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

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...

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Autores principales: Quintero, Jesús, Juamperez, Javier, Gonzales, Emmanuel, Julio, Ecaterina, Mercadal-Hally, Maria, Collado-Hilly, Mauricette, Marín-Sánchez, Ana, Charco, Ramon
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Korean Society of Pediatric Gastroenterology, Hepatology and Nutrition 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7073374/
https://www.ncbi.nlm.nih.gov/pubmed/32206630
http://dx.doi.org/10.5223/pghn.2020.23.2.174
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author Quintero, Jesús
Juamperez, Javier
Gonzales, Emmanuel
Julio, Ecaterina
Mercadal-Hally, Maria
Collado-Hilly, Mauricette
Marín-Sánchez, Ana
Charco, Ramon
author_facet Quintero, Jesús
Juamperez, Javier
Gonzales, Emmanuel
Julio, Ecaterina
Mercadal-Hally, Maria
Collado-Hilly, Mauricette
Marín-Sánchez, Ana
Charco, Ramon
author_sort Quintero, Jesús
collection PubMed
description 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.
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spelling pubmed-70733742020-03-23 Successful Treatment with Rituximab and Immunoadsorption for an Auto-Antibody Induced Bile Salt Export Pump Deficiency in a Liver Transplanted Patient Quintero, Jesús Juamperez, Javier Gonzales, Emmanuel Julio, Ecaterina Mercadal-Hally, Maria Collado-Hilly, Mauricette Marín-Sánchez, Ana Charco, Ramon Pediatr Gastroenterol Hepatol Nutr Case Report 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. The Korean Society of Pediatric Gastroenterology, Hepatology and Nutrition 2020-03 2020-03-04 /pmc/articles/PMC7073374/ /pubmed/32206630 http://dx.doi.org/10.5223/pghn.2020.23.2.174 Text en Copyright © 2020 by The Korean Society of Pediatric Gastroenterology, Hepatology and Nutrition https://creativecommons.org/licenses/by-nc/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (https://creativecommons.org/licenses/by-nc/4.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Quintero, Jesús
Juamperez, Javier
Gonzales, Emmanuel
Julio, Ecaterina
Mercadal-Hally, Maria
Collado-Hilly, Mauricette
Marín-Sánchez, Ana
Charco, Ramon
Successful Treatment with Rituximab and Immunoadsorption for an Auto-Antibody Induced Bile Salt Export Pump Deficiency in a Liver Transplanted Patient
title Successful Treatment with Rituximab and Immunoadsorption for an Auto-Antibody Induced Bile Salt Export Pump Deficiency in a Liver Transplanted Patient
title_full Successful Treatment with Rituximab and Immunoadsorption for an Auto-Antibody Induced Bile Salt Export Pump Deficiency in a Liver Transplanted Patient
title_fullStr Successful Treatment with Rituximab and Immunoadsorption for an Auto-Antibody Induced Bile Salt Export Pump Deficiency in a Liver Transplanted Patient
title_full_unstemmed Successful Treatment with Rituximab and Immunoadsorption for an Auto-Antibody Induced Bile Salt Export Pump Deficiency in a Liver Transplanted Patient
title_short Successful Treatment with Rituximab and Immunoadsorption for an Auto-Antibody Induced Bile Salt Export Pump Deficiency in a Liver Transplanted Patient
title_sort successful treatment with rituximab and immunoadsorption for an auto-antibody induced bile salt export pump deficiency in a liver transplanted patient
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7073374/
https://www.ncbi.nlm.nih.gov/pubmed/32206630
http://dx.doi.org/10.5223/pghn.2020.23.2.174
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