| Respiratory complications of Ehlers-Danlos syndrome type IV. | |
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MedLine Citation:
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PMID: 9147885 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Pulmonary complications are described in a case of Ehlers-Danlos syndrome type IV, established by studies of collagen biosynthesis. At age 20.5 years the patient, who had previously suffered a spontaneous colonic perforation, developed intermittent recurrent hemoptysis and had a spontaneous hemopneumothorax. At presentation, imaging studies revealed multiple scattered cavitary lesions in both lungs. On separate occasions large parenchymal cysts ensued and subsequently regressed. Reviews of other reported patients indicate that pulmonary complications do occur in patients with Ehlers-Danlos syndrome type IV but have not resulted directly in patient mortality. |
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Authors:
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S B Dowton; S Pincott; L Demmer |
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Publication Detail:
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Type: Case Reports; Journal Article; Review |
Journal Detail:
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Title: Clinical genetics Volume: 50 ISSN: 0009-9163 ISO Abbreviation: Clin. Genet. Publication Date: 1996 Dec |
Date Detail:
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Created Date: 1997-05-09 Completed Date: 1997-05-09 Revised Date: 2005-11-16 |
Medline Journal Info:
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Nlm Unique ID: 0253664 Medline TA: Clin Genet Country: DENMARK |
Other Details:
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Languages: eng Pagination: 510-4 Citation Subset: IM |
Affiliation:
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Edward Mallinckrodt Department of Pediatrics, Washington University School of Medicine, St. Louis, MO 63110, USA. dowton@kids.wustl.edu |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adolescent Collagen / genetics Colonic Diseases / etiology Cysts / etiology*, radiography Ehlers-Danlos Syndrome / classification, complications*, genetics Hemothorax / etiology Humans Intestinal Perforation / etiology Lung Diseases / etiology*, radiography Male Pneumothorax / etiology |
| Chemical | |
Reg. No./Substance:
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9007-34-5/Collagen |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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