Autoimmune enteropathy and hepatitis in pediatric heart transplant recipient.
Pediatr Transplant
; 21(2)2017 Mar.
Article
em En
| MEDLINE
| ID: mdl-28097735
ABSTRACT
AIE is a rare disorder in children that presents with severe diarrhea and malabsorption, caused by immune-mediated damage to intestinal mucosa. AIE is often associated with various syndromes of immunodeficiency including IPEX syndrome (immune dysregulation, polyendocrinopathy and enteropathy, X-linked). Dysfunctional T regulatory cells are the source of pathology in both IPEX syndrome and AIE as they are essential in maintaining tolerance to self-antigens and eliminating autoreactive B cells. This case report describes a 10-year-old cardiac transplant and total thymectomy patient on chronic immunosuppression with tacrolimus that presented with AIE and extraintestinal manifestations of cyclical hepatitis. Transition from tacrolimus to sirolimus successfully increased T regulatory cells and resolved enteritis and hepatitis symptoms. Data support that thymectomy at <1 year of age increases risk of autoimmune disease due to abnormal immune maturation. Studies suggest that the sirolimus promotes the upregulation of the FoxP3 protein that is classically associated with Tregs. In turn, Tregs prevent the maturation of autoreactive B cells that lead to autoimmune reactions.
Palavras-chave
Texto completo:
1
Bases de dados:
MEDLINE
Assunto principal:
Transplante de Coração
/
Poliendocrinopatias Autoimunes
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Insuficiência Cardíaca
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Hepatite
Limite:
Child
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Female
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Humans
Idioma:
En
Revista:
Pediatr Transplant
Assunto da revista:
PEDIATRIA
/
TRANSPLANTE
Ano de publicação:
2017
Tipo de documento:
Article
País de afiliação:
Estados Unidos