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: Quintero, Jesús|||0000-0002-6456-9031, Juamperez, Javier|||0000-0002-8556-6374, Gonzales, Emmanuel|||0000-0002-1546-3193, Julio, Ecaterina|||0000-0002-5678-7370, Mercadal-Hally, Maria|||0000-0003-1687-6448, Collado-Hilly, Mauricette|||0000-0002-3225-086X, Marín-Sánchez, Ana|||0000-0003-2873-456X, Charco, Ramon|||0000-0001-9709-6288
Tipo de recurso: artículo
Fecha de publicación:2020
País:España
Institución:Universitat Autònoma de Barcelona
Repositorio:Dipòsit Digital de Documents de la UAB
Idioma:inglés
OAI Identifier:oai:ddd.uab.cat:227683
Acceso en línea:https://ddd.uab.cat/record/227683
https://dx.doi.org/urn:doi:10.5223/pghn.2020.23.2.174
Access Level:acceso abierto
Palabra clave:Cholestasis, progressive familial intrahepatic 2
Liver transplantation
Child
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spelling Successful Treatment with Rituximab and Immunoadsorption for an Auto-Antibody Induced Bile Salt Export Pump Deficiency in a Liver Transplanted PatientQuintero, Jesús|||0000-0002-6456-9031Juamperez, Javier|||0000-0002-8556-6374Gonzales, Emmanuel|||0000-0002-1546-3193Julio, Ecaterina|||0000-0002-5678-7370Mercadal-Hally, Maria|||0000-0003-1687-6448Collado-Hilly, Mauricette|||0000-0002-3225-086XMarín-Sánchez, Ana|||0000-0003-2873-456XCharco, Ramon|||0000-0001-9709-6288Cholestasis, progressive familial intrahepatic 2Liver transplantationChildWe 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.Universitat Autònoma de Barcelona 22020-01-0120202020-01-01Articlehttp://purl.org/coar/resource_type/c_6501VoRhttp://purl.org/coar/version/c_970fb48d4fbd8a85info:eu-repo/semantics/articleapplication/pdfhttps://ddd.uab.cat/record/227683https://dx.doi.org/urn:doi:10.5223/pghn.2020.23.2.174reponame:Dipòsit Digital de Documents de la UABinstname:Universitat Autònoma de BarcelonaInglésengopen accesshttp://purl.org/coar/access_right/c_abf2Aquest document està subjecte a una llicència d'ús Creative Commons. Es permet la reproducció total o parcial, la distribució, la comunicació pública de l'obra i la creació d'obres derivades, sempre que no sigui amb finalitats comercials, i sempre que es reconegui l'autoria de l'obra original.https://creativecommons.org/licenses/by-nc/4.0/info:eu-repo/semantics/openAccessoai:ddd.uab.cat:2276832026-06-06T12:50:31Z
dc.title.none.fl_str_mv 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
spellingShingle Successful Treatment with Rituximab and Immunoadsorption for an Auto-Antibody Induced Bile Salt Export Pump Deficiency in a Liver Transplanted Patient
Quintero, Jesús|||0000-0002-6456-9031
Cholestasis, progressive familial intrahepatic 2
Liver transplantation
Child
title_short 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_sort Successful Treatment with Rituximab and Immunoadsorption for an Auto-Antibody Induced Bile Salt Export Pump Deficiency in a Liver Transplanted Patient
dc.creator.none.fl_str_mv Quintero, Jesús|||0000-0002-6456-9031
Juamperez, Javier|||0000-0002-8556-6374
Gonzales, Emmanuel|||0000-0002-1546-3193
Julio, Ecaterina|||0000-0002-5678-7370
Mercadal-Hally, Maria|||0000-0003-1687-6448
Collado-Hilly, Mauricette|||0000-0002-3225-086X
Marín-Sánchez, Ana|||0000-0003-2873-456X
Charco, Ramon|||0000-0001-9709-6288
author Quintero, Jesús|||0000-0002-6456-9031
author_facet Quintero, Jesús|||0000-0002-6456-9031
Juamperez, Javier|||0000-0002-8556-6374
Gonzales, Emmanuel|||0000-0002-1546-3193
Julio, Ecaterina|||0000-0002-5678-7370
Mercadal-Hally, Maria|||0000-0003-1687-6448
Collado-Hilly, Mauricette|||0000-0002-3225-086X
Marín-Sánchez, Ana|||0000-0003-2873-456X
Charco, Ramon|||0000-0001-9709-6288
author_role author
author2 Juamperez, Javier|||0000-0002-8556-6374
Gonzales, Emmanuel|||0000-0002-1546-3193
Julio, Ecaterina|||0000-0002-5678-7370
Mercadal-Hally, Maria|||0000-0003-1687-6448
Collado-Hilly, Mauricette|||0000-0002-3225-086X
Marín-Sánchez, Ana|||0000-0003-2873-456X
Charco, Ramon|||0000-0001-9709-6288
author2_role author
author
author
author
author
author
author
dc.contributor.none.fl_str_mv Universitat Autònoma de Barcelona
dc.subject.none.fl_str_mv Cholestasis, progressive familial intrahepatic 2
Liver transplantation
Child
topic Cholestasis, progressive familial intrahepatic 2
Liver transplantation
Child
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.
publishDate 2020
dc.date.none.fl_str_mv 2
2020-01-01
2020
2020-01-01
dc.type.none.fl_str_mv Article
http://purl.org/coar/resource_type/c_6501
VoR
http://purl.org/coar/version/c_970fb48d4fbd8a85
dc.type.openaire.fl_str_mv info:eu-repo/semantics/article
format article
dc.identifier.none.fl_str_mv https://ddd.uab.cat/record/227683
https://dx.doi.org/urn:doi:10.5223/pghn.2020.23.2.174
url https://ddd.uab.cat/record/227683
https://dx.doi.org/urn:doi:10.5223/pghn.2020.23.2.174
dc.language.none.fl_str_mv Inglés
eng
language_invalid_str_mv Inglés
language eng
dc.rights.none.fl_str_mv open access
http://purl.org/coar/access_right/c_abf2
https://creativecommons.org/licenses/by-nc/4.0/
dc.rights.openaire.fl_str_mv info:eu-repo/semantics/openAccess
rights_invalid_str_mv open access
http://purl.org/coar/access_right/c_abf2
https://creativecommons.org/licenses/by-nc/4.0/
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv application/pdf
dc.source.none.fl_str_mv reponame:Dipòsit Digital de Documents de la UAB
instname:Universitat Autònoma de Barcelona
instname_str Universitat Autònoma de Barcelona
reponame_str Dipòsit Digital de Documents de la UAB
collection Dipòsit Digital de Documents de la UAB
repository.name.fl_str_mv
repository.mail.fl_str_mv
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