Marfan's Syndrome

Autor: Bear, Edward S., Tung, Millie Y.
Zdroj: JAMA: Journal of the American Medical Association; October 1971, Vol. 218 Issue: 4 p597-597, 1p
Abstrakt: To the Editor.—Lee's points are well taken and possibly we should have stated that the diagnosis of Marfan's syndrome was suggested rather than warranted by our findings. Until a more precise definition of a basic defect (as in homocystinuria) is demonstrated in Marfan's syndrome, there will continue to be confusion surrounding incomplete or forme fruste cases. A recent example is a case of Marfan's syndrome with chronic atrial flutter1 which failed to exhibit ocular disease, positive family history, or what Lee would call "typical aortic disease."The abnormality in the small coronary arteries of patients with Marfan's syndrome as described by James et al2 must make one highly suspicious of Marfan's syndrome in a patient with arachnodactaly and a cardiac conduction defect. Sinclair has mentioned cataracts in Marfan's syndrome, but is not clear if they were in association with ectopia lentis or not.3 Our patient was believed to
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