Intrahepatic Cholestasis in Sickle Cell Disease: A Case Report

被引：18

作者：

Brunetta, Denise Menezes ^{[1
,2
]}

Silva-Pinto, Ana Cristina ^{[1
,2
]}

Favarin de Macedo, Maria do Carmo ^{[1
,2
]}

Bassi, Sarah Cristina ^{[1
,2
]}

Piccolo Feliciano, Joao Victor ^{[1
,2
]}

Ribeiro, Fernanda Borges ^{[1
,2
]}

Almeida Prado, Benedito de Pina, Jr. ^{[1
,2
]}

De Santis, Gil Cunha ^{[1
,2
]}

Angulo, Ivan de Lucena ^{[1
,2
]}

Covas, Dimas Tadeu ^{[1
,2
]}

机构：

[1] Univ Sao Paulo, Med Sch Ribeirao Preto, Dept Internal Med, Hematol Div, BR-14051140 Ribeirao Preto, SP, Brazil

[2] Univ Sao Paulo, Ctr Cell Based Therapy, Med Sch Ribeirao Preto, Dept Internal Med, BR-14051140 Ribeirao Preto, SP, Brazil

来源：

ANEMIA | 2011年 / 2011卷

关键词：

D O I：

10.1155/2011/975731

中图分类号：

R5 [内科学];

学科分类号：

1002 [临床医学]; 100201 [内科学];

摘要：

Intrahepatic cholestasis (SCIC) is an uncommon but potentially fatal complication of sickle cell disease (SCD), with a high death rate, observedmainly 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 hyperbilirubinemia and coagulopathy. There was no evidence of viral hepatitis or biliary obstruction. We performed several sessions of ET in order to reduce the percentage of HbS to levels inferior to 30%, which was successfully accomplished. The patient had a complete recovery of hepatic function. This case has shown that ET is an effective treatment of SCIC and should be introduced early on the onset of this severe complication.

引用

页数：3

共 14 条

[1]

Sickle cell hepatopathy: Clinical presentation, treatment, and outcome in pediatric and adult patients [J].