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Wolfram Syndrome
MOSHE KARP, MD;
ZVI LARON, MD
Institute of Pediatric and Adolescent Endocrinology
URI SANDBANK, MD
Institute of Neuropathology Beilinson Medical Center and Sackler School of Medicine Tel Aviv University Tel Aviv, Israel
Am J Dis Child. 1978;132(8):818-819.
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| Since this article does not have an abstract, we have provided the first 150 words of the full text PDF and any section headings. |
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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
. . . [Full Text PDF of this Article]
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