Brief Report: Recurrence of Bile Salt Export Pump Deficiency after Liver Transplantation.

被引：71

作者：

Jara, Paloma ^{[2
]}

Hierro, Loreto ^{[2
]}

Martinez-Fernandez, Pilar

Alvarez-Doforno, Rita ^{[3
]}

Yanez, Francisca ^{[3
]}

Diaz, Maria C. ^{[2
]}

Camarena, Carmen ^{[2
]}

De la Vega, Angela ^{[2
]}

Frauca, Esteban ^{[2
]}

Munoz-Bartolo, Gema ^{[2
]}

Lopez-Santamaria, Manuel ^{[5
]}

Larrauri, Javier ^{[4
]}

Alvarez, Luis ^{[1
]}

机构：

[1] La Paz Univ Hosp, FIBHULP, Res Unit, Madrid 28046, Spain

[2] La Paz Univ Hosp, Pediat Liver Serv, Madrid 28046, Spain

[3] La Paz Univ Hosp, Serv Immunol, Madrid 28046, Spain

[4] La Paz Univ Hosp, Dept Pathol, Madrid 28046, Spain

[5] La Paz Univ Hosp, Dept Pediat Surg, Madrid 28046, Spain

来源：

NEW ENGLAND JOURNAL OF MEDICINE | 2009年 / 361卷 / 14期

关键词：

FAMILIAL INTRAHEPATIC CHOLESTASIS; RECEPTOR; HEPATOCYTES; EXPRESSION; TRANSPORT; CHILDREN; SISTER; MICE; IGG;

D O I：

10.1056/NEJMoa0901075

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Severe bile salt export pump (BSEP) deficiency is a hereditary cholestatic condition that starts in infancy and leads to end-stage liver disease. Three children who underwent orthotopic liver transplantation for severe BSEP deficiency had post-transplantation episodes of cholestatic dysfunction that mimicked the original disease. Remission of all episodes was achieved by intensifying the immunosuppressive regimen. The phenotypic recurrence of the disease correlated with the presence of circulating high-titer antibodies against BSEP that inhibit transport by BSEP in vitro. When administered to rats, these antibodies targeted the bile canaliculi and impaired bile acid secretion.

引用

页码：1359 / 1367

页数：9

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