The frequent coexistence of familial
xanthomatosis and coronary heart disease
has been well established.1-7 There have
been, however, relatively few reports of the
pathologic findings in such patients,1,2,5,6,8,9
and in only two reports1,9 were 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-workers10,11 and Wilkinson and associates.12
This 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.
Clinical History and Necropsy Findings of Two Patients
Case 1.—The first patient, the sister, was