November 1973

Wolman Disease With Jaundice and Subarachnoid Hemorrhage

Author Affiliations

Madison, Wis
From the departments of pathology (Drs. Kamalian and Dudley) and pediatrics (Dr. Beroukhim), University of Wisconsin, Madison.

Am J Dis Child. 1973;126(5):671-675. doi:10.1001/archpedi.1973.02110190541017

Wolman disease occurred in a 2½-month-old Iranian girl. Besides the characteristic features of failure to thrive, vomiting, diarrhea, fever, anemia, lymphadenopathy, progressive hepatosplenomegaly, and symmetrical enlargement and calcification of the adrenals, this patient had severe liver involvement, jaundice, and leptomeningeal hemorrhage. Reticuloendothelial tissues and other viscera were distended by foamy histiocytes. Lysosomal acid lipase deficiency has been postulated as the cause of intracellular accumulation of triglycerides and cholesterol with concomitant low serum levels of lipoprotein and cholesterol. An autosomal recessive inheritance factor favors genetic counseling. A plea is made for early diagnosis and low-fat diet therapy.