잠시만 기다려 주세요. 로딩중입니다.

Successful Treatment with Rituximab and Immunoadsorption for an Auto-Antibody Induced Bile Salt Export Pump Deficiency in a Liver Transplanted Patient

Quintero Jesus, Juamperez Javier, Gonzales Emmanuel, Julio Ecaterina, Mercadal-Hally Maria, Collado-Hilly Mauricette, Marin-Sanchez Ana, Charco Ramon,
소속 상세정보
 ( Quintero Jesus ) - Universitat Autonoma de Barcelona Vall d’Hebron University Hospital Pediatric Hepatology and Liver Transplantation Unit
 ( Juamperez Javier ) - Universitat Autonoma de Barcelona Vall d’Hebron University Hospital Pediatric Hepatology and Liver Transplantation Unit
 ( Gonzales Emmanuel ) - Universite Paris-Saclay Inserm Unite Mixte de Recherche 1193
 ( Julio Ecaterina ) - Universitat Autonoma de Barcelona Vall d’Hebron University Hospital Pediatric Hepatology and Liver Transplantation Unit
 ( Mercadal-Hally Maria ) - Universitat Autonoma de Barcelona Vall d’Hebron University Hospital Pediatric Hepatology and Liver Transplantation Unit
 ( Collado-Hilly Mauricette ) - Universite Paris-Saclay Inserm Unite Mixte de Recherche 1193
 ( Marin-Sanchez Ana ) - Universitat Autonoma de Barcelona Vall d’Hebron University Hospital Department of Immunology
 ( Charco Ramon ) - Universitat Autonoma de Barcelona Vall d’Hebron University Hospital Department of HPB Surgery and Trasplant

Abstract


We present an 8 years old girl who was diagnosed at 6 months of age of Progressive Familial Intrahepatic Cholestasis type 2. Although liver transplantation (LT) was classically considered curative for these patients, cholestasis recurrence with normal gamma-glutamyl transpeptidase (GGT), mediated by anti-bile salt export pump (BSEP) antibodies after LT (auto-antibody Induced BSEP Deficiency, AIBD) has been recently reported. Our patient underwent LT at 14 months. During her evolution, patient presented three episodes of acute rejection. Seven years after the LT, the patient presented pruritus with cholestasis and elevation of liver enzymes with persistent normal GGT. Liver biopsy showed intrahepatic cholestasis and giant-cell transformation with very low BSEP activity. Auto-antibodies against BSEP were detected therefore an AIBD was diagnosed. She was treated with Rituximab and immunoadsorption with resolution of the AIBD. As a complication of the treatment she developed a pneumocystis infection successfully treated with corticoids, cotrimoxazol and anidulafungin.

키워드

Cholestasis, progressive familial intrahepatic 2; Liver transplantation; Child

원문 및 링크아웃 정보

 

등재저널 정보