ArticleThe American journal of case reports2015
Rare but Lethal Hepatopathy-Sickle Cell Intrahepatic Cholestasis and Management Strategies.
Article in The American journal of case reports, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 12 papers, 1 of them a synthesis that pooled it.
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Who cites it
12 citing papers in PubMed, 1 synthesis or guideline pooled it, 24 citations in OpenAlex.
- Interventions for treating intrahepatic cholestasis in people with sickle cell disease.The Cochrane database of systematic reviews · 2017Pooled it
- Molecular mechanisms of hepatic dysfunction in sickle cell disease: lessons from Townes mouse model.American journal of physiology. Cell physiology · 2022Review
- Fulminant dengue hepatitis in sickle cell disease: Recovery against the odds.Journal of family medicine and primary care · 2022Article
- Pediatric Acute Liver Failure in Sickle Cell Disease.GE Portuguese journal of gastroenterology · 2022Article
- Sickle Cell Intrahepatic Cholestasis: Extremely Rare but Fatal Complication of Sickle Cell Disease.Cureus · 2022Article
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- Article
- Dengue fever as a potential cause of sickle cell intrahepatic cholestasis: A report of two cases.Revista da Sociedade Brasileira de Medicina Tropical · 2021Article
- Successful liver transplantation for acute sickle cell intrahepatic cholestasis: A case report and review of the literature.World journal of hepatology · 2020Article
- Management of liver complications in sickle cell disease.Hematology. American Society of Hematology. Education Program · 2019Review
- Management of Sickle Cell Intrahepatic Cholestasis: An Argument in Favor of Automated Exchange Transfusion.Clinical hematology international · 2019Article
- Sickle Hepatopathy.Journal of clinical and experimental hepatologyReview
Corrections and comments
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Authors and funding
4 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundSickle cell disease can affect the liver by way of the disease process, including sickling in hepatic sinusoids, as well as its treatment, including repeated blood transfusions leading to hemosiderosis and hepatitis. Sickle cell intrahepatic cholestasis (SCIC) is an extreme variant of sickle cell hepatopathy, and is associated with high fatality. CASE REPORT: We present the case of a 31-year-old man with past medical history of sickle cell disease and cholecystectomy who was admitted with uncomplicated vaso occlusive crisis and during the hospital stay developed fever, upper abdominal pain, and jaundice. There was an accelerated rise in total bilirubin to 50 mg/dL, direct bilirubin 38 mg/dL, and Cr 3.0 mg/dL. Hb was 6.4 g/dL, reticulocyte count 16%, ALT 40 IU/L, AST 155 IU/L, ALP 320 IU/L, and LDH 475 IU/L. Hepatitis panel was negative and MRCP showed normal caliber of the common bile duct, with no obstruction. Exchange transfusion of 9 units of packed red blood cells led to great improvement in his condition.
conclusionsSCIC, unlike the other sickle cell hepatopathies, requires urgent and vigorous exchange transfusion. Renal impairment in SCIC has not been well studied but usually is reversible with the hepatic impairment, as in this case. Unresolved renal impairment requires dialysis and is associated with poor outcome. There is limited data on use of hydroxyurea to prevent SCIC, and liver transplant is associated with high mortality. A timely diagnosis of SCIC and appropriate management is life-saving.
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