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Wolfram syndrome

ORPHA:3463· ICD-10 E34.8

Definition

A rare, genetic, endocrine disorder characterized by type I diabetes mellitus (DM), diabetes insipidus (DI), sensorineural deafness (D), bilateral optical atrophy (OA) and neurological signs.

Prevalence
1-9 / 1 000 000
Inheritance
Autosomal recessive
Age of onset
Adolescent, Adult, Childhood