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Predicate | Object |
---|---|
rdf:type | |
lifeskim:mentions | |
pubmed:issue |
4
|
pubmed:dateCreated |
1992-5-21
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pubmed:abstractText |
Dyskeratosis congenita (DC) is an unusual familial disorder primarily affecting the skin and its appendages. We report the case of a DC patient with chronic respiratory tract involvement, confirming the features previously reported by a small number of authors: 1) chronic bronchoalveolar involvement is not unusual in this disorder; 2) the main features are early sputum production with subsequent bronchial and alveolar destruction; 3) after onset of dyspnoea the course is rapidly fatal, with progressive respiratory failure. Immune deficiency and repeated bronchoalveolar infections may be involved in the pathogenesis of these manifestations.
|
pubmed:language |
eng
|
pubmed:journal | |
pubmed:citationSubset |
IM
|
pubmed:status |
MEDLINE
|
pubmed:month |
Apr
|
pubmed:issn |
0903-1936
|
pubmed:author | |
pubmed:issnType |
Print
|
pubmed:volume |
5
|
pubmed:owner |
NLM
|
pubmed:authorsComplete |
Y
|
pubmed:pagination |
497-9
|
pubmed:dateRevised |
2004-11-17
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pubmed:meshHeading |
pubmed-meshheading:1563509-Adult,
pubmed-meshheading:1563509-Bone Marrow Diseases,
pubmed-meshheading:1563509-Bronchiectasis,
pubmed-meshheading:1563509-Humans,
pubmed-meshheading:1563509-Male,
pubmed-meshheading:1563509-Nails, Malformed,
pubmed-meshheading:1563509-Pigmentation Disorders,
pubmed-meshheading:1563509-Pulmonary Fibrosis
|
pubmed:year |
1992
|
pubmed:articleTitle |
Bronchoalveolar disease in dyskeratosis congenita.
|
pubmed:affiliation |
INSERM unit 139, Centre Hospitalier Intercommunal, Creteil, France.
|
pubmed:publicationType |
Journal Article,
Case Reports
|