| Usual interstitial pneumonia complicating dyskeratosis congenita. | |
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MedLine Citation:
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PMID: 15945534 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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. |
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Authors:
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James P Utz; Jay H Ryu; Jeffrey L Myers; Virginia V Michels |
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Publication Detail:
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Type: Case Reports; Journal Article |
Journal Detail:
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Title: Mayo Clinic proceedings. Mayo Clinic Volume: 80 ISSN: 0025-6196 ISO Abbreviation: Mayo Clin. Proc. Publication Date: 2005 Jun |
Date Detail:
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Created Date: 2005-06-10 Completed Date: 2005-06-21 Revised Date: 2007-10-29 |
Medline Journal Info:
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Nlm Unique ID: 0405543 Medline TA: Mayo Clin Proc Country: United States |
Other Details:
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Languages: eng Pagination: 817-21 Citation Subset: AIM; IM |
Affiliation:
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Department of Internal Medicine, Mayo Clinic College of Medicine, Rochester, MN 55905, USA. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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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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