NCJ Number
209111
Journal
Journal of Forensic Sciences Volume: 50 Issue: 2 Dated: March 2005 Pages: 461-464
Date Published
March 2005
Length
4 pages
Annotation
This paper documents factors in the death of a 30-year-old man with Ehlers-Danlos Syndrome Type IV from myocardial ruptures and cardiac tamponade following a myocardial infarction.
Abstract
Ehlers-Danlos Syndrome Type IV is a rare familial disease caused by a mutation in the gene for type III procollagen. This results in either a lack of production of type III procollagen or production of a defective type III procollagen. Production of an abnormal procollagen leads to abnormal formation of collagen fibrils and a decrease in the tensile strength of the tissue. The underproduction of type III procollagen also leads to a decrease in the tensile strength of the tissue. In the case at issue, the deceased, who was known to have Ehlers-Danlos Syndrome Type IV, was found dead in his car with no suspicious circumstances. The direct factors in the man's death were myocardial rupture and cardiac tamponade following a myocardial infarction, events which are rare in a person the age of the deceased. In concluding that the man's Ehlers-Danlos Syndrome Type IV probably contributed to the factors in the man's death, the authors note the possibility that Ehlers-Danlos Syndrome Type IV may have contributed to the early progression of atherosclerotic changes in the decedent, since native fibrillar collagen has been shown to inhibit smooth muscle cell proliferation in vitro, a known step in the process of plaque formation. Also, it is possible that Ehlers-Danlos Syndrome Type IV would make an infarcted myocardium more prone to rupture. 4 figures and 14 references