Wolman's disease: A rare lipidosis with adrenal calcification

Abstract

Two cases of Wolman's disease occurring in Great Britain are described. This familial lipidosis is characterized by the storage of esterified cholesterol and triglyceride in liver, spleen, reticuloendothelial system, intestine, and other organs; in particular, the adrenals are extensively and symmetrically involved and often radiologically calcified. Diarrhoea, wasting, and hepato-splenomegaly in early infancy, sometimes accompanied by radiological adrenal calcification, should suggest the diagnosis, which may be confirmed by bone-marrow and liver biopsy.