Document Detail

Vascular abnormalities in congenital cutis laxa--report of two cases.
MedLine Citation:
PMID:  2116064     Owner:  NLM     Status:  MEDLINE    
Two cases of Japanese girls with congenital cutis laxa associated with cardiovascular abnormalities are described. Case 1: A 12-year-old girl has been under our observation from the age of 6 months. Cardioangiogram revealed dilatation of the ascending aorta, meandering of the descending aorta and the coronary arteries, coiling of the carotid and innominate (brachiocephalic) arteries, and hypoplasia of the pulmonary arteries. Case 2: A 2.8/12-year-old girl died after our follow-up from the age of 3 months. The cause of death was congestive heart failure secondary to peripheral stenosis of the pulmonary arteries. In both cases, skin biopsy revealed a decreased number of elastic fibers and an increased amount of acidic mucopolysaccharides. The same histological features were observed in the pulmonary arteries and other arteries as well. Electron microscopic findings were diffuse thinning of elastic fibers and reduced elastic content. The high blood level of elastase (167.8 micrograms/l) in case 1 may cast a light on the unknown etiology of the disease.
A Tsuji; J Yanai; T Miura; Y Shirai; M Osano; Y Hosoda; M Sato; T Asaishi; Y Oda; H Hajikano
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Acta paediatrica Japonica; Overseas edition     Volume:  32     ISSN:  0374-5600     ISO Abbreviation:  Acta Paediatr Jpn     Publication Date:  1990 Apr 
Date Detail:
Created Date:  1990-09-04     Completed Date:  1990-09-04     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  0370357     Medline TA:  Acta Paediatr Jpn     Country:  JAPAN    
Other Details:
Languages:  eng     Pagination:  155-61     Citation Subset:  IM    
Department of Pediatrics, Yamanashi Medical College, Tokyo.
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MeSH Terms
Abnormalities, Multiple*
Aorta / abnormalities*
Coronary Vessel Anomalies / complications*
Cutis Laxa / complications*
Follow-Up Studies
Pulmonary Artery / abnormalities*

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