Evolving practice: X-linked agammaglobulinemia and lung transplantation.
Am J Transplant
; 15(4): 1110-3, 2015 Apr.
Article
em En
| MEDLINE
| ID: mdl-25736826
ABSTRACT
X-linked agammaglobulinemia (XLA) is a rare primary humoral immunodeficiency syndrome characterized by agammaglobulinemia, recurrent infections and bronchiectasis. Despite the association with end-stage bronchiectasis, the literature on XLA and lung transplantation is extremely limited. We report a series of 6 XLA patients with bronchiectasis who underwent lung transplantation. Short-term outcomes were excellent however long-term outcomes were disappointing with a high incidence of pulmonary sepsis and chronic lung allograft dysfunction (CLAD).
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Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Transplante de Pulmão
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Agamaglobulinemia
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Doenças Genéticas Ligadas ao Cromossomo X
Limite:
Adult
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Humans
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Male
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Middle aged
Idioma:
En
Ano de publicação:
2015
Tipo de documento:
Article