Ramaswamy Ganesh1, Natarajan Suresh, Thiruvengadam Vasanthi, K G Ravikumar. 1. Departments of Pediatrics and *Endocrinology, Kanchi Kamakoti CHILDS Trust Hospital and The CHILDS Trust Medical Research Foundation, Chennai, Tamil Nadu, India. Correspondence to: Dr Ramaswamy Ganesh, Consultant Pediatrician, Kanchi Kamakoti CHILDS Trust Hospital, Chennai 600 034, India. ganeped79@rediffmail.com.
Abstract
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.
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.