PEDIATRICS Vol. 42 No. 1 July 1968, pp. 70-76
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PRIMARY FAMILIAL XANTHOMATOSIS WITH ADRENAL INVOLVEMENT (WOLMAN'S DISEASE)

Report of a Further Case with Nervous System Involvement and Pathogenetic Considerations

David Kahana M.D.1, Moshe Berant M.D.1, and Moshe Wolman M.D.1

1 Department of Pediatrics, Hillel Jaffe Government Hospital, Hadera, and Department of Pathology, Tel-Aviv University Medical School, Government Hospital, Tel-Hashomer, Israel

An apparently sporadic case of primary familial xanthomatosis with adrenal involvement (Wolman's disease) in a Jewish Israeli infant who died at the age of 3 months is described. Clinically, malabsorption and ileus were prominent. Pathological findings included the usual lipid storage process in the intestines, liver, lungs, spleen, and adrenals; storage in interstitial cells in other organs (heart, aorta, etc.); and evidence of lipid storage in the central and peripheral nervous system.

The histochemical findings are believed to suggest that derangement of triglyceride metabolism might be the main feature of the disease.

Submitted on July 14, 1967
Accepted on January 24, 1968




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