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Published on: March 23, 2018
Infantile-onset diabetes mellitus: a 1-year follow-up study
Ramaswamy Ganesh1, Ravindrakumar Arvindkumar, Thiruvengadam Vasanthi
1Department of Pediatrics, Kanchi Kamakoti CHILDS Trust Hospital, Chennai, India. ganeped79@rediffmail.com
Insights
Infantile-onset diabetes mellitus (IODM) in infants under one year is increasingly diagnosed, often linked to genetic syndromes like Wolcott-Rallison syndrome. Early diagnosis and management are crucial for outcomes.
Area of Science:
- Pediatric Endocrinology
- Genetics
- Metabolic Disorders
Background:
- Infantile-onset diabetes mellitus (IODM) is a rare condition requiring specialized evaluation.
- Understanding the diverse etiologies and clinical presentations of IODM is essential for appropriate management.
- Previous studies highlight the challenges in diagnosing and managing diabetes in the first year of life.
Purpose of the Study:
- To evaluate the clinical profiles and outcomes of infants diagnosed with diabetes mellitus before one year of age.
- To identify the underlying causes and associated syndromes in infantile-onset diabetes mellitus.
- To analyze the incidence trends and genetic associations of IODM.
Main Methods:
- Retrospective analysis of 12 infants with IODM presenting between January 2003 and December 2007.
- Comprehensive evaluation including detailed history, clinical examination, and laboratory investigations.
- Human leukocyte antigen (HLA) typing was performed for genetic analysis.
Main Results:
- The median age of onset was 2.5 months, with a higher prevalence in girls (9/12).
- Six infants had features of Wolcott-Rallison syndrome (WRS), four had type 1 diabetes, and one each had Fanconi-Bickel syndrome and maturity-onset diabetes of young.
- Predominant HLA alleles were DQ3 and DR15. Two children with WRS died; others are under follow-up.
Conclusions:
- The incidence of IODM appears to be increasing, with diverse syndromic associations.
- IODM is often associated with specific genetic syndromes, necessitating thorough etiological investigation.
- Early identification of underlying syndromes is critical for prognosis and management of infantile diabetes.
Abstract:
This study evaluates the clinical profiles and outcomes of children with infantile-onset diabetes mellitus (IODM) (onset at <1 year). Twelve infants with IODM presenting to our hospital from January 2003 to December 2007 are analyzed. All undergo thorough history, clinical examination, and investigations and are managed as per hospital-approved protocol and periodically followed up. Of 12 infants (3 boys and 9 girls), 9 have a family history of DM. The median age at onset is 2.5 months. Six infants have features suggestive of Wolcott-Rallison syndrome (WRS), 4 infants have type 1 DM, and 1 infant each has Fanconi-Bickel syndrome and maturity-onset diabetes of young. None have pancreatic agenesis or calculi. Human leukocyte antigen (HLA) typing shows DQ3 and DR15 alleles predominating. Two children with WRS died; the rest are being followed up. The incidence of IODM is increasing, with multiple syndromic associations rather than a single perspective.
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