Among the rarer forms of juvenile heart disease is that which arises in the course of familial xanthomatosis. The following record is presented in some detail not only because of its significance in the development in early life of atherosclerosis and myocardial infarction, but also because of its possible relevance to the pathogenesis of calcific aortic stenosis. REPORT OF A CASE The patient was of Greek parentage but had been born and had spent all of his life in New York City. He was first seen at the New York Hospital on Nov. 17, 1952, and died there at the age of 20 on May 4, 1953. Xanthomas were first noted by his parents when he was 4 years old. Three years later an apical systolic murmur was heard, and his serum cholesterol concentration was found to be 440 mg. per 100 cc. During his boyhood the xanthomas gradually became
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David P. Barr (1954) studied this question.
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