Alloimmunity and Cholestasis After Liver Transplantation in Children With Progressive Familial Intrahepatic Cholestasis

Dorothee Krebs-Schmitt, Andrea Briem-Richter, Florian Brinkert, Verena Keitel, Ieva Pukite, Henning Lenhartz, Lutz Fischer, Enke Grabhorn

Research output: Contribution to journalArticlepeer-review

2 Citations (Scopus)

Abstract

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.
Original languageEnglish
Pages (from-to)169-174
JournalJournal of Pediatric Gastroenterology and Nutrition
Volume68
Issue number2
DOIs
Publication statusPublished - Feb 2019
Externally publishedYes

Keywords*

  • antibody-induced bile salt export pump deficiency
  • cholestasis
  • pediatric liver transplantation
  • progressive familial intrahepatic cholestasis
  • stem cell transplantation

Field of Science*

  • 3.2 Clinical medicine

Publication Type*

  • 1.1. Scientific article indexed in Web of Science and/or Scopus database

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