Document Detail

Familial idiopathic pulmonary fibrosis in three Lebanese siblings. Case report with long-term follow-up.
MedLine Citation:
PMID:  17044635     Owner:  NLM     Status:  MEDLINE    
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.
Nadim Kanj; Mouhamad Abdallah; Ayman Tawil
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Le Journal médical libanais. The Lebanese medical journal     Volume:  54     ISSN:  0023-9852     ISO Abbreviation:  J Med Liban     Publication Date:    2006 Jan-Mar
Date Detail:
Created Date:  2006-10-18     Completed Date:  2007-11-06     Revised Date:  2008-02-15    
Medline Journal Info:
Nlm Unique ID:  0375352     Medline TA:  J Med Liban     Country:  Lebanon    
Other Details:
Languages:  eng     Pagination:  45-9     Citation Subset:  IM    
Department of Internal Medicine, American University of Beirut, Lebanon.
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MeSH Terms
Family Health
Pulmonary Fibrosis* / diagnosis,  genetics,  pathology

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