Document Detail


Mitral valve prolapse: a consistent manifestation of type IV Ehlers-Danlos syndrome. The pathogenetic role of the abnormal production of type III collagen.
MedLine Citation:
PMID:  7237708     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
To evaluate whether abnormal production of type III collagen, the characteristic biochemical feature of patients with the type IV Ehlers-Danlos syndrome, consistently predisposes to mitral valve prolapse, we evaluated the family of a proband with classic type IV Ehlers-Danlos syndrome. Production of type III collagen was assessed with the use of cultured skin fibroblasts. Mitral valve prolapse was detected by M-mode and two-dimensional echocardiography. Biochemical abnormalities in the production of type III collagen and echocardiographic findings of mitral valve prolapse were completely concordant. All patients with abnormal production of type III collagen had mitral valve prolapse and all subjects with normal production of type III collagen had entirely normal echocardiograms. Six of the eight patients with abnormal production of type III collagen had subtle cutaneous abnormalities. The consistent association of abnormal production of type III collagen and mitral valve prolapse in this family suggests that this abnormality of collagen may give rise to mitral valve prolapse.
Authors:
A S Jaffe; E M Geltman; G E Rodey; J Uitto
Related Documents :
7882118 - Incorporation of screening echocardiography in the preparticipation exam.
3415298 - Cardiac anomalies in williams-beuren syndrome.
17338748 - Reinforced edge-to-edge commissural closure with mitral valvuloplasty.
16132998 - Malone antegrade colonic irrigation: ileal neoappendicostomy is the preferred procedure...
7569128 - Hepatic arterial anatomy: demonstration of normal supply and vascular variants with thr...
7552828 - Implications of unsuspected pulmonary embolism detected by computed tomography.
Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  Circulation     Volume:  64     ISSN:  0009-7322     ISO Abbreviation:  Circulation     Publication Date:  1981 Jul 
Date Detail:
Created Date:  1981-08-10     Completed Date:  1981-08-10     Revised Date:  2007-11-14    
Medline Journal Info:
Nlm Unique ID:  0147763     Medline TA:  Circulation     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  121-5     Citation Subset:  AIM; IM    
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms
Descriptor/Qualifier:
Adolescent
Adult
Child
Collagen / biosynthesis*
Echocardiography
Ehlers-Danlos Syndrome / complications*,  diagnosis,  etiology
Female
HLA Antigens
Haploidy
Humans
Male
Middle Aged
Mitral Valve Prolapse / complications*,  diagnosis,  etiology
Physical Examination
Skin Diseases / diagnosis
Grant Support
ID/Acronym/Agency:
5-K04-AM-00455/AM/NIADDK NIH HHS; GM 28833/GM/NIGMS NIH HHS
Chemical
Reg. No./Substance:
0/HLA Antigens; 9007-34-5/Collagen

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


Previous Document:  Size and motion of the mitral valve annulus in man. I. A two-dimensional echocardiographic method an...
Next Document:  Effect of sulfinpyrazone on ventricular fibrillation during acute myocardial ischemia.