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April 1979

Cystinosis and a Dissecting Aortic Aneurysm in a 7-Year-Old Boy

Author Affiliations

Department of Surgical Pathology Barnes Hospital and Washington University School of Medicine 600 S Euclid Ave St Louis, MO 63110

Am J Dis Child. 1979;133(4):436-438. doi:10.1001/archpedi.1979.02130040090020

Dissecting aortic aneurysms are usually afflictions of middle-aged and elderly people with atherosclerosis and hypertension.1 The association with hypertension is not invariable even in the older age group, but in younger patients hypertension is even less prominent. In this age group, the association is more with aortic coarctation and valvular abnormalities.2

There is, however, a group of metabolic abnormalities that predisposes to aortic dissections. Patients with Marfan's syndrome tend to have aneurysms of the ascending aorta and arch develop, and they may succumb any time from the early teens onward.3 Lysyl oxidase, an enzyme that cross-links collagen and elastin, is deficient in lathryism and in copper deficiency, and these conditions are associated with aortic dissections.

This report describes a boy with cystinosis whose condition progressed to renal failure and who died of an acute dissection involving the descending aorta.

Report of a Case.—A 7-year-old white boy

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