Wolman’s Disease: Report of Two New Cases, with a Review of the Literature

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Abstract
Two additional cases of the acute infantile form of Wolman’s disease, occurring in Iranian Moslems, are described. Diagnosis in both cases was made on the basis of clinical findings of hepatosplenomegaly, malabsorption, and adrenal calcification demonstrated by roentgenographic examination. Histochemical studies confirmed the presence of cholesterol and triglycerides in “foam cells.” Extensive involvement of the small intestine, adrenal glands, and reticuloendothelial system, but not of the central nervous system, was found at autopsy. The literature is reviewed, with emphasis on the clinical and pathologic involvement found, recently described milder variants of the disease, and biochemical and genetic aspects.

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