INTRODUCTION: Recently, interest in “neonatal” diabetes has increased because patients could stop taking insulin and improve glycemic control and associated neurologic features.
OBJECTIVE: Our objective was to determine the anticipated increase in prevalence and incidence of permanent neonatal diabetes in children, adolescents, and adults and investigate the impact of the new definition.
METHODS: We studied 293 (53% male) referrals to the Exeter Laboratory (Devon, United Kingdom) as part of the largest international series to date. The referred patients were diagnosed with diabetes below 6 months of age irrespective of current age, and their conditions had not remitted at the time of study. Data on 27 countries were collected, and age of diagnosis, date of birth, and gender were obtained from standardized forms. All referred patients were tested for KCNJ11 mutations.
RESULTS: The minimum observed prevalence of the 5 most representative countries was 1.17 (1.01–1.31) per million population, with the estimated true prevalence twice as high. Prevalence was higher for the pediatric versus adult age range (odds ratio: 0.78 [95% confidence interval: 0.54–1.31] vs 0.42 [95% confidence interval: 0–0.50], respectively; P = .009). Seventy-five percent of the patients were below 16 years of age with a median (interquartile range) of 5.7 (2.4–10.2) years, which implies underdiagnosis beyond 5 years of age. Age of diagnosis was skewed to a median (interquartile range) of 6 (1–13) weeks, with 62% in the first 8 weeks. During 2000–2004, the minimum observed incidence was 2.95 (0–49.1) per million live births.
CONCLUSIONS: This is the first report to show 2 to 25 times higher prevalence than previous reports from 10 years ago. “Neonatal” should be changed to “diagnosed at <6 months of age irrespective of current age,” and awareness should be increased, especially for those who are older than 5 years and present with treatment implications.
Submitted by Annabelle S. Slingerland
- Copyright © 2008 by the American Academy of Pediatrics