Intrahepatic Cholestasis in a Sickle Cell Patient Unresponsive to Exchange Blood Transfusion

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Sickle cell intrahepatic cholestasis unresponsive to exchange blood transfusion: a case report

Sickle cell intrahepatic cholestasis (SCIC) is an uncommon but severe complication in sickle cell disease (SCD) patients homozygous for hemoglobin (Hb) S or with Hb S/ thalassemia.1–3 It is clinically characterized by marked conjugated hyperbilirubinemia, right upper quadrant pain, enlarged liver and moderately elevated hepatic enzymes. In more severe cases, coagulopathy and renal dysfunction m...

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Chronic intrahepatic cholestasis in sickle cell disease requiring exchange transfusion.

A 15 year old boy with sickle cell disease developed intrahepatic cholestasis. A course of exchange transfusion successfully corrected the extreme hyperbilirubinaemia over one year. Upon stopping the exchange transfusion programme the hyperbilirubinaemia relapsed but transfusion was effective when reinstituted.

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Exchange transfusion for intrahepatic cholestasis due to sickle beta thalassaemia.

Intrahepatic cholestasis is a rare but potentially fatal complication of sickle cell disease [1]. Sickle cell intrahepatic cholestasis (SCIC) is characterised by right upper quadrant pain, hepatomegaly and progressive hyperbilirubinaemia. To date 15 adult patients have been reported in the literature and only six survived [2]. We present a case of haemoglobin S/Beta thalassaemia with intrahepat...

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Reversal of liver function without exchange transfusion in sickle cell intrahepatic cholestasis.

Sickle cell intrahepatic cholestasis (SCIC) is a rare but fatal complication of sickle cell disease. It is found mainly in homozygous sickle cell disease. To date, there are no standard diagnostic criteria or well-established therapeutic approaches to this condition. Herein, we report this case of a 48-year-old man with sickle cell anemia and a total bilirubin of 78.5 mg/dL without evidence of ...

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Liver transplantation in a patient with acute liver failure due to sickle cell intrahepatic cholestasis.

BACKGROUND Sickle cell intrahepatic cholestasis is a potentially catastrophic complication of sickle cell anemia Once acute liver failure develops, transplantation is the only option. We describe a patient with sickle cell intrahepatic cholestasis who underwent liver transplantation. METHODS Data were obtained from the chart. Serial hemoglobin S levels were monitored, and measures were taken ...

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ژورنال

عنوان ژورنال: American Journal of Medical Case Reports

سال: 2020

ISSN: 2374-2151

DOI: 10.12691/ajmcr-7-4-4