AUTOIMMUNE POLYENDOCRINOPATHY TYPE II IN FIRST DECADE - A CASE REPORT

Auto immune polyendocrine syndrome type II with its multisystem involvement and its varied presentation is usually diagnosed in third decade of life and is very uncommon in pediatric population. We highlight its early presentation in children and the need for further investigations and regular follow up in all children with suspected polyendocrinopathy. 9 year old child born to 3rd degree consanguineous parents diagnosed with diabetes at the age of 7 years was on follow up in our diabetic clinic. During follow up, the mother complained of increased pigmentation of face and upper limbs. Investigation revealed normal electrolytes and blood counts. ACTH stimulation test revealed hypo adrenalism. Further investigations revealed presence of Thyroid anti microsomal antibodies but no biochemical evidence of hypothyroidism. The child was diagnosed with Autoimmune Polyendocrine syndrome 2, started on steroids, insulin does was modified and the child is being currently followed up in our diabetic clinic.