Successful Treatment with Rituximab and Immunoadsorption for an Auto-Antibody Induced Bile Salt Export Pump Deficiency in a Liver Transplanted Patient.
Child
Cholestasis, progressive familial intrahepatic 2
Liver transplantation
Journal
Pediatric gastroenterology, hepatology & nutrition
ISSN: 2234-8646
Titre abrégé: Pediatr Gastroenterol Hepatol Nutr
Pays: Korea (South)
ID NLM: 101590471
Informations de publication
Date de publication:
Mar 2020
Mar 2020
Historique:
received:
04
07
2019
accepted:
12
12
2019
entrez:
25
3
2020
pubmed:
25
3
2020
medline:
25
3
2020
Statut:
ppublish
Résumé
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
Identifiants
pubmed: 32206630
doi: 10.5223/pghn.2020.23.2.174
pmc: PMC7073374
doi:
Types de publication
Case Reports
Langues
eng
Pagination
174-179Informations de copyright
Copyright © 2020 by The Korean Society of Pediatric Gastroenterology, Hepatology and Nutrition.
Déclaration de conflit d'intérêts
Conflict of Interest: The authors have no financial conflicts of interest.
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