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Wolfram Syndrome

Wolfram Syndrome Abstract Sir.—We read with great interest the report by Carson et al on simultaneous occurrence of diabetes mellitus, diabetes insipidus, and optic atrophy in a brother and sister (Am J Dis Child 131:1382-1385, 1977) and would like to add the following data that seemed to have escaped the attention of the authors. A review of 91 cases with this syndrome called Wolfram syndrome has been published by Cremers et al.1 These authors also report a postmortem on one patient that disclosed noticeable dilation of the renal pelvis, ureters, and bladder. The hypophysis, hypothalamus, thalamus, substantia nigra, and red nucleus were normal. The optic nerve, chiasma, and both optic tracts showed slight gliosis and a loss of myelin structure, but no signs of active myelination. From this clinic, we have reported in 1975,2 two brothers with juvenile diabetes mellitus and optic atrophy with hypothalamic disease as judged by clinical References 1. Cremers CWRJ, Wijdeveld PGAB, Pinckers AJLG: Juvenile diabetes mellitus, optic atrophy, hearing loss, diabetes insipidus, atonia of the urinary tract and bladder and other abnormalities (Wolfram syndrome) . Acta Paediatr Scand (suppl 264) , 1977, pp 1-16. 2. Karp M, Laron Z, Lerman P, et al: The syndrome of juvenile diabetes mellitus and optic atrophy: Report on two siblings , in Laron Z (ed): Diabetes in Juveniles . Basel Switzerland, S. Karger AG, 1975, pp 242-247. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png American Journal of Diseases of Children American Medical Association

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Publisher
American Medical Association
Copyright
Copyright © 1978 American Medical Association. All Rights Reserved.
ISSN
0002-922X
DOI
10.1001/archpedi.1978.02120330090023
Publisher site
See Article on Publisher Site

Abstract

Abstract Sir.—We read with great interest the report by Carson et al on simultaneous occurrence of diabetes mellitus, diabetes insipidus, and optic atrophy in a brother and sister (Am J Dis Child 131:1382-1385, 1977) and would like to add the following data that seemed to have escaped the attention of the authors. A review of 91 cases with this syndrome called Wolfram syndrome has been published by Cremers et al.1 These authors also report a postmortem on one patient that disclosed noticeable dilation of the renal pelvis, ureters, and bladder. The hypophysis, hypothalamus, thalamus, substantia nigra, and red nucleus were normal. The optic nerve, chiasma, and both optic tracts showed slight gliosis and a loss of myelin structure, but no signs of active myelination. From this clinic, we have reported in 1975,2 two brothers with juvenile diabetes mellitus and optic atrophy with hypothalamic disease as judged by clinical References 1. Cremers CWRJ, Wijdeveld PGAB, Pinckers AJLG: Juvenile diabetes mellitus, optic atrophy, hearing loss, diabetes insipidus, atonia of the urinary tract and bladder and other abnormalities (Wolfram syndrome) . Acta Paediatr Scand (suppl 264) , 1977, pp 1-16. 2. Karp M, Laron Z, Lerman P, et al: The syndrome of juvenile diabetes mellitus and optic atrophy: Report on two siblings , in Laron Z (ed): Diabetes in Juveniles . Basel Switzerland, S. Karger AG, 1975, pp 242-247.

Journal

American Journal of Diseases of ChildrenAmerican Medical Association

Published: Aug 1, 1978

References