A Case report of Wolfram Syndrome
Wolfram Syndrome is a neurodegenerative autosomal recessive disorder. The occurrence of the disease should be suspected if non-autoimmune insulin-dependent diabetes occurs in an under-sixteen year old person having bilateral optic nerve atrophy. Diabetes insipidus (DI), neurosensory deafness, urinar...
| Main Authors: | , |
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| Format: | Article |
| Language: | fas |
| Published: |
Birjand University of Medical Sciences and Health Services
2013-05-01
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| Series: | مجله دانشگاه علوم پزشکی بیرجند |
| Subjects: | |
| Online Access: | http://journal.bums.ac.ir/browse.php?a_code=A-10-1083-1&slc_lang=en&sid=1 |
| Summary: | Wolfram Syndrome is a neurodegenerative autosomal recessive
disorder. The occurrence of the disease should be suspected if non-autoimmune
insulin-dependent diabetes occurs in an under-sixteen year old person having
bilateral optic nerve atrophy. Diabetes insipidus (DI), neurosensory deafness,
urinary track disorders, and nervous system complications are also seen in this
disorder. The current report presents a 13-year-old female patient with
diabetes mellitus type I (since age 4) along with recurrent urinary infection
and enuresis and progressive renal failure (since age 10). Bilateral optic
atrophy was evident on fondues examination. The patient also had diabetes
insipidus, neurosensory deafness, neurogenic bladder, short stature, combined
with delayed sexual maturity. |
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| ISSN: | 1607-2197 2423-6152 |