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Sickle Hepatopathy.

Dibya L Praharaj1, Anil C Anand1

  • 1Department of Gastroenterology & Hepatology, Kalinga Institute of Medical Sciences, Bhubneshwar, India.

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|March 8, 2021
PubMed
Summary
This summary is machine-generated.

Sickle hepatopathy, liver injury in sickle cell disease, is prevalent in India. This review covers its epidemiology, pathogenesis, and management including exchange transfusion and liver transplantation.

Keywords:
AASLD, American Association for the Study of Liver DiseasesACLF, Acute on chronic liver failureALF, Acute liver failureALT, Alanine transaminaseAST, Aspartate transaminaseFFP, Fresh frozen plasmaGIT, Gastrointestinal tractHAV, Hepatitis A virusHBV, Hepatitis B virusHCV, Hepatitis C virusHEV, Hepatitis E virusHIC, Hepatic iron contentHbS, Sickle hemoglobinHbSS, Sickle cell disease homozygousINR, International normalized ratioPT, Prothrombin timeRUQ, Right upper quadrantSC, Scheduled casteSCD, Sickle cell diseaseSCIC, Sickle cell intrahepatic cholestasisST, Scheduled tribeTJLB, Transjugular liver biopsyUDCA, Ursodeoxycholic acidcholelithiasisintrahepatic cholestasissickle cell hepatopathysickle cholangiopathysickle hepatic crisis

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Area of Science:

  • Hepatology
  • Hematology
  • Genetics

Background:

  • Sickle hepatopathy encompasses diverse liver injuries in sickle cell disease (SCD) patients.
  • India has a significant SCD prevalence, second only to Sub-Saharan Africa.
  • Liver disease in SCD arises from viral hepatitis, iron overload, or vascular changes.

Purpose of the Study:

  • To review the epidemiology of SCD in India.
  • To elucidate the pathogenesis and classification of hepatobiliary involvement in SCD.
  • To discuss management strategies for hepatic sequestration syndromes.

Main Methods:

  • Literature review focusing on sickle hepatopathy in the Indian context.
  • Analysis of disease spectrum from ischemic injury to acute liver failure.
  • Examination of current and potential therapeutic interventions.

Main Results:

  • Sickle hepatopathy presents with ischemic injury, gallstones, and sequestration syndromes.
  • Severe episodes can lead to acute liver failure, requiring urgent interventions like exchange transfusion or transplantation.
  • Evidence for optimal management of these severe events remains limited.

Conclusions:

  • Understanding SCD epidemiology in India is crucial for addressing sickle hepatopathy.
  • Management of severe hepatic sequestration requires further research regarding efficacy and indications for interventions.
  • Exploring novel therapeutic approaches like hydroxyurea for hepatic complications is warranted.