Increasing incidence of childhood-onset type 1 diabetes mellitus among Estonian children in 1999-2006. Time trend analysis 1983-2006.

BACKGROUND: The incidence of childhood-onset type 1 diabetes mellitus (T1DM) among Estonian children under 15 years of age was 10.1 per 100,000 per year in 1983-1990 and 12.2 per 100,000 per year in 1991-1998 with the highest incidence in age-group 10.0-14.9 years in both periods. From 1983 to 1998, the incidence increased most rapidly in age-group 0-4.9 years. OBJECTIVE: To determine the incidence of T1DM among Estonian children in 1999-2006 and to compare the results with the data from 1983 to 1998. SUBJECTS AND METHODS: In 1999-2006, population-based incidence data were collected from two centers where all children with T1DM are seen after the diagnosis. Data for earlier periods were obtained from previously published data. Subjects were divided into three age-groups: 0-4.9 years, 5.0-9.9 years and 10.0-14.9 years. RESULTS: Between 1999 and 2006, 310 new cases of T1DM were diagnosed in Estonian children aged 0-14.9 years. The age-standardized incidence rate for that period was 17.2 [95% confidence interval (CI) 13.1-21.2]. The incidence was the highest, 21.2 (95% CI 17.7-25.3) in age-group 5.0-9.9 years. Over the time period 1983-2006, the incidence of childhood-onset T1DM in Estonian children under 15 years of age increased annually by an average 3.3% with the most rapid annual increase-9.3%-occurring in the youngest age-group. CONCLUSIONS: The incidence of childhood-onset T1DM in Estonia continues to rise and the age of onset of the disease becomes younger.

Symptomless celiac disease in type 1 diabetes: 12-year experience in Estonia.

BACKGROUND: We aimed to determine the prevalence and characteristics of celiac disease in children with type 1 diabetes in Estonia, a country with a formerly low frequency of both diseases. METHODS: Altogether, 271 patients with diabetes were studied over 12 years (1995-2006): 122 at diagnosis and 149 patients 0.1-14.8 years after diagnosis. In addition, 73 patients were followed up over 1-6 years. Immunoglobulin A type endomysium and tissue transglutaminase antibodies were determined. Patients with antibodies and/or with celiac-disease-related symptoms were invited for a small-intestinal biopsy. RESULTS: At the primary screening, celiac disease was histologically confirmed in nine patients (all without symptoms), that is, in 3.3% (95% confidence interval: 1.63-6.42) of type 1 diabetes cases. At follow up, celiac disease was additionally detected in two (2.7%) of 73 diabetic patients, that is, in 0.016 (95% confidence interval: 0-0.072) celiac disease cases per follow-up year. CONCLUSION: The prevalence of celiac disease among type 1 diabetes patients in Estonia is similar to that in countries with a high incidence of celiac disease and type 1 diabetes. As celiac disease is mostly symptomless, all children with type 1 diabetes, irrespective of their geographic origin, should be regularly screened for celiac disease.

Girl with partial Turner syndrome and absence epilepsy.

This report describes a 16-year-old girl with short stature (-5 standard deviations), normal puberty, panic attacks, absence epilepsy, some stigmata of Turner syndrome, and a Madelung deformity. Routine chromosomal analysis revealed a female karyotype with one abnormal chromosome X, with the suspicion of additional material on the short arm. With fluorescent in situ hybridization and array-multiplex amplifiable probe hybridization methodology, a complex aberration was detected, with a deletion of the distal part of Xp22.33 (including the short-stature homeobox gene) and a duplication of Xp22.32-p22.12 proximal to the deleted segment. The deletion in our patient involves the Xp22.33 region. Two genes in this region may contribute to the patient's phenotype: short-stature homeobox, and visuospatial/perceptual abilities. The duplication in our patient involves the Xp22.12-p22.32 region, which, according to the Online Mendelian Inheritance in Man database, contains at least 93 genes, 49 of which are of unknown function. It is difficult to conjecture which gene overexpression in this region may have contributed to the phenotype of our patient. To our knowledge, this small, complex chromosome X aberration was not described previously.