Chronic intrahepatic cholestasis in sickle cell disease requiring exchange transfusion.
نویسندگان
چکیده
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.
منابع مشابه
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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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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Sickle-cell intrahepatic cholestasis (SCIC) is an uncommon complication of sickle-cell disease (SCD), which can be life-threating if left untreated. We present the case of a 28-year-old man with SCD, who presented with jaundice and abdominal pain, one month after hydroxyurea discontinuation. Laboratory investigation revealed solely increased serum bilirubin, mainly conjugated, and imaging studi...
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Intrahepatic cholestasis (SCIC) is an uncommon but potentially fatal complication of sickle cell disease (SCD), with a high death rate, observed mainly in patients with homozygous sickle cell anemia. Herein, we describe a case of severe SCIC treated successfully with aggressive manual exchange transfusion (ET). The patient was admitted with enlarged liver and signs of hepatic failure, such as h...
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Sickle cell disease can lead to hepatic complications ranging from acute hepatic crises to chronic liver disease including intrahepatic cholestasis, and iron overload. Although uncommon, intrahepatic cholestasis may be severe and medical treatment of this complication is often ineffective. We report a case of a 37 year-old male patient with sickle cell anemia, who developed liver failure and un...
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ورودعنوان ژورنال:
- Gut
دوره 37 1 شماره
صفحات -
تاریخ انتشار 1995