Wolman's disease. A rare lipidosis with adrenal calcification.

Abstract

In 1956 Abramov, Schorr, and Wolman from Israel described a 2-month-old female infant of Persian-Jewish extraction, whose parents were first cousins, who died from a lipidosis in which triglycerides and cholesterol were deposited in various tissues, and in whom the adrenals were extensively involved and diffusely calcified. In 1961 they reported an identical disease in two other sisters of the same family. Crocker et al. (1965) reported the first 3 cases of this disorder to be recognized in America. They were not related and were of widely different racial origin. The male patient in their report had a brother who died of an apparently similar disease. In 1966 the first case in Japan was described by Konno et al. in a 2- month-old female infant who had 3 sibs, 2 of whom had succumbed after a clinically similar illness.