Wolman's disease


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Wolman's disease

 [wol´manz]
primary familial xanthomatosis in infants; associated with involvement and calcification of the adrenal glands, failure to thrive, vomiting, diarrhea, hepatomegaly, splenomegaly, foam cells in the bone marrow and other tissues, and early death.
Miller-Keane Encyclopedia and Dictionary of Medicine, Nursing, and Allied Health, Seventh Edition. © 2003 by Saunders, an imprint of Elsevier, Inc. All rights reserved.

Wolman's disease

Cholesterol ester storage disease Genetics An AR storage disease seen in consanguineous cohorts, due to a defect in lysosomal sterol esterase with accumulation of TGs, and cholesterol esters Clinical Hepatosplenomegaly, bloating, wasting, adrenal calcification, xanthomatosis, steatorrhea Lab Anemia, ↑ TGs, cholesterol esters, foam cells in BM, vacuolated lymphocytes in circulation Prognosis Onset in early infancy, death by age 1.
McGraw-Hill Concise Dictionary of Modern Medicine. © 2002 by The McGraw-Hill Companies, Inc.