Background: Neonatal diabetes mellitusis a rare disorder with an incidence of 1 in 2,60,000 live births.
Methods: Retrospective analysis of clinical and genetic profile of children admitted with neonatal diabetes mellitus in a tertiary-care hospital in Chennai, India over 11 years.
Results: Ten children were diagnosed with neonatal diabetes of whom 9 had permanent neonatal diabetes mellitus. The age range at onset was from 3 days- 5 months. Of the 9 children, KCNJ11 gene mutation was positive in one, and ABCC 8 and INS gene mutation in two children each. Children with KCNJ11 and ABCC 8 gene mutations were switched over to oral sulfonyl urea therapy.
Conclusion: Few genotypes causing NDM can be managed effectively with oral sulfonyl ureas.