Permanent diabetes mellitus in the first year of life.

AIMS/HYPOTHESIS: The pathogenesis of permanent diabetes mellitus diagnosed early in life is heterogeneous and, in most cases, not known. We aimed at identifying markers differentiating between non-autoimmune and autoimmune diabetes. METHODS: The clinical, genetic and epidemiological features of 111 diabetic patients (62 males) who received insulin within 12 months of life were studied. RESULTS: The epidemic curve by age of diabetes onset revealed two subsets of patients at a cutoff of 180 days. In the group with diabetes onset before 180 days ("early onset" permanent diabetes) the analysis of HLA susceptibility heterodimers (available for 21 individuals) showed that 76% had a "protective" HLA genotype for Type I (insulin-dependent) diabetes mellitus as compared to 11.9% (5/42) of the later onset group. Accordingly, "early onset" children were less likely to have autoimmunity markers (4 out of 26 tested) than children with onset after 180 days (13 out 20 tested) (15.4% vs. 65.0%, p<0.01). Of note, 19 out of 20 (or the 95%) patients who were born on the island of Sardinia, an Italian region where the incidence of Type I diabetes is six times higher than continental Italy (33/100,000/year vs 5/100,000/year), were included in the later onset group (>180 days). Small-for-date birthweight, a possible sign of reduced foetal insulin secretion, was more common in the "early onset" group (OR=9.9, 95%-CI 2.6-38.6). CONCLUSION/INTERPRETATION: These results, obtained in the largest population-based cohort of diabetic infants hitherto reported, suggest that "early onset" permanent diabetes cases differ from later onset cases and that most of them do not have an autoimmune pathogenesis.

Incidence of childhood type 1 diabetes mellitus in Gomel, Belarus.

BACKGROUND: The importance of genetic and environmental factors in causing type 1 diabetes mellitus (DM1) in many European countries is unknown. We studied this question by assessing the incidence of the disease in children in Gomel, Belarus, in the years subsequent to the Chernobyl disaster, comparing it to the antecedent period. METHODS: We identified all new patients with DM1 under 15 years at onset who presented between 1976 and 1999; we used as denominator the number of children aged 0-14 born in Gomel, to calculate the incidence. All data were validated by using the capture-recapture method. We compared the incidence rate between the periods before and after 1986; we also calculated the average increment of incidence in the latter period (1987-1999). RESULTS: In the whole period, 438 new patients presented, with an average crude rate of incidence of 4.6/100,000 but with a net difference between the former (before 1986) and latter (after 1986) periods. The average standard incidence in the latter period was 5.7 (C.I. 4.5-7.0), with a significant increase in the 10-14 year age group. The average yearly increase of incidence in the same period was 8.9%, highly significant (p < 0.001). CONCLUSIONS: The results seem to confirm the hypothesis of the influence of environmental pollution subsequent to the Chernobyl accident.

[Hypohidrotic ectodermal dysplasia]. / Displasia ectodermica ipoidrotica.

The authors report a case of Hypohidrotic ectodermal dysplasia (Christ-Siemens-Touraine syndrome). Diagnosed at the age of 2 months.