Document Detail


Usual interstitial pneumonia complicating dyskeratosis congenita.
MedLine Citation:
PMID:  15945534     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
James P Utz; Jay H Ryu; Jeffrey L Myers; Virginia V Michels
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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    
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MeSH Terms
Descriptor/Qualifier:
Adult
Biopsy
Diagnosis, Differential
Dyskeratosis Congenita / complications*,  pathology
Fibrosis / etiology,  pathology
Follow-Up Studies
Humans
Lung / pathology
Lung Diseases, Interstitial / etiology*,  pathology,  radiography
Male
Middle Aged
Radiography, Thoracic
Tomography, X-Ray Computed

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