Document Detail


Respiratory complications of Ehlers-Danlos syndrome type IV.
MedLine Citation:
PMID:  9147885     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
S B Dowton; S Pincott; L Demmer
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Publication Detail:
Type:  Case Reports; Journal Article; Review    
Journal Detail:
Title:  Clinical genetics     Volume:  50     ISSN:  0009-9163     ISO Abbreviation:  Clin. Genet.     Publication Date:  1996 Dec 
Date Detail:
Created Date:  1997-05-09     Completed Date:  1997-05-09     Revised Date:  2005-11-16    
Medline Journal Info:
Nlm Unique ID:  0253664     Medline TA:  Clin Genet     Country:  DENMARK    
Other Details:
Languages:  eng     Pagination:  510-4     Citation Subset:  IM    
Affiliation:
Edward Mallinckrodt Department of Pediatrics, Washington University School of Medicine, St. Louis, MO 63110, USA. dowton@kids.wustl.edu
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MeSH Terms
Descriptor/Qualifier:
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:
9007-34-5/Collagen

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


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