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There has been continuous progress in diabetes management over the last few decades, not least due to the widespread dissemination of continuous glucose monitoring (CGM) and automated insulin delivery systems. These technological advances have radically changed the daily lives of people living with diabetes, improving the quality of life of both children and their families. Despite this, hypoglycemia remains the primary side-effect of insulin therapy. Based on a systematic review of the available scientific evidence, this paper aims to provide evidence-based recommendations for recognizing, risk stratifying, treating, and managing patients with hypoglycemia. The objective of these recommendations is to unify the behavior of pediatric diabetologists with respect to the timely recognition and prevention of hypoglycemic episodes and the correct treatment of hypoglycemia, especially in patients using CGM or advanced hybrid closed-loop systems. All authors have long experience in the specialty and are members of the Italian Society of Pediatric Endocrinology and Diabetology. The goal of treating hypoglycemia is to raise blood glucose above 70 mg/dL (3.9 mmol/L) and to prevent further decreases. Oral glucose at a dose of 0.3 g/kg (0.1 g/kg for children using “smart pumps” or hybrid closed loop systems in automated mode) is the preferred treatment for the conscious individual with blood glucose

This strategy has been recently questioned by studies [[2]] reporting that ~ 20% MODY patients were indeed carriers of mutations in SD genes, thus suggesting to change our current strategy [[2]]. Among the latter group, probands with I WFS1 i mutations (two heterozygous for p.Arg558His and p.Trp700* aged 37 and 60 with diabetes diagnosed at 15 and 47 years, respectively, one compound heterozygous for p.Val415del and p.Gly702Ser 16 years old, with diabetes diagnosed at 13 years and one homozygous for p.Glu202Gly 56 years old, with diabetes diagnosed at 27 years) were treated with insulin (Table 2). The pipeline we used defines heteroplasmic variants with a sensitivity of 1-5% Notably, the p.Ala1458Thr I ABCC8 i mutation found in one of our patient has been described to associate both with hyperinsulinism [[4]] and MODY [[5]], an opposite counter-intuitive biological function that has been described also for other ABCC8 mutations. MODY patients carrying mutation in syndromic diabetes genes. [Extracted from the article]

Objective: Autonomic nervous system is involved in many disorders, and changes in its modulation are the known risk factors for cardiovascular diseases. Its role in metabolic disarrangements in children at high cardio-metabolic risk is not known. Aim of the study is to analyze the relation between clinical-metabolic parameters and autonomic nervous system in children. Materials and Methods: Children affected by type 1 diabetes (group 1), obesity (group 2), and control group (group 3) were enrolled. Autonomic nervous system functionality was assessed with dynamic tests (The Expiration to Inspiration indexes--E/I delta and ratio, 30:15 Ratio Test, Systolic blood pressure response to standing--deltaPA), and ultrasonography was performed to evaluate Intima Media Thickness (cIMT). Clinical parameters were recorded. Results: The study popultaion had a total of 75 children with mean age of 12.5 ± 2.8 years: 26 in group 1, 28 in group 2, 21 in group 3. Obese children had higher cIMT z-score (P < .001). Diabetic patients had lower EI delta values (P < .001) and 30:15 ratio test (P = .01). There was an inverse correlation between E/I delta and microalbuminuria levels (rho -0.955, P < .001) and 30:15 ratio test and microalbuminuria (rho -0.936, P < .001) in group 1, even after adjustment for age. DeltaPA was higher in obese (P = .032) and correlated with BMI z-score and homeostatic model assessment. Conclusion: Our results highlight imbalances in sutonomic nervous system function in children at high metabolic risk, in particular with involvement of parasympathetic function in type 1 Diabetes Mellitus and sympathetic one in obesity. An early screening could lead to a prompt identification of these alterations and could have a predictive role on cardio-metabolic risk. [ABSTRACT FROM AUTHOR]

The aim of this mini-review is to present the current knowledge on iodine requirements in developmental age, from conception to adolescence. It is based on the analysis of updated national and international guidelines on iodine intake and the prevention of iodine deficiency. Health policy initiatives carried out in industrialized countries in previous decades have led to a dramatic improvement in nutritional iodine status in the general population. However, the prevention of iodine deficit continues to be a concern, especially for vulnerable categories, like adolescents and pregnant women. [ABSTRACT FROM AUTHOR]

Purpose: The World Health Organization recommends reduction of salt intake to < 5 g/day and the use of iodized salt to prevent iodine deficiency states. A high prevalence of excess salt consumption and an inadequate iodine intake has been previously shown in an Italian pediatric population. It was appropriate, therefore, to analyse in the same population the relationship occurring between salt consumption and iodine intake. Methods: The study population was made of 1270 children and adolescents. Estimates of salt consumption and iodine intake were obtained by measuring 24 h urinary sodium and iodine excretion. Results: The iodine intake increased gradually across quartiles of salt consumption independently of sex, age and body weight (p < 0.001). Median iodine intake met the European Food Safety Authority adequacy level only in teenagers in the highest quartile of salt consumption (salt intake > 10.2 g/day). We estimated that approximately 65–73% of the total iodine intake was derived from food and 27–35% from iodized salt and that iodized salt made actually only 20% of the total salt intake. Conclusion: In this pediatric population, in face of an elevated average salt consumption, the use of iodized salt was still insufficient to ensure an adequate iodine intake, in particular among teenagers. In the perspective of a progressive reduction of total salt intake, the health institutions should continue to support iodoprophylaxis, in the context of the national strategies for salt reduction. In order for these policies to be successful, in addition to educational campaigns, it is needed that the prescriptions contained in the current legislation on iodoprophylaxis are made compelling through specific enforcement measures for all the involved stakeholders. [ABSTRACT FROM AUTHOR]

Objectives: In this commentary we emphasize the importance of breakfast for children, both from the point of view of proper nutritional education and as a strategy for the prevention of obesity. Methods: We reviewed the international literature, drawing particularly on information regarding the possible negative effects of skipping breakfast. Results: Obese children often do not eat breakfast and may face metabolic problems such as insulin resistance and hypercholesterolemia. The child who does not eat breakfast often becomes an adult who does not eat breakfast. The role of parents is fundamental in the acquisition of correct eating habits. Conclusions: The awareness that habits during childhood become elements of everyday life in adulthood, makes us understand the importance of the long-term consequences of skipping breakfast during childhood and adolescence. [ABSTRACT FROM AUTHOR]

Objectives Prader-Willi syndrome (PWS) significantly impacts health-related quality of life; however, its relational and existential aspects remain unknown in Italian clinical and social debate. The project aimed to investigate the impact of PWS on illness experience through narrative medicine (NM) to understand the daily life, needs and resources of patients with PWS and their caregivers, and to furnish insights for clinical practice. Design and setting The project involved 10 medical centres of the Italian Network for Rare Diseases and PWS family associations and targeted underage and adult patients with PWS and their caregivers. Written interviews, composed by a sociodemographic survey and a narrative, were collected through the project’s website. Three dedicated illness plots employed evocative and open words to facilitate individual expression and to encourage reflection. Narratives were analysed through NVivo software. Researchers discussed the results with the project’s steering committee. Participants Twenty-one children and adolescents and 34 adults with PWS joined the project, as well as 138 caregivers. A PWS diagnosis or the caregiving of a patient with PWS older than 5 years represented the eligibility criteria, as well as the willingness to share their illness experience by writing and the ability to communicate in Italian. Results The analysis of narratives led to understanding the PWS social and relational issues concerning diagnosis and current management, PWS daily experiences and social contexts, PWS implications in the working sphere and participants’ future perspectives. Narratives demonstrated that PWS management affects relationships and work-life balance and that social stigma remains present. Conclusion The project represented the first effort to investigate the impact of PWS on illness experience in Italy through NM while considering the perspectives of patients with PWS and their caregivers. The findings indicated that a multi professional approach is fundamental to ensure adequate treatment and provided elements for its improvement. [ABSTRACT FROM AUTHOR]

This is a retrospective multicenter nationwide Italian study collecting neonatal anthropometric data of Caucasian subjects with Prader–Willi syndrome (PWS) born from 1988 to 2018. The aim of the study is to provide percentile charts for weight and length of singletons with PWS born between 36 and 42 gestational weeks. We collected the birth weight and birth length of 252 male and 244 female singleton live born infants with both parents of Italian origin and PWS genetically confirmed. Percentile smoothed curves of birth weight and length for gestational age were built through Cole's lambda, mu, sigma method. The data were compared to normal Italian standards. Newborns with PWS showed a lower mean birth weight, by 1/2 kg, and a shorter mean birth length, by 1 cm, than healthy neonates. Females with a 15q11‐13 deletion were shorter than those with maternal uniparental maternal disomy of chromosome 15 (p < .0001). The present growth curves may be useful as further traits in supporting a suspicion of PWS in a newborn. Because impaired prenatal growth increases risk of health problems later in life, having neonatal anthropometric standards could be helpful to evaluate possible correlations between the presence or absence of small gestational age and some clinical and metabolic aspects of PWS. [ABSTRACT FROM AUTHOR]

Aims: To evaluate physicians' adjustments of insulin pump settings based on continuous glucose monitoring (CGM) for patients with type 1 diabetes and to compare these to automated insulin dose adjustments. Methods: A total of 26 physicians from 16 centres in Europe, Israel and South America participated in the study. All were asked to adjust insulin dosing based on insulin pump, CGM and glucometer downloads of 15 patients (mean age 16.2 ± 4.3 years, six female, mean glycated haemoglobin 8.3 ± 0.9% [66.8 ± 7.3 mmol/mol]) gathered over a 3‐week period. Recommendations were compared for the relative changes in the basal, carbohydrate to insulin ratio (CR) and correction factor (CF) plans among physicians and among centres and also between the physicians and an automated algorithm, the Advisor Pro (DreaMed Diabetes Ltd, Petah Tikva, Israel). Study endpoints were the percentage of comparison points for which there was full agreement on the trend of insulin dose adjustments (same trend), partial agreement (increase/decrease vs no change) and full disagreement (opposite trend). Results: The percentages for full agreement between physicians on the trend of insulin adjustments of the basal, CR and CF plans were 41 ± 9%, 45 ± 11% and 45.5 ± 13%, and for complete disagreement they were 12 ± 7%, 9.5 ± 7% and 10 ± 8%, respectively. Significantly similar results were found between the physicians and the automated algorithm. The algorithm magnitude of insulin dose change was at least equal to or less than that proposed by the physicians. Conclusions: Physicians provide different insulin dose recommendations based on the same datasets. The automated advice of the Advisor Pro did not differ significantly from the advice given by the physicians in the direction or magnitude of the insulin dosing. [ABSTRACT FROM AUTHOR]

Aim: No data are available on caffeine consumption among Italian adolescents. We investigated caffeine intake from coffee, soft drinks and energy drinks in a sample of Italian adolescents and determined if they exceeded the recommended limits.Methods: The study comprised 1213 adolescents with a mean age of 15.1 years (range 12-19) from four schools in Foggia, southern Italy. Caffeine intake was assessed using an anonymous self-reported questionnaire during the 2013/2014 school year. We calculated the percentage of daily caffeine consumers, their mean intake of caffeine from beverages and the contribution of each beverage category to the total caffeine intake.Results: Approximately 76% of the sample consumed caffeine every day, amounting to 125.5 ± 69.2 mg/day and 2.1 ± 1.2 mg/kg/day. When we applied the reference values from the Academy of Pediatrics, we found that 46% of the adolescents exceeded the recommended upper limits. Coffee was the most frequently consumed caffeinated drink and the main contributor to daily caffeine intake.Conclusion: More than three quarters (76%) of the Italian adolescents in our study drank coffee on a daily basis and nearly half (46%) exceeded the recommended upper limits. Strategies are needed to reduce caffeine consumption by adolescents. [ABSTRACT FROM AUTHOR]

A. Acquafredda (Cerignola), Dr. N. Aricò (Reggio Calabria, I), Dr. N. Attafi (Cassago Brianza, I), Dr. M. G. Baroni (L'Aquila, I), Dr. L. Belli (Isernia, I), Dr. R. M. Benagiano (Massafra, I), Dr. O. Bitterman (Roma, I), Prof. R. Buzzetti (Roma, I), Dr. M. Campo (Foggia, I), Dr. G. Cardinale (Casarano, I), Dr. A. Giaccari (Roma, I), Dr. G. Grassi (Torino, I), Dr. S. Guercio Nuzio (Salerno, I), Prof. D. Iafusco (Napoli, I), Dr. D. Ingletto (Tricase, I), Prof. O. Lamacchia (Foggia, I), Prof. F. Lombardo (Messina, I), Dr. A. Corsi (Bergamo, I), Dr. F. Costantino (Roma, I), Dr. V. Delmonte (Taranto, I), Dr. M. Delvecchio (Bari, I), Dr. N. Di Biase (Roma, I), Dr. M. F. Faienza (Bari, I), Prof. [Extracted from the article]

Actual and perceived physical abilities are important correlates of physical activity (PA) and fitness, but little research has explored these relationships over time in obese children. This study was designed: (a) to assess the feasibility of a multi-modal training programme promoting changes in PA, fundamental motor skills and real and perceived physical abilities of obese children; and (b) to explore cross-sectional and longitudinal relationships between real and perceived physical competence in boys and girls. Forty-one participants (9.2 ± 1.2 years) were assessed before and after an 8-month intervention with respect to body composition, physical fitness, self-reported PA and perceived physical ability. After treatment, obese children reported improvements in the body mass index, PA levels, gross motor performance and actual and perceived physical abilities. Real and perceived physical competence was correlated in boys, but not in girls. Results indicate that a multi-modal programme focused on actual and perceived physical competence as associated with the gradual increase in the volume of activity might be an effective strategy to improve adherence of the participants and to increase the lifelong exercise skills of obese children. [ABSTRACT FROM AUTHOR]

Objectives: (1) To examine relationships among changes in physical activity, physical fitness and some psychosocial determinants of activity behavior in a clinical sample of obese children involved in a multi-component program; (2) to investigate the causal relationship over time between physical activity and one of its strongest correlates (i.e. perceived physical ability). Methods: Self-reported physical activity and health-related fitness tests were administered before and after a 9-month intervention in 24 boys and 20 girls aged 8 to 11 years. Individuals' perceptions of strength, speed and agility were assessed using the Perceived Physical Ability Scale, while body image was measured using Collins' Child Figure Drawings. Results: Findings showed that body mass index, physical activity, performances on throwing and weight-bearing tasks, perceived physical ability and body image significantly improved after treatment among obese children. Gender differences were found in the correlational analyses, showing a link between actual and perceived physical abilities in boys, but not in girls. For the specific measurement interval of this study, perception of physical ability was an antecedent and not a potential consequence of physical activity. Conclusions: Results indicate that a multi-component activity program not based merely on a dose-effect approach enhances adherence of the participants and has the potential to increase the lifelong exercise skills of obese children. Rather than focusing entirely on diet and weight loss, findings support the inclusion of interventions directed toward improving perceived physical ability that is predictive of subsequent physical activity. [ABSTRACT FROM AUTHOR]

Monogenic diabetes is a genetic disorder caused by one or more variations in a single gene. It encompasses a broad spectrum of heterogeneous conditions, including neonatal diabetes, maturity onset diabetes of the young (MODY) and syndromic diabetes, affecting 1–5% of patients with diabetes. Some of these variants are harbored by genes whose altered function can be tackled by specific actions ("actionable genes"). In suspected patients, molecular diagnosis allows the implementation of effective approaches of precision medicine so as to allow individual interventions aimed to prevent, mitigate or delay clinical outcomes. This review will almost exclusively concentrate on the clinical strategy that can be specifically pursued in carriers of mutations in "actionable genes", including ABCC8, KCNJ11, GCK, HNF1A, HNF4A, HNF1B, PPARG, GATA4 and GATA6. For each of them we will provide a short background on what is known about gene function and dysfunction. Then, we will discuss how the identification of their mutations in individuals with this form of diabetes, can be used in daily clinical practice to implement specific monitoring and treatments. We hope this article will help clinical diabetologists carefully consider who of their patients deserves timely genetic testing for monogenic diabetes. [ABSTRACT FROM AUTHOR]

Prader–Willi syndrome (PWS) is a rare disease determined by the loss of the paternal copy of the 15q11-q13 region, and it is characterized by hypotonia, hyperphagia, obesity, short stature, hypogonadism, craniofacial dysmorphisms, and cognitive and behavioral disturbances. The aims of this retrospective study were to analyze interictal EEG findings in a group of PWS patients and to correlate them with genetic, clinical, and neuroimaging data. The demographic, clinical, genetic, EEG, and neuroimaging data of seventy-four patients were collected. Associations among the presence of paroxysmal EEG abnormalities, genotype, and clinical and neuroimaging features were investigated. Four patients (5.4%) presented drug-sensitive epilepsy. Interictal paroxysmal EEG abnormalities—focal or multifocal—were present in 25.7% of the cases, and the normalization of the EEG occurred in about 25% of the cases. In 63.2% of the cases, the paroxysmal abnormalities were bilaterally localized over the middle–posterior regions. Brain magnetic resonance imaging (MRI) was performed on 39 patients (abnormal in 59%). No relevant associations were found between paroxysmal EEG abnormalities and all of the other variables considered. Interictal paroxysmal EEG abnormalities—in particular, with a bilateral middle–posterior localization—could represent an important neurological feature of PWS that is not associated with genotype, cognitive or behavioral endophenotypes, MRI anomalies, or prognosis. [ABSTRACT FROM AUTHOR]

The prevalence of primary hypertension in pediatric patients is increasing, especially as a result of the increased prevalence of obesity in children. New diagnostic guidelines for blood pressure were published by the American Academy of Pediatrics (AAP) in 2017 to better define classes of hypertension in children. The aim of our study is to evaluate the impact of new guidelines on diagnosis of hypertension in pediatrics and their capacity to identify the presence of cardiovascular and metabolic risk. Methods: Retrospective clinical and laboratory data from 489 overweight and obese children and adolescents were reviewed. Children were classified according to the 2004 and 2017 AAP guidelines for systolic and diastolic blood pressure. Lipid profile and glucose metabolism data were recorded; triglyceride/HDL ratio (TG/HDL) was calculated as an index of endothelial dysfunction. Hepatic steatosis was detected using the ultrasonographic steatosis score. Results: Children with elevated blood pressure increased from 12.5% with the 2004 AAP to 23.1% with the 2017 AAP criteria (p < 0.001). There was a statistically significant increase in children with high blood pressure in all age groups according to the new cut-off values. Notably, the diagnosis of hypertension according to 2017 AAP criteria had a greater positive association with Hepatic Steatosis (rho 0.2, p < 0.001) and TG/HDL ratio (rho 0.125, p = 0.025). Conclusions: The 2017 AAP tables offer the opportunity to better identify overweight and obese children at risk for organ damage, allowing an earlier and more impactful prevention strategy to be designed. [ABSTRACT FROM AUTHOR]

The obesity epidemic and the decline of fitness among children highlights the need for suitable interventions designed to promote Physical Activity (PA) and healthy habits. The purpose of our pilot study was to assess the feasibility of a school-based program among overweight and obese children, by examining changes in their body composition, PA, physical fitness, and some psychosocial determinants of active behavior. An additional objective was to investigate the reciprocal relationship over time between PA and body image. Self-reported PA and health-related fitness tests were administered to 18 overweight and obese children (11.3 ± 0.4 years), before and after a 6-month intervention including nutritional education, skill-learning, playful physical activities, and exercise training. Participants were assessed on body composition, perceived physical ability, body image, and pleasant and unpleasant psychobiosocial states towards PA. After treatment, children showed decreased body mass index, body fat percentage, arm and waist circumferences, and skinfold thickness. Actual and perceived physical abilities, body image, and PA also improved over six months. Cross-lagged correlations suggest body image to be an antecedent to being physically active. Results showed that the multi-component school program may have sustainable benefits in reducing adiposity indicators and improving exercise adherence, physical fitness, and psychological well-being. [ABSTRACT FROM AUTHOR]