Beschreibung:
<jats:title>ABSTRACT</jats:title><jats:sec><jats:title>Objectives:</jats:title><jats:p>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 <jats:italic>ABCB11</jats:italic> 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.</jats:p></jats:sec><jats:sec><jats:title>Methods:</jats:title><jats:p>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.</jats:p></jats:sec><jats:sec><jats:title>Results:</jats:title><jats:p>AIBD seems to be induced by triggering factors such as initial impaired graft function or infections after LT.</jats:p></jats:sec><jats:sec><jats:title>Conclusions:</jats:title><jats:p>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.</jats:p></jats:sec>