Xanthomatosis and Coronary Heart Disease

Abstract

The frequent coexistence of familial xanthomatosis and coronary heart disease has been well established.^1-7There have been, however, relatively few reports of the pathologic findings in such patients,^1,2,5,6,8,9and in only two reports^1,9were the histologic findings reported in any detail. To our knowledge, necropsy studies of affected siblings have not been previously reported. The current findings relate to a sister and brother who died in their teens and belonged to a family previously studied by Curtis and co-workers^10,11and Wilkinson and associates.¹²This family has been recently reexamined¹³; the sister died in the interval between the two studies; the brother, during the course of the present survey. Both parents, although hypercholesteremic, are living and healthy. Four of their other children had died suddenly, presumably of xanthomatous heart disease; of the surviving twelve children, seven have hypercholesteremia without xanthomatosis and are clinically healthy. <h3>Clinical History and Necropsy Findings of Two Patients</h3> Case 1.—The first patient, the sister, was