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Year : 2023  |  Volume : 3  |  Issue : 1  |  Page : 115-117

The tale of Wolfram syndrome in a young child versus an adolescent: Two case reports

1 DNB Resident, General Ophthalmology, Aravind Eye Hospital, Coimbatore, Tamil Nadu, India
2 Department of Pediatric Ophthalmology and Strabismus and Neuro-Ophthalmology, Dr. Shroff's Charity Eye Hospital, New Delhi, India
3 Department of Neuro-Ophthalmology, Aravind Eye Hospital, Coimbatore, Tamil Nadu, India

Correspondence Address:
Hirika Gosalia
Aravind Eye Hospital, Avinashi Road, Coimbatore - 641 014, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_1001_22

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Wolfram Syndrome (WS) is a rare autosomal recessive progressive neurodegenerative disorder characterized by juvenile-onset diabetes mellitus (DM) and optic atrophy (OA), known as DIDMOAD (diabetes insipidus [DI], diabetes mellitus, optic atrophy, deafness). Patients present with DM and OA in the first decade, DI and sensorineural deafness in the second decade, urinary tract anomalies, and neurological manifestations later in life. We report two cases with late diagnosed WS in an asymptomatic young child and an adolescent with DI, DM, OA, and deafness to raise awareness regarding the identification of individuals with WS and provide them with immediate treatment and appropriate care.

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