Document Detail


Wolfram Syndrome: A Rare Optic Neuropathy in Youth with Type 1 Diabetes.
MedLine Citation:
PMID:  21892113     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
Wolfram Syndrome (WS) is a rare, autosomal recessive disorder that causes non-autoimmune type 1 diabetes. The etiology involves a single gene mutation of the wolframin protein inducing endoplasmic reticulum stress and apoptosis in selected cell types with resultant diabetes insipidus, diabetes mellitus, optic atrophy, and sensory-neural deafness. Symptoms are initially absent and signs within the posterior segment of the eye are usually the earliest indicator of WS.These cases characterize unusual and poorly described findings of pigmentary maculopathy in WS and illustrate the importance of collaboration between diabetes and eye care providers; especially in cases of non-autoimmune type 1 diabetes exhibiting atypical human leukocyte-associated antigen haplotypes.
Authors:
Brian C Bucca; Georgeanna Klingensmith; Jeffrey L Bennett
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2011-9-1
Journal Detail:
Title:  Optometry and vision science : official publication of the American Academy of Optometry     Volume:  -     ISSN:  1538-9235     ISO Abbreviation:  -     Publication Date:  2011 Sep 
Date Detail:
Created Date:  2011-9-5     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8904931     Medline TA:  Optom Vis Sci     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Affiliation:
*OD, FAAO †MD ‡MD, PhD University of Colorado, Denver, Anschutz Medical Campus, School of Medicine, The Barbara Davis Center for Childhood Diabetes (BCB, GK), Departments of Pediatrics and Ophthalmology (BCB), Department of Pediatrics (GK), and Departments of Neurology and Ophthalmology (JLB), University of Colorado, Denver, Aurora, Colorado.
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