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    
Abstract/OtherAbstract:
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.
Authors:
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    
Affiliation:
Department of Internal Medicine, American University of Beirut, Lebanon. dk01@aub.edu.lb
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MeSH Terms
Descriptor/Qualifier:
Adult
Family Health
Female
Humans
Lebanon
Male
Pulmonary Fibrosis* / diagnosis,  genetics,  pathology

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