Managing neonatal diabetes in a resource-challenged setting: a case report on empirical switch to sulfonylurea.

Abstract

Neonatal diabetes mellitus (NDM) caused by activating mutations in the genes (KCNJ11 or ABCC8), which account for over 50% of cases, frequently responds to sulfonylurea. Sulfonylurea is costeffective, easy to administer and has better metabolic control. It improves neurocognitive development and has less risk of hypoglycaemia compared to insulin. Identifying patients with mutations in these two genes and hence those who might benefit from sulfonylurea requires genetic testing. In resource-poor settings with zero capacity for genetic testing, an empirical trial of sulfonylurea in all clinically eligible NDM becomes imperative. Case presentation: The child is a 7-month-old girl who has been symptomatic for 5- months, referred from a peripheral facility where she was being managed for newly onset type 1 diabetes mellitus with insulin therapy. She was stabilised on insulin; however, she kept having marked fluctuation in blood glucose readings. She was empirically switched to sulfonyl urea at 0.1mg/kg/day in 12-hourly divided doses. She showed marked response to sulfonylurea on the first day of therapy, and insulin was withdrawn, and she maintained perfect glucose readings with no documented hypoglycaemia. Conclusion: Neonatal diabetes mellitus remains a challenging case to manage due to its rarity and poor awareness among health care providers, particularly in a poor-resource setting. While insulin therapy remains the cornerstone of therapy at diagnosis, empirical trial of sulfonylurea in all eligible NDM where the capacity for genetic testing is lacking could be rewarding for the patient and managing physician.