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MARFAN'S SYNDROME: THE NATURE OF THE AORTIC DEFECT
Author(s) -
HURLEY J. V.
Publication year - 1959
Publication title -
australasian annals of medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.596
H-Index - 70
eISSN - 1445-5994
pISSN - 0571-9283
DOI - 10.1111/imj.1959.8.1.45
Subject(s) - marfan syndrome , abnormality , aorta , medicine , aneurysm , connective tissue , glycosaminoglycan , aortic aneurysm , cardiology , pathology , anatomy , surgery , psychiatry
SUMMARY Defects of the aortic wall involving one or more elements were present in five of seven cases of dissecting aneurysm of the aorta occurring in association with Marfan's syndrome ; in two of them no histological abnormality was seen. No significant difference was found in the hexosamine content of the walls (determined in five cases) as compared with that of normal aortas from subjects of the same age group. Although gross malformation of the aortic wall was present in a minority of cases, both in this series and in previously recorded cases, the basic defect common to all examples of Marfan's syndrome appears to involve the mucopolysaccharides of the intercellular substance. The chemical findings suggest that the defect may be in the degree of polymerization of these polysaccharides, rather than in alteration of the total amount present.