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Infantile onset diabetes mellitus in developing countries - India
1Poovazhagi Varadarajan, Department of Pediatrics, Chengalpattu Medical College and Hospital, Chengalpattu, Tamil Nadu 603001, India.
Insights
Infantile onset diabetes mellitus (IODM) is a rare childhood metabolic disorder. Genetic diagnosis and targeted treatments, like oral medications, significantly improve outcomes for infants with monogenic diabetes.
Area of Science:
- Pediatrics
- Endocrinology
- Genetics
Background:
- Infantile onset diabetes mellitus (IODM) is a rare metabolic disorder in children under one year.
- It presents heterogeneously, often with diabetic ketoacidosis, and is frequently misdiagnosed.
- Monogenic causes, such as potassium channel mutations and Wolcott Rallison syndrome, are implicated.
Purpose of the Study:
- To review the literature on infantile onset diabetes mellitus (IODM) in India.
- To highlight diagnostic challenges and recent advancements in management.
Main Methods:
- Review of existing literature on infantile onset diabetes mellitus (IODM).
- Analysis of clinical presentation, genetic causes, and management strategies.
Main Results:
- 83% of IODM cases present with diabetic ketoacidosis; 67% are initially misdiagnosed.
- Potassium channel mutations (sulfonylurea-responsive) and Wolcott Rallison syndrome are common.
- Associated conditions include developmental delay and seizures.
Conclusions:
- Genetic diagnosis revolutionizes IODM management, enabling a switch from insulin to oral hypoglycemic agents in specific cases.
- Mortality rates in Indian neonatal diabetes studies reach 32.5%.
Abstract:
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.
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