Familial idopathic pulmonary fibrosis in three lebanese siblings. Case report with long-term follow-up
LMJ-Lebanese Medical Journal. 2006; 54 (1): 45-49
em Inglês
| IMEMR
| ID: emr-182745
ABSTRACT
This is the first report of a familial cluster of idiopathic pulmonary fibrosis [IPF] in Lebanon. This rare variant of IPF has an autosomal dominant mode of inheritance with variable expressivity, and is commonly associated with a mutation of the surfactant protein C gene. The patients are younger at diagnosis but have otherwise identical clinical, radiological, and histological features as the more common non-familial cases. IPF is an invariably fatal disease with no effective treatment. Lung transplantation remains the only chance for more prolonged survival and must be considered in young patients
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Índice:
IMEMR (Mediterrâneo Oriental)
Assunto principal:
Fibrose Pulmonar
/
Seguimentos
/
Transplante de Pulmão
Tipo de estudo:
Relato de Casos
Limite:
Feminino
/
Humanos
/
Masculino
Idioma:
Inglês
Revista:
Lebanese Med. J.
Ano de publicação:
2006
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