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Wolfram (DIDMOAD) syndrome: a complex long-term problem in management.

N R Peden, J D Gay, R T Jung

    The Quarterly Journal of Medicine
    |February 1, 1986
    PubMed
    Summary
    This summary is machine-generated.

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    Wolfram syndrome, a rare genetic disorder, affects multiple endocrine and neurological systems. This study details five patients with diabetes insipidus, diabetes mellitus, optic atrophy, and deafness, highlighting varied symptom onset and severity.

    Area of Science:

    • Genetics and Endocrinology
    • Neuro-ophthalmology
    • Otolaryngology

    Background:

    • Wolfram syndrome is a rare autosomal recessive disorder characterized by a combination of clinical features.
    • Key manifestations include early-onset diabetes mellitus, diabetes insipidus, optic atrophy, and sensorineural hearing loss.
    • Understanding the phenotypic variability and progression is crucial for patient management.