Elastin and collagen in the aortic wall: changes in the Marfan syndrome and annuloaortic ectasia.
Elastin and collagen concentrations were determined in intimal-medial samples of ascending aortas from healthy controls of different ages and from 20 patients with annuloaortic ectasia (AAE). Five patients had the Marfan syndrome. In controls the highest elastin concentrations (estimated from desmosine concentrations or insoluble residues after hot-alkali extraction) were found in children. During aging until 60 years, elastin concentration decreased when determined by the hot-alkali extraction method while desmosine concentration changed less. Aorta samples from the Marfan-syndrome patients showed a great variation of elastin concentration from total lack to normal values. Samples from the other AAE patients could be divided into two groups. One contained clearly less elastin and more collagen than the controls whereas in the other group this difference was less marked. Histological examination of the aortic wall of the first group also showed marked fibrosis accompanied by severe elastin fragmentation and acellularity. From the 15 non-Marfan patients 14 were men. By means of clinical examination these patients could also be divided into "familial" and "nonfamilial" groups, because increased diameter of the aortic root was found in relatives of almost half of the patients. However, there were no differences in elastin and collagen concentrations between the familial and nonfamilial cases. As well, no correlation was found between biochemical findings and diameters of the aortic roots. These results point to altered elastin and/or collagen metabolism in the aortic wall of AAE patients.
D000293:Adolescent; D000328:Adult; D000368:Aged; D000596:Amino Acids / Q000378:metabolism; D001011:Aorta / Q000378:metabolism; D001018:Aortic Diseases / Q000378:metabolism*; D002648:Child; D003094:Collagen / Q000378:metabolism*; D003895:Desmosine / Q000378:metabolism; D004549:Elastin / Q000378:metabolism*; D005260:Female; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000378:metabolism*; D008875:Middle Aged
D000596:Amino Acids; D003895:Desmosine; D003094:Collagen; D004549:Elastin