and cirrhosis by adulthood.
    • Hepatomegaly and elevated liver enzymes are common findings in asymptomatic SCD patients.

    Purpose of the Study:

    • To review literature on sickle cell disease and its impact on the hepatobiliary system.
    • To discuss the differential diagnosis of liver complications in SCD patients.
    • To highlight the challenges in managing hepatobiliary issues in the context of SCD.

    Main Methods:

    • Literature review of published reports on sickle cell disease and the hepatobiliary system.
    • Analysis of clinical and laboratory features of SCD-related liver complications.
    • Comparison with differential diagnoses such as viral hepatitis and gallstones.

    Main Results:

    • SCD can cause chronic liver injury, fibrosis, and decreased function.
    • Acute hepatic crisis occurs in 10% of patients, presenting with jaundice and elevated transaminases.
    • Gallstones affect 50-70% of adult SCD patients; surgical management is debated for asymptomatic cases.
    • Nine cases of acute hepatic failure in SCD patients suggest a potential metabolic etiology.

    Conclusions:

    • Sickle cell disease poses a significant risk for hepatobiliary complications, including chronic liver disease and acute hepatic crisis.
    • Distinguishing SCD liver issues from viral hepatitis and cholelithiasis is crucial and may require serology and biopsy.
    • Management of gallstones in SCD patients requires careful consideration of risks and benefits.

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