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BACKGROUND/OBJECTIVE The aim of this study was to systematically assess the association of insulin-manipulation (intentional under- and/or overdosing of insulin), psychiatric comorbidity and diabetes complications. METHODS Two diagnostic interviews (Diabetes-Self-Management-Patient-Interview and Children's-Diagnostic-Interview for Psychiatric Disorders) were conducted with 241 patients (age 10-22) with type 1 diabetes (T1D) from 21 randomly selected Austrian diabetes care centers. Medical data was derived from medical records. RESULTS Psychiatric comorbidity was found in nearly half of the patients with insulin-manipulation (46.3%) compared to a rate of 17.5% in patients, adherent to the prescribed insulin therapy. Depression (18.3% vs 4.9%), specific phobia (21.1% vs 2.9%), social phobia (7.0% vs 0%), and eating disorders (12.7% vs 1.9%) were elevated in patients with insulin-manipulation. Females (37.7%) were more often diagnosed (P = 0.001) with psychiatric disorders than males (18.4%). In females, the percentage of psychiatric comorbidity significantly increased with the level of non-adherence to insulin therapy. Insulin-manipulation had an effect of +0.89% in HbA1c (P =

Background/Aims: Intrauterine exposure to hyperglycemia might impact the risk for future metabolic deteriorations. The aim was to characterize the association between different adipokines and neuropeptides and insulin resistance and BMI-SDS in children affected by diabetes during pregnancy. Methods: 76 children (mean age: 6 years, male:female = 36:40) born to mothers with gestational or pregestational diabetes and nondiabetic women were consecutively included for clinical assessments comprising anthropometrics and metabolic characterization [2-hour glucose tolerance test, leptin, peptide YY (PYY), neuropeptide Y (NPY), ghrelin, growth differentiation factor 15 (GDF-15), and adiponectin]. Results: The level of insulin resistance was associated with BMI-SDS (p < 0.001), leptin (p < 0.001), ghrelin (p = 0.002), age (p < 0.002) and negatively with GDF-15 (p = 0.005). BMI-SDS, leptin and GDF-15 were shown to have independent effects on insulin resistance by using a multiple regression model (additionally including age, and maternal diabetes status), whereas ghrelin lost significance (p = 0.345). No differences were present in adipokines and insulin resistance when children were evaluated by maternal glucometabolic status. However, we observed more strengthened associations between insulin resistance and covariates BMI-SDS and leptin in offspring of diabetic pregnancies. Conclusions: Young children with elevated BMI or leptin are affected by higher indices of insulin resistance, particularly those who were born to mothers with diabetes during pregnancy. The impact of this special risk constellation should be considered in future studies.

AimsImproving glycaemic control in people with Type1 diabetes is known to reduce complications. Our aim was to compare glycaemic control among people with Type1 diabetes using data gathered in regional or national registries. MethodsData were obtained for children and/or adults with Type1 diabetes from the following countries (or regions): Western Australia, Austria, Denmark, England, Champagne-Ardenne (France), Germany, Epirus, Thessaly and Thessaloniki (Greece), Galway (Ireland), several Italian regions, Latvia, Rotterdam (The Netherlands), Otago (New Zealand), Norway, Northern Ireland, Scotland, Sweden, Volyn (Ukraine), USA and Wales) from population or clinic-based registries. The sample size with available data varied from 355 to 173880. Proportions with HbA(1c) We present HbA(1c) data from registries in 19 different countries describing control in 324501 people with Type1 diabetes, across all age groups. These data are the best representation of diabetes care available and therefore describe the state of the art'. We show clearly that Type1 diabetes control is not as good as suggested in guidelines, but that some healthcare systems appear to result in better control than others. These data present a challenge to diabetes services. Leaders in diabetes units/service can compare their local data to our data and encourage improvement.

Introduction Regular physical activity (RPA) is a major therapeutic recommendation in children and adolescents with type 2 diabetes mellitus (T2DM). We evaluated the association between frequency of RPA and metabolic control, cardiovascular risk factors, and treatment regimes. Methods The Pediatric Quality Initiative (DPV), including data from 225 centers in Germany and Austria, provided anonymous data of 578 patients (10–20 yr; mean 15.7 ± 2.1 yr; 61.9% girls) with T2DM. Patients were grouped by the frequency of their self-reported RPA per week: RPA 0, none; RPA 1, 1–2×/wk; RPA 2, >2×/wk. Results The frequency of RPA ranged from 0 to 9×/wk (mean 1.1×/wk ±1.5). 55.7% of the patients reported no RPA (58.1% of the girls). Hemoglobin A1c (HbA1c) differed significantly among RPA groups (p < 0.002), being approximately 0.8 percentage points lower in RPA 2 compared to RPA 0. Body mass index (BMI-SDS) was higher in the groups with less frequent RPA (p < 0.00001). Multiple regression analysis revealed a negative association between RPA and HbA1c (p < 0.0001) and between RPA and BMI-SDS (p < 0.01). The association between RPA and high density lipoprotein (HDL)-cholesterol was positive (p < 0.05), while there was no association to total cholesterol, low density lipoprotein (LDL)-cholesterol or triglycerides. Approximately 80% of the patients received pharmacological treatment (oral antidiabetic drugs and/or insulin) without differences between RPA groups. Conclusion More than half of the adolescents with T2DM did not perform RPA. Increasing physical activity was associated with a lower HbA1c, a lower BMI-SDS, a higher HDL-cholesterol, but not with a difference in treatment regime. These results suggest that regular exercise is a justified therapeutic recommendation for children and adolescents with T2DM.

Increased weight gain has been reported prior to disease onset (accelerator hypothesis) and as a side effect of intensified insulin therapy in type 1 diabetes (T1D). Paediatric studies are complicated by the age-dependency and gender-dependency of BMI, and also by a trend towards obesity in the general population. The aim of this study was to evaluate factors related to the increase in BMI during the course of diabetes in children and adolescents with T1D in a large multicentre survey.Within the DPV database (Diabetespatienten Verlaufsdokumentation) a standardised, prospective, computer-based documentation programme, data of 53,108 patients with T1D, aged20 years, were recorded in 248 centres. 12,774 patients (53% male, mean age 13.4±3.9, mean diabetes duration 4.7±3.0 years and mean age at diabetes onset 8.7±4.0 years) were included in this analysis. Population-based German reference data were used to calculate BMI-SDS and define overweight and obesity.12.5% of T1D patients were overweight and 2.8% were obese. Multiple longitudinal regression analysis revealed that female gender, low BMI at diabetes onset, intensified insulin therapy and higher insulin dose, as well as pubertal diabetes onset, long diabetes duration and onset in earlier calendar years among girls, were related to higher BMI-SDS increase during the course of diabetes (p0.01; all).Intensified insulin regimen is associated with weight gain during T1D treatment, in addition to demographic variables. Optimisation of diabetes management, especially in females, might limit weight gain in order to reduce overweight and obesity together with comorbidities among paediatric T1D patients.

Objective Recent literature suggests an association between type 1 diabetes (T1D) and depression. So far, most studies explored this link in adult populations, with few data being available on diabetes and depression from minors and young adults. This study aimed to look for associations between symptoms of depression/antidepressant treatment and metabolic outcomes of T1D. Methods We conducted an observational study using the German diabetes database (Diabetes-Patienten-Verlaufsdokumentation – DPV) and searched for patients up to the age of 25 yr, with depressive symptoms and/or receiving antidepressant medication. Results Of 53 986 T1D patients below the age of 25 yr, antidepressant medication and/or depressive symptoms were reported in 419 (0.78%). After adjustment for age, gender, diabetes duration and center heterogeneity, minors and young adults with depressive symptoms showed worse outcome parameters such as a higher rate of severe hypoglycemia (0.56 vs. 0.20/patient year, p = 0.005) and more episodes of diabetic ketoacidosis (0.20 vs. 0.07/patient year, p < 0.001). Hemoglobin A1c (HbA1c) was higher in the depression group (74.50 vs. 67.58 mmol/mol, p < 0.001) and young patients with T1D and depression showed longer duration of inpatient treatment (7.04 vs. 3.10 hospital days/patient year, p < 0.001) and more frequent admissions to hospital care (0.63 vs. 0.32/patient year, p < 0.001). Antidepressant medication was recorded in 52.3% of the depressed patients, with selective serotonin reuptake inhibitors (SSRIs) being the most widely described class of antidepressants (29.1%). Conclusions Our findings demonstrate an adverse treatment outcome for young patients with T1D and comorbid depressive symptoms underlining an urgent need for collaborative mental and somatic health care for patients with T1D and depression.

OBJECTIVE The risk of cardiovascular death before the age of 40 is 20-fold higher in patients with type 1 diabetes mellitus (T1DM). Endothelial progenitor cells (EPCs) predict cardiovascular morbidity and mortality in patients without diabetes. We hypothesized that EPCs are modified in children with T1DM and are related to characteristics of T1DM such as glycemic control. RESEARCH DESIGN AND METHODS Children (n = 190; 156 T1DM subjects and 34 control subjects) were included in an observational cohort study and matched for age and sex. EPCs were enumerated by flow cytometry at the beginning (cross-sectional) and 1 year later (longitudinal). To analyze changes of variables during the observation, Δ values were calculated. RESULTS EPCs were significantly reduced in T1DM children versus control subjects (609 ± 359 vs. 1,165 ± 484, P < 0.001). Multivariate regression modeling revealed that glycated hemoglobin A1c (HbA1c) was the strongest independent predictor of EPCs (β = −0.355, P < 0.001). Overall glycemic control at the beginning and end of study did not differ (7.8 ± 1.2 vs. 7.8 ± 1.2 relative %, P = NS), but we observed individual HbA1c changes of −4.30/+3.10 relative %. The strongest EPC increase was observed in the patients with the most favorable HbA1c lowering during the 1-year follow-up. Accordingly, the strongest EPC decrease was demonstrated in the patients with the strongest HbA1c worsening during the time period. CONCLUSIONS This is the first prospective study demonstrating diminished EPCs in children with T1DM. The association of better glycemic control with an increase in EPC numbers within 1 year suggests that a reduction of the high cardiovascular disease burden might be mediated likewise.

To characterize the clinical and immunological features of HLA-typed youth with pediatric onset of type 2 diabetes mellitus (T2DM).One hundred and seven patients with clinically diagnosed T2DM (aged

Background The interaction between type 1 diabetes mellitus (T1DM) and attention deficit hyperactivity disorder (ADHD) in children and adolescents has been studied rarely. We aimed to analyse metabolic control in children and adolescents with both T1DM and ADHD compared to T1DM patients without ADHD. Patients and methods Auxological and treatment data from 56.722 paediatric patients (

Die Hyperglykamie ist wesentlich an der Entstehung mikro- als auch makrovaskularer Spatkomplikationen bei an Diabetes mellitus Typ 2 erkrankten Patienten beteiligt. Wahrend Lebensstilmasnahmen die Eckpfeiler jeder Diabetestherapie bleiben, benotigen im Verlauf die meisten PatientInnen mit Typ 2 Diabetes eine medikamentose Therapie. Bei der Definition individueller Behandlungsziele stellen die Therapiesicherheit bzw. die Effektivitat der Therapie die wichtigsten Faktoren dar. In dieser Leitlinie haben wir die rezenten evidenzbasierten Daten fur die klinische Praxis zusammengestellt.

Im Kindes- und Jugendalter ist im Gegensatz zum Erwachsenenalter der Diabetes mellitus Typ 1 (DMT1) die am haufigsten auftretende Form des Diabetes mellitus (> 95 %). Nach der Diagnosestellung sollte die Betreuung dieser Kinder- und Jugendlichen in einer Kinderabteilung mit Erfahrung in padiatrischer Diabetologie erfolgen und nicht im niedergelassenen Bereich. Eine lebenslange Insulintherapie ist notwendig, wobei diese individuell an das Alter angepasst werden soll (konventionelle Therapie, intensivierte Therapie oder Pumpentherapie). Ein wesentlicher Teil in der Betreuung ist die Schulung von Patienten und Eltern von einem entsprechend ausgebildetem Team. Der von der ISPAD (International Society for Pediatric and Adolescent Diabetes) vorgegebene optimale HbA1c-Wert von

This position statement is based on the cur-rent evidence available on the safety and benefits of continous subcutaneous insulin pump therapy (CSII) in diabetes mellitus with an emphasis on the effects of CSII on glycemic control, hypoglycaemia rates, occur-ance of ketoacidosis, quality of life and the use of insu-lin pump therapy in pregnancy. The current article rep-resents the recommendations of the Austrian Diabetes Association for the clinical praxis of insulin pump treat-ment in children and adults.

OBJECTIVE To investigate if there is a reduced risk of type 1 diabetes in children breastfed or exclusively breastfed by performing a pooled analysis with adjustment for recognized confounders. RESEARCH DESIGN AND METHODS Relevant studies were identified from literature searches using MEDLINE, Web of Science, and EMBASE. Authors of relevant studies were asked to provide individual participant data or conduct prespecified analyses. Meta-analysis techniques were used to combine odds ratios (ORs) and investigate heterogeneity between studies. RESULTS Data were available from 43 studies including 9,874 patients with type 1 diabetes. Overall, there was a reduction in the risk of diabetes after exclusive breast-feeding for >2 weeks (20 studies; OR = 0.75, 95% CI 0.64–0.88), the association after exclusive breast-feeding for >3 months was weaker (30 studies; OR = 0.87, 95% CI 0.75–1.00), and no association was observed after (nonexclusive) breast-feeding for >2 weeks (28 studies; OR = 0.93, 95% CI 0.81–1.07) or >3 months (29 studies; OR = 0.88, 95% CI 0.78–1.00). These associations were all subject to marked heterogeneity (I2 = 58, 76, 54, and 68%, respectively). In studies with lower risk of bias, the reduced risk after exclusive breast-feeding for >2 weeks remained (12 studies; OR = 0.86, 95% CI 0.75–0.99), and heterogeneity was reduced (I2 = 0%). Adjustments for potential confounders altered these estimates very little. CONCLUSIONS The pooled analysis suggests weak protective associations between exclusive breast-feeding and type 1 diabetes risk. However, these findings are difficult to interpret because of the marked variation in effect and possible biases (particularly recall bias) inherent in the included studies.

CLTA4 is relevant for FOXP3(+)Treg cells, and the link between skewed X chromosome inactivation (XCI) and autoimmunity is recognized. The observation of immune dysregulation polyendocrinopathy enteropathy X-linked syndrome and multiorgan endocrine autoimmune phenomena in various members of one family, associated with a CTLA4 polymorphism and skewed XCI, provides an in vivo model of how mechanisms of immune dysregulation may cooperate.

Mortality of cardiovascular diseases in patients with type 1 diabetes is increased 2- to 20-fold compared to non-diabetic individuals. In young adults with type 1 diabetes, cardiovascular events are more often the cause of premature death than nephropathy. The aim of this study was to evaluate the prevalence and extent of cardiovascular risk factors in children and adolescents with type 1 diabetes in Austria. In a cross sectional study data of children with type 1 diabetes

Deterioration of microvascular function may have an early onset in individuals with type 1 diabetes mellitus. We hypothesised that microvascular autoregulation is impaired in children with type 1 diabetes and can be detected non-invasively by postocclusive reactive hyperaemia (PORH). Microvascular autoregulation was assessed in 58 children with type 1 diabetes and 58 age- and sex-matched healthy controls by PORH using laser Doppler fluxmetry. Baseline perfusion, biological zero (defined as a ‘no flow’ laser Doppler signal during suprasystolic occlusion), peak perfusion following occlusion, time to peak and recovery time (time until baseline perfusion is resumed) were recorded and compared between the groups. Peak perfusion was higher in children with type 1 diabetes than in healthy controls (1.7 ± 0.93 AU [arbitrary units] vs 1.29 ± 0.46 AU; p = 0.004), and biological zero was lower in children with type 1 diabetes vs controls (0.14 ± 0.04 AU vs 0.19 ± 0.04 AU; p

To determine whether screening for islet autoantibodies in children prevents ketoacidosis and other metabolic complications at diabetes onset and improves the clinical course after diagnosis.The German BABYDIAB and the Munich Family Study follow children with a first-degree family history of type 1 diabetes for the development of islet autoantibodies and type 1 diabetes. The Diabetes Prospective Documentation (DPV) Initiative registers and collects information on pediatric patients with type 1 diabetes throughout Germany. Here, clinical characteristics at diabetes onset [ketoacidosis, mean hemoglobin A1c (HbA1c), and length of hospitalization] and the 5-yr clinical course (HbA1c and insulin dose) of screened and followed islet autoantibody-positive children (n = 101) and 49 883 non-screened children within the DPV registry were compared.At diabetes onset, children who were followed after screening and were positive for islet autoantibodies had lower HbA1c (8.6 vs. 11%, p< 0.001) and a lower prevalence of diabetic ketoacidosis (3.3 vs. 29.1%, p< 0.001). Screened children also had a shorter hospitalization period at onset (11.4 vs. 14.9 d, p = 0.005). Similar results were observed when the analysis was restricted to 759 non-screened DPV children with a first-degree family history of type 1 diabetes. No differences between screened and non-screened children were observed with respect to HbA1c and insulin dose during the first 5 yr after diagnosis.Screening for islet autoantibodies in children likely leads to earlier diabetes diagnosis resulting in less complications at diagnosis. However, no substantial benefit in the clinical outcome during the first 5 yr after diagnosis was observed.

OBJECTIVE To describe the diabetes phenotype in Wolfram syndrome compared with type 1 diabetes, to investigate the effect of glycemic control on the neurodegenerative process, and to assess the genotype-phenotype correlation. RESEARCH DESIGN AND METHODS The clinical data of 50 patients with Wolfram syndrome-related diabetes (WSD) were reviewed and compared with the data of 24,164 patients with type 1 diabetes. Patients with a mean HbA1c during childhood and adolescence of ≤7.5 and >7.5% were compared with respect to the occurrence of additional Wolfram syndrome symptoms. The wolframin (WFS1) gene was screened for mutations in 39 patients. WFS1 genotypes were examined for correlation with age at onset of diabetes. RESULTS WSD was diagnosed earlier than type 1 diabetes (5.4 ± 3.8 vs. 7.9 ± 4.2 years; P < 0.001) with a lower prevalence of ketoacidosis (7 vs. 20%; P = 0.049). Mean duration of remission in WSD was 2.3 ± 2.4 vs. 1.6 ± 2.1 in type 1 diabetes (NS). Severe hypoglycemia occurred in 37 vs. 7.9% (P < 0.001). Neurologic disease progression was faster in the WSD group with a mean HbA1c >7.5% (P = 0.031). Thirteen novel WSF1 mutations were identified. Predicted functional consequence of WFS1 mutations correlated with age at WSD onset (P = 0.028). CONCLUSIONS Endoplasmic reticulum stress–mediated decline of β-cells in WSD occurs earlier in life than autoimmune-mediated β-cell destruction in type 1 diabetes. This study establishes a role for WFS1 in determining the age at onset of diabetes in Wolfram syndrome and identifies glucose toxicity as an accelerating feature in the progression of disease.

Fritsch M, Rosenbauer J, Schober E, Neu A, Placzek K, Holl RW. Predictors of diabetic ketoacidosis in children and adolescents with type 1 diabetes. Experience from a large multicentre database. Objective: Diabetic ketoacidosis (DKA) remains a major cause of hospitalization and death in children and adolescents with established type 1 diabetes despite DKA preventing strategies. The aim of the study was to determine incidence and risk factors for DKA in a large cohort of young diabetic patients. Methods: This investigation uses the dpv-wiss base containing data on 28 770 patients with type 1 diabetes

Schober E, Wagner G, Berger G, Gerber D, Mengl M, Sonnenstatter S, Barrientos I, Rami B, Karwautz A, Fritsch M, on behalf of the Austrian Diabetic Incidence Study Group. Prevalence of intentional under- and overdosing of insulin in children and adolescents with type 1 diabetes. Objective: The aim of this study was to evaluate the prevalence of insulin under- and overdosing in paediatric patients. Research design and methods: Cross-sectional study including 241 patients (age 14.0 + 2.7 yr, 42.5% males) with type 1 diabetes from 21 diabetic outpatient clinics. Haemoglobin A1c (HbA1c), height, and weight were available from clinical records. Patients were interviewed with the Diabetes Self-Management Profile (DSMP) interview. T test, U test, and chi-squared test were used for comparison. Results: On the basis of the DSMP, 103 (42.7%) patients (group A) showed adherence to the therapeutic insulin regimen, while 71 (29.5%) patients (group B) confessed intentional over and/or under-dosing of insulin. Sixty-seven (27.8%) adolescents (group C) reported management problems leading to unintended inappropriate insulin dosages. In group B, 55 (22.8%) injected higher insulin doses and 58 (24.1%) omitted insulin. Patients of group B compared to group A were older 15.0 (±2.5) vs. 14.0 (±2.5) yr (p < 0.01), older at onset 9.5 (±3.6) vs. 8.3 (±3.8) yr (p = 0.05), were more often girls (69 vs. 45.6%), had a higher actual HbA1c (8.7 ± 1.7 vs. 7.8 ± 1.2%), and a higher average HbA1c in the previous year (8.3 ± 1.6 vs. 7.9 ± 1.2%) (p < 0.01). No significant differences could be found between group A and group C. Conclusion: Intentional overdosing of insulin is almost as prevalent in children and adolescents as insulin omission. Females are more at risk.

Objectives. Pathology of the regulative mechanisms of self seems to be connected with eating disorders (EDs). The present study aimed to explore the hypothesis that there are differences in self-regulation in adolescent girls with Type 1 diabetes with and without EDs. Design. A cross-sectional design was employed comparing patterns of self-regulation in adolescent girls with Type 1 diabetes with and without EDs in two eating status groups. Methods. For the presence of EDs, 76 adolescent girls with Type 1 diabetes were assessed. Of these, 23 were diagnosed with an ED. In addition, dimensions of self-regulation as conceptualized in terms of Kohuts' psychodynamic theory of self were assessed. Results. Adolescent girls with Type 1 diabetes and an ED were higher in three aspects of self-regulation – negative body self, object depreciation, and narcissistic gain from illness – in comparison with their peers without EDs. Conclusions. This study is the first to show evidence of deficits in self-regulation in adolescent girls with Type 1 diabetes and EDs. The importance of evaluating parameters of self-regulation for treatment planning for these youths is outlined.

Diabetes type 1 seems to be more prevalent in epilepsy, and low-carbohydrate diets improve glycemic control in diabetes type 2, but data on the use of the classic ketogenic diet (KD) in epilepsy and diabetes are scarce. We present 15 months of follow-up of a 3 years and 6 months old girl with diabetes type 1 (on the KD), right-sided hemiparesis, and focal epilepsy due to a malformation of cortical development. Although epileptiform activity on electroencephalography (EEG) persisted (especially during sleep), clinically overt seizures have not been reported since the KD. An improved activity level and significant developmental achievements were noticed. Glycosylated hemoglobin (HbA1c) levels improved, and glycemic control was excellent, without severe side effects. Our experience indicates that diabetes does not preclude the use of the KD.

OBJECTIVE: The aims of the study were to determine time trends in cardiovascular risk factors among young Austrian males between 1986 and 2005 and to examine socioeconomic and geographic differences. METHODS: Data on Austrian conscripts were derived from the nationwide compulsory medical investigations held at military induction at 18 years of age. Four cohorts (1986–1990: n = 252,799; 1991–1995: n = 209,266; 1996–2000: n = 208,427; 2001–2005: n = 209,168) were examined with respect to their place of residence and level of education. Height, weight, waist circumference, blood pressure, serum total-cholesterol and triglycerides were measured. Mean body mass index (BMI) was calculated: overweight was defined as BMI between 25 and

Background: Neonatal diabetes mellitus (NDM) is a rare monogenic form of diabetes which is diagnosed in the first 6 months of life. Several studies in the last few years provide information on genetic causes for NDM. Objective: The aim of this study was to identify all patients with diabetes in the first 6 months of life through the Austrian Diabetes Register, which is available since 1989. A retrospective data analyses was performed to calculate the current incidence of NDM. Subjects and Methods: Ten patients were registered with diabetes onset within the first 6 months of life in the Austrian Diabetes Register. Evaluation of detailed clinical data was performed by sending a questionnaire to all diabetes centers. Results: Ten patients from nine different families with NDM were diagnosed in Austria from 1989 until September 2007. Seven patients (one male, six females) had transient NDM (TNDM), three (two males, one female) showed a permanent course [permanent neonatal diabetes mellitus (PNDM)]. One had immunodeficiency, polyendocrinopathy and enteropathy X-linked (IPEX) syndrome and another showed aplasia of the pancreas; no genetic etiology was found in the third case. In three out of seven patients with a transient course of NDM a genetic diagnosis was possible. Two female siblings had activating point mutations in the ABCC8 gene, although one patient had paternal uniparental isodisomy of chromosome 6q24. One patient's family did not consent to genetic testing. Conclusions: The incidence of NDM in Austria is 1/160 949, with an incidence of 1/ 536 499 for PNDM and 1/229 928 for TNDM.

Diabet. Med. 27, 709–712 (2010) Abstract Aims The aim of this study was to elucidate the entities and the frequency of neonatal diabetes mellitus (NDM) in a large representative database for paediatric diabetes patients in Germany and Austria. Methods Based on the continuous diabetes data acquisition system for prospective surveillance (DPV), which includes 51 587 patients with onset of diabetes before the age of 18 years from 299 centres in Germany and Austria, we searched for patients with onset of diabetes mellitus in the first 6 months of life. Results Ninety patients were identified, comprising 0.17% of all paediatric cases in the DPV registry. This represented an incidence of approximately one case in 89 000 live births in Germany. A monogenic basis for NDM was established in 30 subjects (seven UPD6, 10 KCNJ11, seven ABCC8, two FOXP3, two PDX1, one INS, one EIF2AK3). Pancreatic hypoplasia or agenesis was reported in 10 patients and seven subjects were classified as having Type 1 diabetes by their centres. Transient neonatal diabetes (TNDM) accounted for approximately 10% of all cases with NDM. No aetiology was defined in 41 subjects, which may reflect incomplete genetic testing or novel genetic aetiologies. Conclusion Based on a large database, we identified a higher rate of NDM in Germany than has been reported previously. Full molecular genetic testing should be performed in all patients diagnosed before 6 months of age.

aChildren’s Hospital auf der Bult, Hannover, Germany; bthe Children’s Hospital at Westmead, NSW, Australia; cUniversity of Helsinki, Finland; dMedical University Vienna, Austria; eUniversity of Genova, Italy; fUniversitat Ulm, Uni West, Germany; gUniversity of Sydney, Australia Corresponding author: Prof. Dr. med. Olga Kordonouri Diabetes Center for Children and Adolescents Children’s Hospital auf der Bult Hannover, Germany Tel: +49 511/8115-3340; fax: +49 511/8115-3334; e-mail: kordonouri@hka.de

Insulin dependent diabetes mellitus shows a strong familial predisposition and an unexplained geographical variation in incidence. It is not known whether the risk of IDDM in first degree relatives depends on the risk in the background population. The aim of the study was to assess the prevalence of IDDM in parents and siblings of newly diagnosed children with IDDM in Austria, a known area of low risk for IDDM. The family history data of all diabetic children (< 15 years) diagnosed between 1988-1994 in Austria were analysed. The cumulative incidence of IDDM in siblings of newly diagnosed diabetic children was 0.0026772 cases/year, this means 29.7 times increased risk compared to the background population. Of the diabetic children 5.8% had at least one parent with IDDM and the prevalence of IDDM in fathers (3.9%) was higher (p = 0.015) compared to mothers (1.9%). The risk of IDDM tended to be higher for offsprings of diabetic fathers (OR 3.8, p < 0.003) in families with 2 or more children than in single child families, where the prevalence was 4.2% both in fathers and mothers. In conclusion the prevalence of IDDM in parents of diabetic children in Austria was lower than reported in populations with high IDDM incidence. This may reflect a lesser degree of genetic predisposition of the Austrian population. The prevalence of IDDM in siblings was similar to that in high risk populations. We saw an interaction of gender of the diabetic parent and diabetic offspring and the family size.

BackgroundThe relationship between metabolic control and cognitive function in adolescents with type 1 diabetes (DM type 1) is not clear. We compared the quality of glycemic control (GC) and cognitive measures in adolescents with DM type 1 to find out if the quality of diabetes management is related to cognitive impairment.MethodWe assessed executive functions (EFs) and other neuropsychological and psychosocial variables in 70 adolescent patients with DM type 1 and 20 age-matched controls. Patients were divided into two groups according to their last hemoglobin A1c (HbA1c): acceptable (HbA1c 5.9–8.0%, mean 6.9%, 36 patients, mean age 14 years) and non-optimal (HbA1c 8.2–11.6%, mean 9.3%, 34 patients, mean age 15.6 years).ResultsWe found impaired EFs, mainly problems of concept formation (p=0.038), cognitive flexibility (p=0.011) and anticipation (p=0.000), in the patients with DM type 1. Both groups did not differ in intelligence, most assessed EFs and adjustment to chronic illness (Youth Self-Report; YSR). Younger patients (ConclusionsDM type 1 is associated with cognitive deficits in adolescents independent of the quality of metabolic control and the duration of the disease. These deficits are probably related to the disease, especially in patients with early-onset diabetes.

Objective: Type 1 diabetes mellitus (T1DM) is associated with other autoimmune diseases such as celiac disease (CD) and Hashimoto thyroiditis. The aim of this study was to evaluate the screening frequency for CD and thyroid antibodies in a multicentre survey. Methods: The Diabetes Patienten Verlaufsdokumentationssystem (DPV) initiative is based on standardized, prospective, multicentre documentation in children and adolescents with diabetes. Data from 31 104 patients

Aims/hypothesis To assess the use of paediatric continuous subcutaneous infusion (CSII) under real-life conditions by analysing data recorded for up to 90 days and relating them to outcome.Methods Pump programming data from patients aged 0-18 years treated with CSII in 30 centres from 16 European countries and Israel were recorded during routine clinical visits. HbA(1c) was measured centrally.Results A total of 1,041 patients (age: 11.8 +/- 4.2 years; diabetes duration: 6.0 +/- 3.6 years; average CSII duration: 2.0 +/- 1.3 years; HbA(1c): 8.0 +/- 1.3% [means +/- SD]) participated. Glycaemic control was better in preschool (n=142; 7.5 +/- 0.9%) and pre-adolescent (6-11 years, n=321; 7.7 +/- 1.0%) children than in adolescent patients (12-18 years, n=578; 8.3 +/- 1.4%). There was a significant negative correlation between HbA(1c) and daily bolus number, but not between HbA(1c) and total daily insulin dose. The use of 7.5%. The incidence of severe hypoglycaemia and ketoacidosis was 6.63 and 6.26 events per 100 patient-years, respectively.Conclusions/interpretation This large paediatric survey of CSII shows that glycaemic targets can be frequently achieved, particularly in young children, and the incidence of acute complications is low. Adequate substitution of basal and prandial insulin is associated with a better HbA(1c).

Childhood overweight and obesity--especially among migrant children--are health problems in several European countries. The prevalence rates of overweight and obesity among 936 migrant boys of low socio-economic status from Turkey and former Yugoslavia were compared with those of Austrian boys in Vienna. In the longitudinal study, children were measured at the ages of 6, 10 and 15 years. Weight status was estimated by means of the body mass index and percentile curves were used for weight status classification. The prevalence of overweight and obesity was high among migrant boys as well as Austrian boys at all age groups. Ten-year-old boys from Yugoslavia exhibited the highest percentage of overweight (nearly 38%). The lowest percentage of overweight was among 6-year-old Austrian boys (17.2%). Being overweight or obese at the age of 6 years increased the risk of being overweight at 10 and 15 years significantly (p

Aim: To assess the neurological and clinical long-term outcome of patients diagnosed with congenital hyperinsulinism (CHI) in Austria. Patients and Methods: Fourteen patients diagnosed with CHI (1978-2000) were investigated retrospectively by reviewing hospital records. Thirteen of them were evaluated with either a questionnaire or clinical, neurological and biochemical investigations (age at evaluation 4.2- 25.5 years) in a follow-up study in the year 2004. Results: Fifty percent of the patients needed a pancreatectomy. The prevalence of mental retardation was 31%, of epilepsy 15% and of pancreatic insufficiency 14%. None of our patients had developed diabetes mellitus. Additionally the prevalence of obesity was 43% in patients after pancreatectomy. Sixty-nine percent of the patients had no further treatment at the time of follow-up. Conclusion: Despite early diagnosis and intensive treatment, 31% of the patients presented with mental retardation.

Background: To investigate the rapid increase in incidence of type 1 diabetes mellitus (T1DM) in children

Childhood overweight and obesity, especially among migrant children, are current health problems in several European countries. In the present study the prevalence of overweight and obesity among migrant children from Turkey and the former Yugoslavia was documented and compared with that of Austrian children in Vienna. Anthropometric data from 1786 children were collected at the ages of 6, 10 and 15 years. Body mass was estimated by means of the body mass index and percentile curves were used to determine weight status. The prevalence of overweight and obesity was found to be significantly higher among migrant children. Children and adolescents from the former Yugoslavia and Turkish girls exhibited especially high rates of overweight and obesity. Biosocial and cultural factors are discussed as causes of these observations.

ZusammenfassungDie Prävalenz der Zöliakie ist bei Kindern und Jugendlichen mit Diabetes mellitus Typ 1 höher als in der Normalbevölkerung. Meistens liegt eine atypische oder silente Form der Zöliakie vor. Die beiden Erkrankungen sind assoziiert, es sind Autoimmunerkrankungen mit einem gemeinsamen genetischen Hintergrund. Weiters nehmen die Prävalenz der Zöliakie sowie die Inzidenz des Diabetes mellitus Typ 1 laufend zu. Die meisten diabetischen Patienten mit Zöliakie werden erst durch ein gezieltes Screening mittels Antikörper entdeckt und es sind eher jüngere Patienten betroffen. Der Goldstandard der Zöliakie-Therapie ist nach wie vor eine lebenslange glutenfreie Diät. Widersprüchliche Ansichten bestehen über den klinischen Benefit einer frühzeitigen Diagnose der silenten Zöliakie durch Screening von Patienten mit Diabetes mellitus Typ 1 und frühzeitiger Therapie mit einer glutenfreien Diät bei diesen Patienten.Eine klare Aussage, ob die glutenfreie Diät in diesen Fällen einen Einfluss auf die Längen- und Gewichtsentwicklung bzw. die metabolische Kontrolle hat, ist derzeit noch unklar.

To investigate the influence of celiac disease (CD) on growth and metabolic control in a nationwide cohort of children and adolescents with type 1 diabetes (T1D).We analyzed data from 19,796 pediatric patients with T1D in the German pediatric multicenter DPV-database for occurrence of CD.CD-specific antibodies were present in 1326 patients (6.7%). The diagnosis was confirmed in 127 patients (0.6%) by small-bowel biopsy. Female subjects were significantly more predisposed to have T1D and CD. The CD-affected patients in our cohort were significantly younger at diabetes onset. Furthermore, they had significantly lower height-SDS at onset (-0.49 vs -0.06, P.05), a difference that increased during the course of the disease (-0.80 vs -0.26 after 9 years of diabetes, P.05). In addition, body mass index-SDS significantly differed between the groups (0.22 vs 0.47, P.05). Evidence for thyroid disease was more commonly observed in the T1D with CD group (6.3% vs 2.3%, P.05). HbA1c values were lower in the patients with T1D and CD.The CD-positive patients were characterized by earlier onset of diabetes and decreased growth and weight gain. These findings emphasize the clinical relevance of celiac disease in patients with autoimmune diabetes.

Diabetes mellitus is one of the major causes of morbidity and mortality in EU/EFTA countries. Monitoring risk factors for diabetes and its complications will offer the possibility to evaluate the development in time as well as the influence of possible interventions. In this investigation a list with core and secondary indicators is proposed. Availability of these indicators and their data sources is discussed. An important variability of data sources is used in EU/EFTA countries, interfering with the comparability of the outcome. Further harmonisation as well as continuous evaluation of data sources will be necessary to provide reliable tools to monitor diabetes mellitus and its outcome on a routine basis.

The prevalence of celiac disease (CD) in children with diabetes mellitus type 1 (DM) is significantly higher than in the nondiabetic population. Most patients with DM and associated CD do not experience typical gastrointestinal symptoms of CD. There is no agreement on the necessity of screening and management of silent CD for patients with DM or on the time scale for screening. Only few data on follow-up evaluation of children with DM and CD-related antibodies are available.One hundred fifty-seven patients with DM (mean age, 14.8 years; range, 4-21 years; male, 83) were screened with endomysial antibodies (EMA) between 1993 and 2001. A follow-up period of at least 3 years, with at least 2 EMA measurements, was possible. Group 1 comprised 37 patients whose first measurement was at the onset of DM. Group 2 comprised 120 patients whose first measurement was during the course of the disease. In patients with positive EMA, small bowel biopsy was performed. Thyroid peroxidase (TPO), thyroglobulin (TgA), glutamate decarboxylase (GAD), antiinsulin (IAA), and islet cell antibodies (IA2) were measured in all patients.EMA was positive in 16 patients, in 5 at onset of DM and in 11 during the course of DM (mean duration, 33.6 months; range, 11-105 months). Biopsy results showed normal mucosa in seven patients, increased intraepithelial lymphocyte counts in one, and flat mucosa in eight. There was no significant difference between EMA-positive and EMA-negative patients regarding height, weight, HbA1c level, frequency of hypoglycemia or hyperglycemia, TPO, TgA, GAD, IAA, or IA2.This study emphasizes the high prevalence of CD in patients with DM. Although several patients already had positive EMA titers at the onset of DM, seroconversion may also occur during the course of the disease. Screening for CD with EMA or tissue transglutaminase should be included in the initial investigation of DM, but should also be repeated over time to detect late seroconversion.

One of the main difficulties in managing type 1 diabetes mellitus (T1DM) in the young is nutritional treatment. Studies have shown that adolescents (particularly adolescent girls and young women) have an increased risk for clinical and sub-clinical eating disorders. Adjustment to the nutritional regimen and, consequently, to the management of the disease in adolescence seems to involve a complex interplay of various psychosocial and biological aspects. The aim of this review is to consider the relationship between T1DM and eating disorders in adolescence in the light of some important biological psychological and familial factors. Further research is required in order to detect the degree of the interactions between these factors in adolescents with T1DM.

This study was performed to investigate whether the body mass index (BMI) of diabetic children at manifestation is related to the non-random risk distribution in Austria and whether there is an association with the BMI of the background population. All newly diagnosed cases aged15 years (n=1644) from 1989 to 2000 were allocated to districts using the postal code. BMI at the time of diagnosis was derived from the Austrian incidence data sheet in 99.3% of the registered cases. Data on BMI of the whole population14 years of age for the year 1991 and birth weight for the years 1989-1999 were derived from Statistics Austria. Linear regression as well as ecological regression (Poisson) models with correction for non-linearity were used. BMI at diabetes manifestation was positively associated with year of diagnosis (P0.05) and the age at diagnosis (P0.01). An increase in BMI when moving from West to East (P0.01) and a higher BMI at diagnosis in boys than girls (P0.01) was observed. The ecological regression model showed a positive significant association of BMI in newborns and a positive trend of BMI in the background population with incidence rates.our data support the hypothesis that obesity may be a risk factor not only for type 2 but for type 1 in children acting as an accelerator for the clinical manifestation.

We analyzed the associations of environmental factors with the regional distribution of Type 1 diabetes mellitus in Austria. All newly diagnosed cases (n=1449) from 1989 to 1999 were allocated to districts using the postal code. Nitrate content of the water was measured by the Austrian Federal Environmental Agency. Data on infant mortality, population density, and percentage of employment by industry were derived from Statistics Austria. An inverse effect was seen between the proportion of children younger than 15 years of age and the risk ratio (P.01). Infant mortality, population density, and percentage of persons with employment in industry were not of significant influence. The mean nitrate level was positively associated (P=.07). In regions with a higher percentage of children younger than 15 years of age, fewer children developed diabetes, which is in agreement with the observation that early social mixing is a protective factor. Nitrate levels may have a confounding effect.