Document Detail

Usual interstitial pneumonia complicating dyskeratosis congenita.
MedLine Citation:
PMID:  15945534     Owner:  NLM     Status:  MEDLINE    
Dyskeratosis congenita (DC) is a rare disorder characterized by skin hyperpigmentation, nail dystrophy, and leukoplakia of mucous membranes. Pulmonary complications occur in approximately 20% of patients, although the specific histopathologic features, the temporal relationship between the diagnosis of DC and the development of pulmonary fibrosis, and the response to specific treatment are largely undefined. We describe 2 patients with DC who developed usual interstitial pneumonia. Pulmonary fibrosis developed 18 and 38 years after the original manifestations of DC. Both patients died of respiratory failure, 4 and 6 months after lung biopsy. Pulmonary fibrosis in patients with DC may be linked to underlying abnormalities of fibroblast function.
James P Utz; Jay H Ryu; Jeffrey L Myers; Virginia V Michels
Related Documents :
17882044 - Pulmonary and sinusal changes in 45 patients with primary immunodeficiencies: computed ...
6391944 - Pulmonary function of marrow transplant patients. i. effects of marrow infusion, acute ...
18931474 - Induced sputum in systemic sclerosis interstitial lung disease: comparison to healthy c...
17106154 - Clinical characteristics of obesity-hypoventilation syndrome in japan: a multi-center s...
766894 - Diverticular disease: threee studies. part ii - treatment with bran.
1307904 - Myocardial ischemia during the weaning period.
Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Mayo Clinic proceedings     Volume:  80     ISSN:  0025-6196     ISO Abbreviation:  Mayo Clin. Proc.     Publication Date:  2005 Jun 
Date Detail:
Created Date:  2005-06-10     Completed Date:  2005-06-21     Revised Date:  2013-12-13    
Medline Journal Info:
Nlm Unique ID:  0405543     Medline TA:  Mayo Clin Proc     Country:  United States    
Other Details:
Languages:  eng     Pagination:  817-21     Citation Subset:  AIM; IM    
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms
Diagnosis, Differential
Dyskeratosis Congenita / complications*,  pathology
Fibrosis / etiology,  pathology
Follow-Up Studies
Lung / pathology
Lung Diseases, Interstitial / etiology*,  pathology,  radiography
Middle Aged
Radiography, Thoracic
Tomography, X-Ray Computed

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

Previous Document:  Sepsis and myocardial depression in a young woman.
Next Document:  Estimation of the efficacy of screened natural hypolipidemic compounds in a model of rabbit hypercho...