Alloimmunity and Cholestasis After Liver Transplantation in Children With Progressive Familial Intrahepatic Cholestasis.
J Pediatr Gastroenterol Nutr
; 68(2): 169-174, 2019 Feb.
Article
em En
| MEDLINE
| ID: mdl-30664572
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.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Colestase Intra-Hepática
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Transplante de Fígado
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Doença Hepática Terminal
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Anticorpos
Limite:
Child, preschool
/
Female
/
Humans
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Infant
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Male
Idioma:
En
Revista:
J Pediatr Gastroenterol Nutr
Ano de publicação:
2019
Tipo de documento:
Article