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Wolfram-like syndrome

Endocrine, Nutritional and Metabolic Diseases

Wolfram-like syndrome is a rare autosomal dominant genetic disorder that shares some features with the autosomal recessive Wolfram syndrome, which is a separate condition, and is a type of WFS1-related disorder. People with it usually have progressive hearing loss beginning around age ten, optic atrophy appearing from the mid teens into adulthood, and adult-onset diabetes mellitus; psychiatric symptoms such as depression and anxiety can also occur. It is caused by mutations in the WFS1 gene, and in rare cases the CDK13 gene. There is no standard management method. Only about 15 to 20 cases in four European families, from Denmark, the Netherlands, France and Sweden, had been described in the literature, and the disorder was seemingly first described in 1940 in a Swedish family.

Facts
Classification
Body System
Ear 1
Communicable or Non-communicable
Non-Communicable 1
Causative Agent
Causative Agent (category)
Genetic / Hereditary 1
First Described
1940 1
Sources
1. Wolfram-like syndrome (Wikipedia)
  • History
    The disorder was seemingly first described in 1940 by Samuelson et al.
  • Genetics
    The condition is caused by mutations in the WFS1 gene
  • Signs and symptoms
    early-onset progressive hearing loss which starts around the age of 10 years old
  • Lead
    Wolfram-like syndrome is a rare autosomal dominant genetic disorder
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