Published online Mar 25, 2016. doi: 10.4239/wjd.v7.i6.134
Peer-review started: October 20, 2015
First decision: November 11, 2015
Revised: December 14, 2015
Accepted: January 21, 2016
Article in press: January 22, 2016
Published online: March 25, 2016
Processing time: 157 Days and 19.6 Hours
Infantile onset diabetes mellitus (IODM) is an uncommon metabolic disorder in children. Infants with onset of diabetes mellitus (DM) at age less than one year are likely to have transient or permanent neonatal DM or rarely type 1 diabetes. Diabetes with onset below 6 mo is a heterogeneous disease caused by single gene mutations. Literature on IODM is scanty in India. Nearly 83% of IODM cases present with diabetic keto acidosis at the onset. Missed diagnosis was common in infants with diabetes (67%). Potassium channel mutation with sulphonylurea responsiveness is the common type in the non-syndromic IODM and Wolcott Rallison syndrome is the common type in syndromic diabetes. Developmental delay and seizures were the associated co-morbid states. Genetic diagnosis has made a phenomenal change in the management of IODM. Switching from subcutaneous insulin to oral hypoglycemic drugs is a major clinical breakthrough in the management of certain types of monogenic diabetes. Mortality in neonatal diabetes is 32.5% during follow-up from Indian studies. This article is a review of neonatal diabetes and available literature on IODM from India.
Core tip: The clinical presentation of infantile onset diabetes mellitus (IODM) as syndromic and non-syndromic forms from South India is discussed in this article. Associated co-morbid states, mortality pattern, difficulty in the management and need for genetic evaluation among this group of infants are also discussed. Identification of this form of monogenic diabetes by clinical evaluation and appropriate genetic evaluation to identify the subtypes helps in the management of these infants to improve the overall morbidity and mortality. Reported mortality in IODM is high from South India.