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Wolman Disease With Jaundice and Subarachnoid Hemorrhage

Wolman Disease With Jaundice and Subarachnoid Hemorrhage Abstract 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. References 1. Abramov A, Schorr S, Wolman M: Generalized xanthomatosis with calcified adrenals . Am J Dis Child 91:282-286, 1956. 2. Wolman M, et al: Primary familial xanthomatosis with involvement and calcification of the adrenal . Pediatrics 28:742-757, 1961. 3. Crocker AC, et al: Wolman's disease: Three new patients with a recently described lipoidosis . Pediatrics 35:627-640, 1965. 4. Konno T, Fujii M: Wolman's disease: The first case in Japan . Tohoku J Exp Med 90:375-389, 1966.Crossref 5. Guazzi GC, et al: Wolman's disease . Eur Neurol 1:334-362, 1968.Crossref 6. Kahana D, Berant M, Wolman M: Primary familial xanthomatosis with adrenal involvement (Wolman's disease): Report of a further case with nervous system involvement and pathogenetic considerations . J Pediatr 42:270-276, 1968. 7. Marshall WC, et al: Wolman's disease: A rare lipoidosis with adrenal calcification . Arch Dis Child 44:331-341, 1969.Crossref 8. Lake BD, Patrick AD: Wolman's disease: Deficiency of E600-resistant acid esterase activity with storage of lipids in lysosomes . J Pediatr 76:262-266, 1970.Crossref 9. Lough J, Fawcet J, Wiegensberg B: Wolman's disease: An electron microscopic, histochemical and biochemical study . Arch Pathol 89:103-110, 1970. 10. Young EP, Patrick AD: Deficiency of acid esterase activity in Wolman's disease . Arch Dis Child 45:664-668, 1970.Crossref 11. Eto Y, Kitagawa T: Wolman's disease with hypolipoproteinemia and acanthocytosis: Clinical and biochemical observations . J Pediatr 77:862-867, 1970.Crossref 12. Barson AJ: Wolman's disease . Arch Dis Child 45:710, 1970.Crossref 13. Raafat F, Hashemian MP, Abrishami MA: Wolman's disease: Report of two new cases with a review of the literature . Am J Clin Pathol 59:490, 1973. 14. Philippart M: Wolman's disease . J Pediatr 79:173-174, 1971.Crossref 15. Patrick AD, Lake BD: Deficiency of an acid lipase in Wolman's disease . Nature 22:1067-1068, 1969.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png American Journal of Diseases of Children American Medical Association

Wolman Disease With Jaundice and Subarachnoid Hemorrhage

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Publisher
American Medical Association
Copyright
Copyright © 1973 American Medical Association. All Rights Reserved.
ISSN
0002-922X
DOI
10.1001/archpedi.1973.02110190541017
Publisher site
See Article on Publisher Site

Abstract

Abstract 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. References 1. Abramov A, Schorr S, Wolman M: Generalized xanthomatosis with calcified adrenals . Am J Dis Child 91:282-286, 1956. 2. Wolman M, et al: Primary familial xanthomatosis with involvement and calcification of the adrenal . Pediatrics 28:742-757, 1961. 3. Crocker AC, et al: Wolman's disease: Three new patients with a recently described lipoidosis . Pediatrics 35:627-640, 1965. 4. Konno T, Fujii M: Wolman's disease: The first case in Japan . Tohoku J Exp Med 90:375-389, 1966.Crossref 5. Guazzi GC, et al: Wolman's disease . Eur Neurol 1:334-362, 1968.Crossref 6. Kahana D, Berant M, Wolman M: Primary familial xanthomatosis with adrenal involvement (Wolman's disease): Report of a further case with nervous system involvement and pathogenetic considerations . J Pediatr 42:270-276, 1968. 7. Marshall WC, et al: Wolman's disease: A rare lipoidosis with adrenal calcification . Arch Dis Child 44:331-341, 1969.Crossref 8. Lake BD, Patrick AD: Wolman's disease: Deficiency of E600-resistant acid esterase activity with storage of lipids in lysosomes . J Pediatr 76:262-266, 1970.Crossref 9. Lough J, Fawcet J, Wiegensberg B: Wolman's disease: An electron microscopic, histochemical and biochemical study . Arch Pathol 89:103-110, 1970. 10. Young EP, Patrick AD: Deficiency of acid esterase activity in Wolman's disease . Arch Dis Child 45:664-668, 1970.Crossref 11. Eto Y, Kitagawa T: Wolman's disease with hypolipoproteinemia and acanthocytosis: Clinical and biochemical observations . J Pediatr 77:862-867, 1970.Crossref 12. Barson AJ: Wolman's disease . Arch Dis Child 45:710, 1970.Crossref 13. Raafat F, Hashemian MP, Abrishami MA: Wolman's disease: Report of two new cases with a review of the literature . Am J Clin Pathol 59:490, 1973. 14. Philippart M: Wolman's disease . J Pediatr 79:173-174, 1971.Crossref 15. Patrick AD, Lake BD: Deficiency of an acid lipase in Wolman's disease . Nature 22:1067-1068, 1969.Crossref

Journal

American Journal of Diseases of ChildrenAmerican Medical Association

Published: Nov 1, 1973

References