Glycemic control and clinic attendance of emerging adults with type 1 diabetes at a transition care clinic.

BACKGROUND: Emerging adulthood is a challenging period for diabetes management. Our aim was to determine whether a dedicated transition clinic for emerging adults with type 1 diabetes can improve glycemic control and visit attendance. METHODS: An observational study of 53 emerging adults (30 males) treated during 2010-2014 in a newly established transition clinic. The clinic was operated jointly by pediatric and adult endocrinologists and included a transition coordinator. Data collected included the source of referral, HbA1c levels, frequency of visit attendance, and acute complications. For 27 patients who had attended the pediatric clinic at the same medical center, data from up to 2 years preceding the transition were also collected. Patients filled the Diabetes Quality of Life-Youth questionnaire at the transition and 1 year later. RESULTS: Mean ± SD age at the transfer to the transition clinic was 22.1 ± 2.7 years; mean disease duration was 8.4 ± 5.0 years. Follow-up duration at the transition clinic was 1.2 ± 1.1 years. Mean HbA1c levels decreased from 67 mmol/mol (95 % CI 63-72) [8.3 % (95 % CI 7.9-8.7)] at transfer to 57 mmol/mol (95 % CI 52-63) [7.4 % (95 % CI 6.9-7.9)] after 1 year (p < 0.001). Thirty-six patients (68 %) attended three or more visits during their first year in the transition clinic. The impact of diabetes on quality of life, disease-related worries, and life satisfaction did not change significantly during 1-year attendance in the transition clinic. CONCLUSIONS: A dedicated transition clinic for emerging adults, with tailored support according to the developmental needs of emerging adulthood, showed improved glycemic control and visit attendance.

Prevalence of overweight, obesity and metabolic syndrome components in multiple sclerosis patients with significant disability.

BACKGROUND AND PURPOSE: Information about metabolic comorbidities in patients with multiple sclerosis (MS) is scarce. Our aim was to examine the prevalence of the metabolic syndrome (MetS) and its components in patients with long duration of MS and significant disability. METHODS: Demographic and clinical data, weight, height, waist circumference, blood pressure, and levels of fasting glucose, triglycerides and high density lipoprotein cholesterol (HDL-C) were obtained from 130 MS patients with Extended Disability Status Scale (EDSS) score ≥3.0. RESULTS: Seventy-two percent were female, mean ± SD age 55.8 ± 6.0, range 45-65 years, disease duration 18.2 ± 10.1 years, EDSS 5.5 ± 1.0. Obesity [body mass index (BMI) ≥ 30 kg/m(2) ] was present in 18.5% and overweight (BMI 25.0-29.9 kg/m(2) ) in 34.6%. The prevalence of the MetS was 30% with no gender difference. Fifty-six percent had central obesity by waist circumference, 28% treated hypertension, 45.8% elevated blood pressure, 11% type 2 diabetes mellitus, 31.4% treated dyslipidemia, 28.8% elevated triglyceride levels and 31.4% had low HDL-C. MS patients with MetS were significantly older (59.0 ± 5.5 vs. 53.8 ± 5.5, P < 0.0001) and heavier (BMI 29.0 ± 6.9 vs. 25.1 ± 4.7, P = 0.0009). There were no differences between the groups in neurological disability by the EDSS (5.7 ± 1.0 vs. 5.4 ± 1.0), disease duration (18.4 ± 9.9 vs. 18.2 ± 10.2 years) and number of steroid courses received (6.6 ± 9.5 vs. 6.3 ± 8.4). CONCLUSIONS: Compared to the general population, adult disabled MS patients had lower rates of obesity and overweight, as assessed by BMI. Despite these reduced rates, the prevalence of the MetS was similar to the general population. Specifically higher rates of increased waist circumference were found, suggesting that the lower BMI may be misleading in terms of health risk.

The use of continuous glucose monitoring systems in a pediatric population with type 1 diabetes mellitus in real-life settings: the AWeSoMe Study Group experience.

AIMS: The aim of the study was (a) to compare annual glycemic control in pediatric patients with type 1 diabetes mellitus (T1DM) who used a healthcare-funded continuous glucose monitoring system (RT-CGMS) to that of those who performed self-monitoring blood glucose (SMBG) only, in a real-life setting, and (b) to define parameters associated with compliance and glycemic control. METHODS: A total of 149 youth with T1DM (52.3 % females), mean age 11.8 ± 3.6 years, and 83 in the CGMS group were followed prospectively for 12 months. Glycemic control parameters and compliance to RT-CGMS were assessed periodically. RESULTS: Glycemic parameters did not differ significantly between the groups during follow-up periods. The time spent with RT-CGMS decreased and only 38 % used it for more than 75 % of the time during the 12 months (consistent users). Mean HbA1c decreased by 0.27 % in consistent users and increased by 0.21 % among intermittent users (used RT-CGMS less than 75 % of time), p = 0.013. Consistent users were younger 10. 6 ± 4.2 vs. 12.5 ± 3.6, p = 0.07, and had higher frequency of SMBG at baseline, 10.6 ± 4.9 vs. 6.3 ± 2.8, p = 0.011. CONCLUSIONS: The adoption of RT-CGMS was low, even in a healthcare system that funds its use. Caregivers should consider patient characteristics when recommending RT-CGMS use.

Novel insights into adrenal insufficiency in childhood.

Adrenal insufficiency (AI) is a rare disease in childhood and adolescence that results from disruption in the hypothalamic-pituitary-adrenal axis. Pediatricians should be familiar with this entity since acute adrenal crisis is a life-threatening condition that requires immediate treatment. In the early stages of AI, the clinical manifestations may be subtle and non-specific; thus, they are frequently unrecognized. The main therapeutic approach in all forms of adrenal insufficiency is glucocorticoid replacement; the dose should be titrated appropriately to avoid under or overtreatment. Patient and family education is particularly important, to enable adjustment of dosage replacement therapy during stress and to prevent crisis. This article summarizes the current knowledge of AI and provides new insights on its management in children.

Parental sleep quality and continuous glucose monitoring system use in children with type 1 diabetes.

To compare sleep quality and sleep-wake patterns in parents of children with type 1 diabetes before routine use of the continuous glucose monitoring system (CGMS) and while using it. Thirteen parents completed the Pittsburg Sleep Quality Index (PSQI), a 7-day sleep diary, and wore an actigraph (a wristwatch-size motion detector) during the night for 1 week before pediatric use of CGMS and 4-8 weeks after initiating routine use of the CGMS. Mean age of parents (ten mothers, three fathers) was 39 (range 32-47) years; mean age of children was 9.3 years (range 5.5-16.5 years); mean disease duration was 3.4 (range 0.6-11.2) years. PSQI total score demonstrated similar quality of sleep with and without use of the CGMS (4.6 and 4.9, respectively, p = 0.45). Six of the 13 parents reported severe sleep problems (PSQI ≥ 5) with and without the CGMS. The sleep diary indicated a greater number of awakening episodes during CGMS use than without the CGMS (1.6 and 1, respectively, p = 0.03), and actigraphy documented an increase in the number of wake bouts (22.9 and 19.7, p = 0.03) as well as in total wake time (48.3 and 42.2 min, p = 0.03) during CGMS use as compared with the period prior to CGMS use. Although self-perception of sleep quality remained unchanged, CGMS use appeared to affect actual parental sleep continuity somewhat negatively. This should be made clear to parents who may hold expectations of improvement in sleep quality following initiation of CGMS use.

Benefits of continuous subcutaneous insulin infusion (CSII) therapy in preschool children.

OBJECTIVE: The incidence of type 1 diabetes mellitus (T1DM) in young children has increased considerably over recent years. The purpose was to examine the effectiveness and safety of continuous subcutaneous insulin infusion (CSII) therapy in preschool children with T1DM. METHODS: A retrospective chart review of 113 children diagnosed with T1DM while younger than age 6 years. Mean age at diagnosis was 3.5±1.5 years and mean duration of follow 9.7±7.0 years. Patients were divided into 3 groups. Group1 initiated CSII therapy before the age of 6 years (n=26), Group 2 was treated with multiple daily injections (MDI) throughout follow-up (n=34), and Group 3 initiated CSII after age 6 (n=53). Metabolic control was assessed by HbA1C levels and safety by rates of severe hypoglycemia and diabetic ketoacidosis (DKA) events. RESULTS: In Group 1, the highest mean HbA1C value (8.5%) was observed 1-2 years prior to CSII initiation. During the 5 year period following CSII initiation, mean HbA1C levels ranged between 7.4 and 8.0%. Throughout the entire follow-up period, mean HbA1C levels were lower for Group 1 than Group 2 (p=0.05). In Group 3, mean HbA1C level decreased from 8.7% pre-CSII to 8.3% post-CSII (p<0.001). Nevertheless HbA1C levels remained higher than for those who started pump therapy before age 6 (p=0.02). CONCLUSIONS: Our study demonstrated better metabolic control in pre-school children treated with CSII compared to those treated with MDI. This benefit sustained for 5 years after CSII initiation and was not accompanied by increased risk of severe hypoglycemia or DKA events.

Enuresis--an unattended comorbidity of childhood obesity.

OBJECTIVE: Obstructive sleep apnea (OSA), attention deficit/hyperactivity disorder (ADHD), type 2 diabetes mellitus and psychopathological problems co-occur at increased rates among both obese and enuretic children. We hypothesized that the prevalence of enuresis will be increased in obese children and adolescents. DESIGN: A cross-sectional study. SUBJECTS: 281 children and adolescents aged 7-18 years, who completed a questionnaire regarding enuresis, medical conditions and sociodemographic parameters; 158 were normal weight, 37 overweight (85thBMI (body mass index)<95th percentiles) and 86 obese (BMI95th percentile). MAIN OUTCOME MEASURE(S): Occurrence of enuresis among obese children and adolescents. RESULTS: Enuresis was reported in 14 (8.8%) normal weight, 6 (16%) overweight and 26 (30%) obese youth. Odds ratio (OR)=6.5, 95% confidence interval (CI)=2.67-15.78 for enuresis among obese compared with normal weight (P<0.0001). Each increment of one BMI-Z score unit was associated with an increased risk of enuresis, OR of 2.14, 95% CI (1.46-3.12), P=0.00008. Male gender (OR 2.84, 95% CI (1.10-5.58), P=0.028), first-degree relative with current/past enuresis (OR 4.24, 95% CI (1.62-11.08), P=0.003), voiding dysfunction symptoms (OR 3.067, 95% CI (1.05-9.00), P=0.041) and ADHD (OR 2.31, 95% CI (0.99-5.34), P=0.051) increased the risk of enuresis. OSA-related symptoms, academic achievements in school, sharing a bedroom, family size relative to number of rooms in home, parental education, family status and religious observance were not found to increase the risk for enuresis. CONCLUSIONS: Obese children are at increased risk for enuresis. Enuresis should be clarified during the primary workup of every obese child and adolescent.

Type 1 diabetes in pre-school children--long-term metabolic control, associated autoimmunity and complications.

AIMS: To identify clinical characteristics and co-morbidity rates of children diagnosed with Type 1 diabetes mellitus at younger than 6 years of age. METHODS: Data were obtained from a retrospective chart review of 103 patients diagnosed with Type 1 diabetes at younger than 6 years (study group) and 220 patients at older than 6 years (comparison group). Measures of glycaemic control and occurrence of co-morbidities (coeliac disease, autoimmune thyroid disease, hypertension, nephropathy and retinopathy) were compared. RESULTS: The mean follow-up period was more than 8 years. For the study group, mean HbA(1c) levels ranged from 64 mmol/mol to 66 mmol/mol (8.0-8.2%) until age 10 years, and then rose to 73 mmol/mol (8.8%). The HbA(1c) levels were higher in the study than in the comparison group for comparable ages (P = 0.003). After adjustment for duration of diabetes this difference was not significant. The overall rate of severe hypoglycaemic events was greater in the study group than in the comparison group (P = 0.03). Kaplan-Meier diagnosis rates of celiac disease, 10 years after Type 1 diabetes diagnosis, were 14.4% and 4.2% in the study and comparison groups, respectively (P log-rank = 0.03). There were no differences in rates of autoimmune thyroid disease, hypertension, nephropathy or retinopathy. CONCLUSIONS: Children diagnosed with Type 1 diabetes before the age of 6 years were in greater risk of developing celiac disease, compared with children diagnosed after the age of 6 years. For children diagnosed with Type 1 diabetes aged under 6 years, good metabolic control was achievable until age 10 years, after which it deteriorated. Higher HbA(1c) levels observed in children diagnosed before the age of 6 years were associated with longer duration of disease.

Prevalence of overweight in kindergarten children in the centre of Israel - association with lifestyle habits.

OBJECTIVES: (1) To determine the prevalence of overweight and at risk for overweight in a sample of 4- to 6-year-old Israeli children; and (2) to delineate factors which may be associated with excess of overweight such as food intake, food habits physical and sedentary activity, family and socio-economic factors. METHODS: In a cross-sectional survey in six kindergartens in the centre of Israel, 204 children (104 boys), mean age 5.5 years were studied. RESULTS: In total, 40% of the children were either at risk for overweight (25%) or overweight (15%). Parents of one-third of all children reported their children ate unbalanced meals; however, caloric intake was not different between the weight groups. Overweight children were reported to eat according to mood status and preferred eating candies. Knowledge of dietary requirements was fairly good and similar in all three weight groups. Screen viewing time was more than twofold greater than the recommended in all weight groups. The rate of parental obesity was not different in the weight groups. CONCLUSIONS: A high rate of overweight was documented among pre-schoolers in the centre of Israel. Poor lifestyle habits were noted in about one-third of all children; however, no association was found between the poor lifestyle habits and overweight but more overweight children eat according to mood status and preferred eating candies as a snack.

Long-term neurodevelopmental outcome in conservatively treated congenital hyperinsulinism.

BACKGROUND: Congenital hyperinsulinism (CH) is treated surgically in many centers (near-total and partial pancreatectomy for diffuse and focal disease respectively). Most patients treated with near-total pancreatectomy developed diabetes during childhood/puberty. CH patients are at increased risk of neurodevelopmental disorders, some being severe, which are reported to occur in 14-44% of patients from highly heterogenous cohorts. Over the last few decades, we have treated children with CH conservatively without surgery. The aim of this study was to assess the neurodevelopmental outcome of these patients. DESIGN AND METHODS: The study included 21 Ashkenazi CH medically treated patients: 11 homozygotes (diffuse disease) and 9 heterozygotes with mutations on the paternal allele (presumed focal disease). The mean age was 13.7 years (range 8-23). Neurodevelopmental outcomes were assessed by telephone interviews of parents, using a standard questionnaire. Closest age siblings of CH patients served as controls. RESULTS: Ten CH patients had perinatal seizures of short duration. Four had post-neonatal seizures, which remitted entirely. During early childhood, four patients (19%) had hypotonia, eight (38%) had fine motor problems, seven (33%) had gross motor problems (clumsiness), and one had mild cerebral palsy. Three patients (14%) had speech problems. Eight patients required developmental therapy, compared to one in the control group. Most of these problems were resolved by age 4-5 years. At school age, all were enrolled in regular education, some excelled in their studies, 6 out of 21 patients (29%) had learning problems (2 out of 21 controls). None had overt diabetes. CONCLUSIONS: Good neurodevelopmental outcome was observed in our conservatively treated CH patients, with no diabetes as reported in patients undergoing pancreatectomy.

Health-related quality of life among children and adolescents: associations with obesity.

OBJECTIVE: To assess the impact of obesity on health-related quality of life (HRQOL) of children, and to compare HRQOL scores of obese children in a hospital versus community setting. DESIGN: A cross-sectional study in two clinical samples. SUBJECTS: A total of 182 children and adolescents recruited from the community pediatric clinics and a hospital-based obesity clinic. MEASUREMENTS: Obesity was defined as body mass index (BMI) >95th percentile for age and gender. Subjects were divided into quartiles of BMI Z-scores each containing 45 or 46 children. The first two quartiles correspond to normal BMI, 3rd and 4th quartiles represent moderate and severe obesity, respectively. HRQOL was assessed by the Pediatric Quality of Life Inventory (PedsQL) questionnaire submitted by both children and their parents. RESULTS: Obese children reported significantly lower HRQOL in physical, social and school domains compared with normal weight children (P<0.01). Analyzing results of HRQOL by BMI quartiles showed that the emotional and school domains scores of the moderately obese children were similar to the normal BMI quartiles. Only in the 4th quartile, that of children with severe obesity, were scores significantly lower. In contrast, in both physical and social domains scores decreased progressively with increased BMI Z-scores. In the obese group, parents' scores were lower than the children's in all domains. HRQOL scores of obese children assessed in the hospital clinic were similar to that of obese children assessed in the community. CONCLUSIONS: Moderately obese children had similar emotional and school HRQOL scores as normal weight children, whereas in the physical domain, a significant difference was documented even in moderate obesity. Parents of obese children perceived their child's HRQOL lower than the children themselves. We believe that intervention programs aimed at improving HRQOL should be directed to both parents and children. Understanding the resilience of moderately obese children in school and emotional domains may assist us in the management of childhood obesity.

Greater prevalence of iron deficiency in overweight and obese children and adolescents.

OBJECTIVE: To assess whether overweight children and adolescents, who often have poor dietary habits, are at increased risk of iron deficiency (ID). METHODS: The study sample included 321 children and adolescents followed in two endocrine centers in Israel between 1999 and 2001. The subjects were divided into three groups on the basis of body mass index (BMI) for age and gender as follows: group 1-BMI below 85th percentile (normal weight); group 2-BMI above 85th, but below 97th percentile (overweight); and group 3-BMI above 97th percentile (obese). ID was defined as iron levels <8 micromol/l (45 mcg/dl), and iron-deficiency anemia (IDA) was defined as ID and hemoglobin level below 2 standard deviation score (SDS) for the mean for age and gender. RESULTS: Iron levels below 8 micromol/l (45 mcg/dl) were noted in 38.8% of the obese children and 12.1% of the overweight children, compared with 4.4% of the normal-weight group (P<0.001). There was a significant negative correlation of low iron levels with BMI SDS (r=-0.44, P<0.001), but not with age or gender. Among the children with ID, 26.6% also had IDA. Groups 1, 2, and 3 accounted for 6.7%, 35%, and 58.3% of the children with IDA, respectively. CONCLUSIONS: ID is common in overweight and obese children. A significantly greater proportion of obese than normal-weight children have IDA. Insufficient dietary intake of iron, whether absolute or relative to body mass, and increased iron needs may be a result of unbalanced nutrition or repeated short-term restrictive diets. Because of potentially harmful effects of ID, obese children should be routinely screened and treated as necessary.

Multiple sclerosis in childhood and adolescence: clinical features and management.

The presentation of multiple sclerosis (MS) in childhood has traditionally been thought to be rare. However, more paediatric cases are now being reported, as a result of progress in diagnostic techniques with the use of sensitive imaging modalities of the brain and spinal cord. Management from an early age and the availability of new treatment options have changed the outcome of paediatric MS. Drugs currently available for treatment, such as beta-interferons, copolymer-1 and intravenous immunoglobulin G, have been found to reduce relapse rate, disease severity and progression to disability in adults, but have not been investigated in children and adolescents. The overall outcome of MS in children is apparently no worse than in adults and the disease may even be less aggressive in children. In juvenile MS, disease progression does not appear to be related to age of onset, severity of neurological involvement or mono/polysymptomatic involvement at presentation. The potential to treat MS has significantly changed the prognosis. Early diagnosis is important, as early treatment can prevent or delay the development of disability.

Type 1 diabetes environmental factors and correspondence analysis of HLA class II genes in the Yemenite Jewish community in Israel.

OBJECTIVE: The Israeli Yemenite Jewish community has displayed an exceptionally rapid increase in the frequency of type 1 diabetes, having the highest rate of all Israeli ethnic groups. We studied the role of the environment, in relation to the nature and frequency of HLA class II genes, to evaluate its possible involvement in the development of diabetes. RESEARCH DESIGN AND METHODS: We interviewed 196 elderly Yemenite women, who had immigrated to Israel as adults, in programmed encounters about signs and symptoms of type 1 diabetes, infant feeding customs, and infectious diseases in Yemen. We also performed HLA oligotyping of DRB1, DQA1, and DQB1 genes in 120 unrelated Yemenite Jews, including 44 type 1 diabetic patients and 76 healthy control subjects, and used these data in correspondence analysis comparing Yemenites with different Israeli ethnic groups. RESULTS: Interviews indicated that early exposure to cow's milk was very common in Yemen. However, none of the women could recall classical presentations of diabetes. HLA oligotyping showed that gene frequencies of non-Asp-57 (of the HLA-DQB chain) in the patients (0.94) and control subjects (0.6) were similar to those of other populations with a known high incidence of type 1 diabetes. Correspondence analysis revealed that Yemenite Jews are genetically distinct from other ethnic groups in Israel. CONCLUSIONS: The genetic distinctiveness of Yemenite Jews may explain their unusually high incidence of type 1 diabetes in Israel. Despite the presence of highly susceptible diabetogenic HL4 class II genes in this community, early exposure to cow's milk did not cause phenotypic expression of diabetes in Yemen. This finding suggests that in this population, either cow's milk does not play a crucial role in triggering diabetes, or environmentally conferred protection, such as frequent infectious disease in Yemen, was dominant.

The development of the fetal penis--an in utero sonographic evaluation.

OBJECTIVE: To establish a nomogram for fetal penile length during gestation. DESIGN: A prospective, cross-sectional study of normal singleton pregnancies. SUBJECTS: Four hundred and nineteen male fetuses between 14 and 38 weeks were studied. METHODS: Measurements of fetal penis length were performed by high resolution transvaginal ultrasonography between 14 and 17 weeks of gestation, and by transabdominal ultrasonography beyond 18 weeks of gestation. RESULTS: Adequate penile length measurements were obtained in all 419 fetuses. Penile length as a function of gestational age was expressed by the regression equation: (square root) penile length (mm) = 0.277 + 0.121 x gestational age (weeks). The correlation coefficient, r = 0.967 was found to be highly statistically significant (P < 0.0001). The normal mean and the 90% prediction limits were defined. During the study period, we identified three fetuses with abnormalities involving penile development. Using the above reference data range, it has been shown that their penile length was below the lower limit. CONCLUSIONS: The present data provide a normal range of fetal penile length from early stages of gestation that may allow intrauterine assessment of the development of the male external genitalia.

Immunogenetics of HLA class II in Israeli Ashkenazi Jewish, Israeli non-Ashkenazi Jewish, and in Israeli Arab IDDM patients.

The distribution of HLA class II alleles and genotypes in IDDM patients was examined in the three main Israeli ethnic groups: Ashkenazi Jews, non-Ashkenazi Jews, and Arabs. Molecular sequence specific oligonucleotide probe analysis was performed for DRB1, DQA1, and DQB1 genes. The DRB1*03011, DQA1*05 DQB1*02/DRB1*0402, DQA1*03, DQB1*0302 genotype was found to be the main susceptibility genotype in all three groups, with differences in the degree of association. In addition to DRB1*0402 (more frequent among Ashkenazi Jews), DRB1*0405, another subtype of DRB1*04, was found to be more prevalent among non-Ashkenazi Jews and Arabs. Many alleles were found to be negatively associated with insulin dependent diabetes mellitus (IDDM). This could be a result of the high frequency of susceptible alleles, or of linkage disequilibrium to a primary negatively associated allele. The strongest negative association was observed for DQB1*0301 in all three ethnic groups. The alleles DRB1*1401, DRB1*1501, DQB1*05031, DQB1*0602, and DQB1*0609 were not detected in any of the 202 IDDM patients, and are probably either strongly protective or in linkage with such alleles. Despite the differences found between the three ethnic groups, an overall analysis shows that the DRB1*04 alleles that account for susceptibility to IDDM in the Israeli population (DRB1*0402 and *0405) are the same as those responsible for susceptibility to IDDM in a number of other Mediterranean populations. In contrast, the susceptible allele in most Caucasian populations is DRB1*0401. It is noteworthy that the susceptible alleles DRB1*0402/05 for Mediterranean and DRB1*0401 for Caucasian populations are also frequent in the respective healthy populations. These findings support the results obtained in other studies, which point to a genetic relationship between the Israeli and Mediterranean populations.