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Year : 2016  |  Volume : 5  |  Issue : 1  |  Page : 181-183

An unusual case of adolescent type 2 diabetes mellitus: Prader-Willi syndrome

1 Department of Family Medicine, Lakeshore Hospital and Research Centre, Ernakulam, Kerala, India
2 Department of Endocrinology, Lakeshore Hospital and Research Centre, Ernakulam, Kerala, India

Correspondence Address:
Riyas Basheer
Department of Family Medicine, Lakeshore Hospital and Research Centre, NH 47 Bypass, Nettoor P.O., Maradu, Ernakulam - 682 040, Kerala
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2249-4863.184661

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Prader-Willi syndrome (PWS) is a complex genetic disorder, characterized by neonatal hypotonia, developmental delay, short stature, childhood obesity, hypogonadism, and characteristic facial features. Here we report a 21-year-old male who presented with uncontrolled glycemic status. He was diagnosed to have diabetes mellitus at the age of 15 with osmotic symptoms - polyuria, polydipsia, and polyphagia. In the early period, after diagnosis, his blood sugars were reasonably controlled with oral hypoglycemic agents. However, a year back, he was switched onto insulin therapy due to secondary OHA failure. On examination, his body mass index was 36 kg/m 2 . He had bilateral gynecomastia, decreased biparietal diameter, almond shaped eyes with esotropia. He had hypogonadism and also had mild cognitive impairment. He did not have any proximal myopathy or other focal neurological deficits. Hormonal evaluation showed low testosterone and inappropriately normal fluorescence in situ hybridization suggestive of central hypogonadism. With fetal and neonatal hypotonia, delayed developmental milestones, hypogonadism, and early onset diabetes, he fulfilled the clinical criteria for the diagnosis of PWS. Multidisciplinary approach of clinicians together with family and social support are essential to bring out the optimal outcome for such syndromic cases.

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