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Background. Easy accessibility of psychosocial care is recommended for children and adolescents with type 1 diabetes (T1D) and their families. Objective. The study aimed to evaluate the availability of psychological care and its associations with glycemic control in centers from the multinational SWEET (Better control in Pediatric and Adolescent diabeteS: Working to crEate CEnTers of Reference) registry. Subjects. Centers participating in SWEET (n = 112) were invited to complete a structured online survey, designed for the study, regarding their psychology service. Methods. Linear/logistic regression models adjusted for several confounders were used to determine the patient’s HbA1c (mmol/mol) and odds ratios (ORs) for diabetic ketoacidosis (DKA) and severe hypoglycemia (SH) related to survey responses. Results. 76 (68%) centers with relevant data in the SWEET database responded to the survey. Psychological services were provided in 89% of the centers. The availability of psychological service in centers was associated with a slightly lower HbA1c of the patients (72 (62–82) vs. 67 (57–78) mmol/mol, p=0.004) and significantly lower odds for DKA (1.8 (1.1–2.9), p=0.027). Conclusions. Most centers from the SWEET registry offered some form of structured psychological care, consistent with the recommendations of easy access to psychosocial care for children and adolescents with T1D and their families. The main benefit of this psychological care appears to be in the incidence of DKA between centers. The study data also continues to emphasize the importance of treatment targets in shaping the outcomes of pediatric diabetes care. These findings should inform health-service planners and the diabetes community of the importance of mental healthcare in multidisciplinary diabetes teams.

Context: One of the most common endocrine disorders in children is diabetes which is the leading cause of premature cardiovascular disease in adulthood. Aims: This study is aimed to investigate the extend of cardiac involvement in diabetic children by speckle tracking echocardiography (STE) in comparison to two-dimensional (2D) echocardiography and routine laboratory data. Settings and Design: A cross-sectional study conducted on patients under 18 years of age who deal with type one diabetes mellitus for more than 5 years. Subjects and Methods: To compare the STE results, we included the STE data of 25 normal age-matched children. All patients underwent laboratory analysis for lipid profile, blood sugar, and 2D echocardiography plus STE. Statistical Analysis Used: Two-sample independent t-test, Chi-square test, logistic regression test, Spearman's rank correlation coefficient, and the Pearson correlation coefficient. Results: From March 2018 to 2019, we included 53 patients, mean age 15.8 ± 0.39 years and 52.8% female, and 25 nondiabetic control in this study. STE revealed global longitudinal strain (LS) −18.4 versus − 24.2 for patient (44 valid cases) versus control group, respectively, with significant statistical difference. Diabetic patients had lower LS in all segments compared to the control group. Conclusions: STE has very high sensitivity to detect cardiac involvement far earlier than 2D echocardiography. None of the routine biomarkers or demographic features can predict cardiac involvement based on segmental abnormalities of STE. Active investigation to clear the remote impact of STE abnormalities and its practical role in childhood diabetes management is highly recommended.

We would like to thank the Metabolic ERN (MetabERN) for their support to our study. Phosphomannomutase 2 (PMM2-CDG) is the most common congenital disorder of N-glycosylation and is caused by a deficient PMM2 activity. The clinical presentation and the onset of PMM2-CDG vary among affected individuals ranging from a severe antenatal presentation with multisystem involvement to mild adulthood presentation limited to minor neurological involvement. Management of affected patients requires a multidisciplinary approach. In this article, a systematic review of the literature on PMM2-CDG was conducted by a group of international experts in different aspects of CDG. Our managment guidelines were initiated based on the available evidence-based data and experts' opinions. This guideline mainly addresses the clinical evaluation of each system/organ involved in PMM2-CDG, and the recommended management approach. It is the first systematic review of current practices in PMM2-CDG and the first guidelines aiming at establishing a practical approach to the recognition, diagnosis and management of PMM2-CDG patients. publishersversion published

Background: Although selenium is one of the nutrients that has an important role in the metabolism of thyroid hormones, it has been investigated in rare studies. This study aimed to evaluate role of selenium deficiency in children and adolescents with acquired hypothyroidism. Methods: This case and control study was conducted on 60 acquired hypothyroidism and 60 healthy children who had been referred to the pediatrics endocrine clinic in Shiraz, Iran, from November 2018 to April 2019. Some information such as age, gender, weight, height, duration of disease, and level of plasma selenium were recorded. Plasma selenium level was measured by atomic absorption spectrophotometer. Data were analyzed using SPSS software 21.0. Results: The mean of selenium level in the case and control groups were 93.77 ± 24.90 μg/dl and 85.96 ± 25.20 μg/dl, respectively. There was no significant difference between the two groups in the mean levels of selenium. Independent t-test showed no significant difference in the mean levels of selenium in male and female samples in the case group, but this difference was significant in the control group. Conclusion: Selenium deficiency may not have significant role as a cause of acquired hypothyroidism in pediatric and adolescent age group, in south of Iran. Thus, it does not seem necessary to routinely check the level of selenium for patients with thyroid dysfunction.

Introduction and aim: The aim of this study was to evaluate nutritional status in children without prior hospital admission or evidence of chronic disease. Subjects and methods: The current study is a cross-sectional and observational study which was conducted for assessing the nutritional status of children. In this study, consecutive sampling was used, with a sample size about 400 children aged 6 months to 18 years at first hospital admission. All subjects were hospitalized consecutively in the Pediatric Emergency Department of the Nemazee Teaching Hospital of Shiraz University of Medical Sciences (Shiraz, Islamic Republic of Iran). All children with evidence or history of chronic diseases such as liver cirrhosis, microcephaly, macrocephaly due to congenital infection or abnormal skull shape, patients with muscular or neurological problem, with prolonged steroid usage, nephrotic syndrome, cardiac problem, and protein loosing enteropathy were excluded from the study. The duration of the study was six months, starting from January 2016. Results: In the current study, 430 (225 boys and 175 girls) cases were included. Mean age was 5.39 (SEM 0.23) for boys and 5.78 (SEM 0.38) for girls. According to H/A criteria (current height/ideal height), 194 (48.5%) patients were stunted, 75 (18.8%) were mild, 49 (12.3%) were moderate and 70 (17.5%) were severe. According to W/A data (current weight/ideal weight), 188 (47%) of the patients studied were malnourished. Of these cases, 118 (29.5%) had mild malnutrition; 54 (13.5%), moderate; and 16 (4%), severe. According to body mass index (BMI), 40 (10%) patients were overweight, four (16%) were obese, and four (1%) were severe obese. In relation to middle upper arm circumference (MUAC), 56 (14%) patients were malnourished, 33 (8.3%) were at risk, 14 (3.5%) presented moderate malnutrition, and 9 (2.3%), severe malnutrition. Conclusion: Prevalence of malnutrition among children without prior admission was 48.5% (according to H/A). According to W/A, 47% of children had different degrees of malnutrition. Resumen Introducción y objetivo: el propósito de este estudio fue evaluar el estado nutricional de los niños ingresados por primera vez en el hospital y sin historia de enfermedad crónica. Pacientes y métodos: se trata de un estudio transversal, observacional para valorar el estado nutricional al ingreso. La muestra estuvo constituida por algo más de 400 niños, de entre 6 meses y 18 años, ingresados por primera vez en el hospital. Se trataba de pacientes ingresados de forma consecutiva desde el servicio de Urgencias del hospital Nemazee de la Universidad Shiraz, en la República Islámica de Irán. Se excluyeron los pacientes con historia de enfermedad crónica como cirrosis hepática, microcefalia, macrocefalia por infección congénita o forma craneal anómala, pacientes con enfermedades musculares o neurológicas, con uso prologado de corticoides, síndrome nefrótico, cardiopatía o enteropatía pierde-proteínas. La duración del estudio fuere de seis meses, a partir de enero de 2016. Resultados: se incluyeron 430 niños (225 varones y 175 niñas). La edad media fue de 5,39 años (SEM 0,23) para los varones y de 5,78 (SEM 0,38) en las niñas. De acuerdo al peso para la talla ideal, 194 (48,5%) tenían desnutrición crónica, 75 (18,8%) leve, 49 (12,3%) moderada y en 70 (17,5%) era grave. Según el porcentaje del peso ideal, 188 pacientes (47%) presentaban desnutrición aguda, de los cuales en 118 (29,5%) era leva, en 54 (13,5%) moderada y en 16 (4%), grave. Según el índice de masa corporal (IMC), 40 (10%) tenían sobrepeso, 16 (4%) eran obesos y cuatro (1%) presentaban obesidad mórbida. Según el perímetro del brazo (MUAC), 56 (14%) tenían desnutrición, 33 (8,3%) estaban en riesgo de desnutrición, 14 (3,5%) presentaban desnutrición moderada y 9 (2,3%) intensa. Conclusión: la prevalencia de desnutrición en los niños ingresado por vez primera fue del 48,5%. De acuerdo al peso para la edad en 47% de los niños había algún grado de desnutrición.