Histochemical studies in Wolman's disease--report of an autopsy case accompanied with a large amount of milky ascites.

This is a case of a male infant who suffered from “Primary familial xanthomatosis with involvement and calcification of adrenals” (Wolman's disease). The clinical symptoms were characteristic hepatosplenomega-ly, abdominal distention and a large amount of milky ascites. The patient died at the age of 2 1/2 months. X-ray revealed enlarged, well-shaped, calcified adrenal glands. Foamy cells in different organs which were observed at autopsy confirmed the diagnosis. On histochemical examination, deposition of a triglyceride-cholesterol mixture was found in mesenchymal and epithelial cells of the liver, adrenals, spleen, lymph nodes and mucosa of the small intestine.