Open AccessAlloimmunity and Cholestasis After Liver Transplantation in Children With Progressive Familial Intrahepatic Cholestasis
Author(s) -
KrebsSchmitt Dorothee,
BriemRichter Andrea,
Brinkert Florian,
Keitel Verena,
Pukite Ieva,
Lenhartz Henning,
Fischer Lutz,
Grabhorn Enke
Publication year - 2019
Publication title -
journal of pediatric gastroenterology and nutrition
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.206
H-Index - 131
eISSN - 1536-4801
pISSN - 0277-2116
DOI - 10.1097/mpg.0000000000002200
Subject(s) - medicine , progressive familial intrahepatic cholestasis , cholestasis , alloimmunity , immunosuppression , liver transplantation , transplantation , liver disease , gastroenterology , immunology
Objectives: Bile salt export pump (BSEP) deficiency is an important reason for chronic cholestasis leading to liver transplantation (LT) in early childhood. The underlying pathology is a dysfunction of BSEP due to various mutations in the ABCB11 gene. Cases of clinical recurrence after LT due to alloantibodies directed against BSEP (antibody‐induced BSEP deficiency [AIBD]) have been reported. Most of these patients could be controlled by intensified immunosuppression. Methods: We here report on 3 children with BSEP‐deficiency and end‐stage liver disease, which developed AIBD after LT refractory to extensive immunosuppressive and immunomodulatory treatments; retransplantation was necessary in all 3 patients. In 1 patient, a stem cell transplantation was performed successfully. Results: AIBD seems to be induced by triggering factors such as initial impaired graft function or infections after LT. Conclusions: The underlying mutation may play a role in this process. Intensifying immunosuppression may be able to control AIBD, but some cases seem to be refractory to treatment and require retransplantation. Stem cell transplantation may provide a new therapeutic option for cases refractory to conservative treatment.