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The expansion of newborn screening (NBS) for congenital hypothyroidism (CH) is essential to reducing the number of preventable intellectual disabilities in children. Because of logistical issues, including geographic extremes, distinct cultures, and 4.8 million births annually, Indonesia has struggled to achieve universal NBS coverage. A national cross-sectional electronic survey was conducted to explore challenges in CH NBS. Responses from 423 healthcare professionals and program administrators across 30 provinces in Indonesia were collected. The major challenges reported were refusal from families (39.2%), newborns being discharged <24 h (38.3%), and limited availability of filter paper (35.9%). The respondents considered refusal from families to be due to fear, while others did not understand the necessity of CH NBS. The vast majority of respondents believed that parents do not have sufficient understanding regarding CH NBS (96.5%). Our study found that only 38.5% of respondents had received formal CH NBS training, with pediatric endocrinologists being the only profession in which all respondents had been trained. Concerted efforts are needed to improve the access to and availability of resources, increase the capacity for sample collection and analysis, empower healthcare professionals, and develop educational resources to promote understanding and acceptance of NBS amongst families.

Competing Interests: All authors report no contributions received for the writing of this Comment. NJG reports an unrelated grant from the Commonwealth Fund of New York City. She has current or recent leadership roles in the International Association for Adolescent Health (supported by a grant from the AstraZeneca Foundation), the UK Association for Young People's Health, and NCD Child (supported through grants to the Centre for Global Child Health at The Hospital for Sick Children from the AstraZeneca Young Health Programme and Friends of Cancer Patients). The Hospital for Sick Children, Toronto, also provides in-kind contributions to support the NCD Child Secretariat. NJG also reports a family member employed by EMIS Health in the UK. She is the co-chair of the UNESCO Chair Global Health and Education, which is supported by non-profit organisations MGEN and Groupe Vyv. University Clermont-Auvergne and the University of Huddersfield provide in-kind contributions to support the Chair secretariat. CK reports an unrelated grant from the World Diabetes Association, consultancy fees or other payments from Novo Nordisk Foundation and Roche Diabetes Care, and current or recent leadership roles in East Africa NCD Alliance and the NCD Child Task Force on Essential Medicines & Technologies. All other authors declare no competing interests.

Objectives: Unrecognized hypoglycemia, especially in the neonatal population, is a significant cause of morbidity and poor neurologic outcomes. Children with congenital hyperinsulinism (HI) are at risk of hypoglycemia and point of care testing (POCT) is the standard of care. Studies have shown that continuous glucose monitoring (CGM) improves glycemic control and reduces the frequency of hypoglycemia among children with type 1 diabetes. There is limited experience with the use of CGM in children with HI. To assess the glycemic pattern of children with HI on stable therapy and evaluate the frequency of undetected hypoglycemia using Dexcom G6 ® CGM., Methods: A cross-sectional, observational pilot study was done in 10 children, ages 3 months to 17 years. Each child had a clinical or genetic diagnosis of HI on stable medical therapy. Participants were asked to continue their usual POCT blood glucose monitoring, as well as wear a blinded Dexcom G6 ® CGM during a 20-day study period with the potential of unblinding if there was severe hypoglycemia detected during the study trial., Results: During the study period, 26 hypoglycemic events were noted by CGM in 60 % of the participants with 45 % occurring between 0600 and 0800., Conclusions: CGM can help detect hypoglycemia and blood glucose trends during a time when there is usually no POCT, which can guide medical management. 30 % of our population had a dose adjustment in their medications. This study was limited by population size., (© 2023 the author(s), published by De Gruyter, Berlin/Boston.)

Context: Prader-Willi syndrome (PWS) is a rare genetic disorder characterized by endocrine and neuropsychiatric problems including hyperphagia, anxiousness, and distress. Intranasal carbetocin, an oxytocin analog, was investigated as a selective oxytocin replacement therapy., Objective: To evaluate safety and efficacy of intranasal carbetocin in PWS., Design: Randomized, double-blind, placebo-controlled phase 3 trial with long-term follow-up., Setting: Twenty-four ambulatory clinics at academic medical centers., Participants: A total of 130 participants with PWS aged 7 to 18 years., Interventions: Participants were randomized to 9.6 mg/dose carbetocin, 3.2 mg/dose carbetocin, or placebo 3 times daily during an 8-week placebo-controlled period (PCP). During a subsequent 56-week long-term follow-up period, placebo participants were randomly assigned to 9.6 mg or 3.2 mg carbetocin, with carbetocin participants continuing at their previous dose., Main Outcome Measures: Primary endpoints assessed change in hyperphagia (Hyperphagia Questionnaire for Clinical Trials [HQ-CT]) and obsessive-compulsive symptoms (Children's Yale-Brown Obsessive-Compulsive Scale [CY-BOCS]) during the PCP for 9.6 mg vs placebo, and the first secondary endpoints assessed these same outcomes for 3.2 mg vs placebo. Additional secondary endpoints included assessments of anxiousness and distress behaviors (PWS Anxiousness and Distress Behaviors Questionnaire [PADQ]) and clinical global impression of change (CGI-C)., Results: Because of onset of the COVID-19 pandemic, enrollment was stopped prematurely. The primary endpoints showed numeric improvements in both HQ-CT and CY-BOCS which were not statistically significant; however, the 3.2-mg arm showed nominally significant improvements in HQ-CT, PADQ, and CGI-C scores vs placebo. Improvements were sustained in the long-term follow-up period. The most common adverse event during the PCP was mild to moderate flushing., Conclusions: Carbetocin was well tolerated, and the 3.2-mg dose was associated with clinically meaningful improvements in hyperphagia and anxiousness and distress behaviors in participants with PWS., Clinical Trials Registration Number: NCT03649477., (© The Author(s) 2023. Published by Oxford University Press on behalf of the Endocrine Society.)

Background: Pediatric endocrinology is a specialty that is struggling worldwide to maintain adequately trained professionals. Pediatric endocrine care in Central America and Caribbean countries is often performed by pediatricians or adult endocrinologists due to the limited number of pediatric endocrinologists. These health care providers are seldom members of endocrine societies and frequently lack formal training in the field., Objective: In this study, we describe the scope of a virtual conference in pediatric endocrinology and diabetes targeted to low- and middle-income countries to provide equal opportunities for access to medical education for health care professionals., Methods: The virtual conference was sponsored by the Pediatric Endocrine Society (North America), Asociación Costarricense de Endocrinología (previously, Asociación Nacional Pro Estudio de la Diabetes, Endocrinología y Metabolismo), and Asociacion Centroamericana y del Caribe de Endocrinologia Pediátrica. The conference was free to participants and comprised 23 sessions that were either synchronous with ability for real-time interactive sessions or asynchronous sessions, where content was available online to access at their convenience. Topics included idiopathic short stature, polycystic ovarian syndrome, diabetes mellitus, telemedicine, Turner syndrome, congenital adrenal hyperplasia, obesity, central precocious puberty, and subclinical hypothyroidism. The participants were asked to evaluate the conference after its completion with a questionnaire., Results: A total of 8 speakers from Spain, Canada, Costa Rica, and the United States delivered the virtual event to 668 health care professionals from Guatemala, Venezuela, Dominican Republic, Costa Rica, Ecuador, Peru, Uruguay, Mexico, Honduras, Argentina, the United States, Bolivia, Chile, Panama, El Salvador, Nicaragua, Paraguay, Belize, Spain, and Colombia. Name, profession, and country were fully disclosed by 410 (61.4%) of the 668 health care professionals. The profession or level of training of participants were as follows: pediatric endocrinologists (n=129, 19.3%), pediatricians (n=116, 17.4%), general practitioners (n=77, 11.5%), adult endocrinologists (n=34, 5.1%), medical students (n=23, 3.4%), residents in various specialties (n=14, 2.1%), and others (n=17, 2.6%). A total of 23 sessions were offered, most of which were bilingual (Spanish and English). Feedback from the evaluation questionnaire indicated that the content of the conference was very relevant to the participants' professional practice. Additionally, the participants reported that they were very satisfied with the organization, the web-based platform, and the sessions of the conference., Conclusions: Lack of accessibility to the latest and cutting-edge medical education in pediatric endocrinology and diabetes for medical professionals from low- and middle-income countries can be overcome with a virtual conference. Online availability, low cost, and easy-to-use technology were well received from the participants, who were overall very satisfied by the quality and the relevance of the sessions to their professional practice., (©Roberto Bogarin, Luis Elizondo, Evangelia Kalaitzoglou, Jadranka Popovic, Alan Rogol, Erick Richmond, Jean-Pierre Chanoine, Jose M Lopez-Pedrosa, Francis Ruiz Salazar, Patricia Vuguin. Originally published in the Interactive Journal of Medical Research (https://www.i-jmr.org/), 08.05.2023.)

Background: The co-presentation of severe obesity (SO) and global developmental delay (GDD) in Canadian preschool children has not been examined. However, SO and GDD may require syndromic diagnoses and unique management considerations., Objectives: To determine (1) minimum incidence; (2) age of onset and risk factors; and (3) health care utilization for co-presenting SO and GDD., Methods: Through the Canadian Paediatric Surveillance Program (CPSP), a monthly form was distributed to participants from February 2018 to January 2020 asking for reports of new cases of SO and GDD among children ≤5 years of age. We performed descriptive statistics for quantitative questions and qualitative content analysis for open-ended questions., Results: Forty-seven cases (64% male; 51% white; mean age: 3.5 ± 1.2 years) were included. Age of first weight concern was 2.5 ± 1.3 years and age of GDD diagnosis was 2.7 ± 1.4 years. Minimum incidence of SO and GDD was 3.3 cases per 100,000 for ≤5 years of age per year. Identified problems included school and/or behavioural problems (n = 17; 36%), snoring (n = 14; 30%), and asthma/recurrent wheeze (n = 10; 21%). Mothers of 32% of cases (n = 15) had obesity and 21% of cases (n = 10) received neonatal intensive care. Microarray was ordered for 57% (n = 27) of children. A variety of clinicians and services were accessed. As reported by CPSP participants, challenges faced by families and health service access were barriers to care., Conclusion: Children with SO and GDD have multiple comorbidities, and require early identification and referral to appropriate services. These cases may also benefit from additional testing to rule out known genetic obesity syndromes., Competing Interests: WTG received research funding from the Canadian Institutes of Health Reearch. AMH is a member of the Clinical Advisory Board, Setmelanotide for BBS/Alstrom’s disease, Rhythm Pharmaceuticals; Chair, Scientific Advisory Board, PWS USA and PI for clinical trials with Rhythm pharmaceuticals and Levo Therapeutics. KMM has served as a member of an advisory board for Novo Nordisk and is on a Data Safety Monitoring committee for Novartis. She has received research funding from the Canadian Institutes of Health Research and McMaster University. JH has consulted for Novo Nordisk and the Pediatric Obesity National Advisory Board. She has received funding from Mead Johnson, Levo Therapeutics, and the Foundation for Prader-Willi Research. MH holds a Fonds de la recherche du Québec – Santé (FRQS) Junior 2 Clinical Research Scholar Award and is the recipient of the 2019 Canadian Society for Endocrinology and Metabolism Young Investigator Award. She is member of Canada’s Children’s Hospitals Foundation (CCHF) and Sun Life Child and Youth Type 2 Diabetes Prevention Initiative. She has received research funding from the Canadian Institutes of Health Research, the Heart and Stroke Foundation of Canada, Diabète Québec, the Fonds de Recherche du Québec – Santé, the Quebec Cardiometabolic Health, Diabetes and Obesity Network and the Quebec Network on Nursing Intervention Research, as well as through the Canada’s Children’s Hospitals Foundation (CCHF)/Sun Life initiative. GDCB served as a member of a national advisory board with Novo Nordisk Canada. He received research funding from the Canadian Institutes of Health Research, Public Health Agency of Canada, Alberta Health Services, Alberta Innovates, and the Women and Children’s Health Research Institute (UAlberta). All authors have submitted the ICMJE Form for Disclosure of Potential Conflicts of Interest. Conflicts that the editors consider relevant to the content of the manuscript have been disclosed., (© The Author(s) 2022. Published by Oxford University Press on behalf of the Canadian Paediatric Society. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Objectives: To: (1) complete an integrative literature review of transition studies that focus on individuals with intersex traits; (2) conduct an environmental scan of the current resources (practice guidelines, policies and procedures) used by healthcare providers working with Canadians with intersex traits; (3) investigate the experiences of Canadians with intersex traits in their healthcare transitions across the lifespan and (4) assess the understanding of healthcare providers about these transitions., Design: A qualitative prospective community participation study was conducted. It used mixed methods including an environmental scan and semistructured engagement sessions., Setting: The environmental scan examined resources available throughout Canada. The engagement sessions took place in British Columbia and Ontario, Canada., Participants: Sixteen participants were recruited. These included 13 individuals with intersex traits (a heterogeneous group of congenital conditions affecting the development of sex characteristics) and three caregivers., Methods: Mixed methods included an integrated literature review, environmental scan and qualitative approaches developed in collaboration with community partners., Results: The literature review identified gaps in transition care for individuals with intersex traits. The environmental scan uncovered no specific resources used by healthcare providers working with patients with intersex traits, though several general guidelines were used. Engaging providers in the study was problematic. Thematic analysis generated three main themes that emerged from the engagement sessions: (1) transition is a lifespan activity; (2) building personal agency is valuable and (3) well-being promotion is an application of health literacy., Conclusions: Transition resources for individuals living in Canada with intersex are scarce. Transitions happen across the lifespan with ownership of thought and actions seen as acts of personal agency. Health literacy skills and knowledge change with increased age, yet the primary source of knowledge often remained important in the individual's autobiographical self., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2022. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Our study purpose was to determine the prevalence of metabolically healthy obesity (MHO) and examine factors associated with MHO in children with obesity. This cross-sectional study was a secondary, exploratory analysis of data that included 2-17 years old with a body mass index (BMI) ≥85th percentile from the CANadian Pediatric Weight management Registry. Children were classified as having MHO or metabolically unhealthy obesity (MUO) using consensus-based criteria. Those with MHO had normal triglycerides, high-density lipoprotein cholesterol, blood pressure, and fasting glucose. Logistic regression was used to examine factors associated with MHO, which included calculating odds ratios (ORs) and 95% confidence intervals (CIs). In total, 945 children were included (mean age: 12.3 years; 51% female). The prevalence of MHO was 31% (n = 297), with lower levels across increasing age categories (2-5 years [n = 18; 43%], 6-11 years [n = 127; 35%], 12-17 years [n = 152; 28%]). Children with MHO were younger, weighed less, and had lower BMI z-scores than their peers with MUO (all p < 0.01). MHO status was positively associated with physical activity (OR: 1.18; 95% CI: 1.01-1.38), skim milk intake (OR: 1.10; 95% CI: 1.01-1.19), and fruit intake (OR: 1.12; 95% CI: 1.01-1.24) and negatively associated with BMI z-score (OR: 0.69; 95% CI: 0.60-0.79), total screen time in hours (OR: 0.79; 96% CI: 0.68-0.92), and intake of fruit flavoured drinks (OR: 0.91; 95% CI: 0.84-0.99). These findings may help guide clinical decision-making regarding obesity management by focusing on children with MUO who are at relatively high cardiometabolic risk., (© 2021 World Obesity Federation.)

Objectives: (1) To explore individual and family characteristics related to anthropometric and cardiometabolic health indicators and (2) examine whether characteristics that correlate with cardiometabolic health indicators differ across severity of obesity at time of entry to Canadian pediatric weight management clinics., Methods: We conducted a cross-sectional analysis of 2-17 year olds with overweight or obesity who registered in the CANadian Pediatric Weight Management Registry (CANPWR) between May 2013 and October 2017 prior to their first clinic visit. Individual modifiable health behaviors included dietary intake, physical activity, screen time, and sleep. Family characteristics included parental BMI, family medical history, socioeconomic status and family structure. Linear mixed effects stepwise regression analysis was performed to determine which characteristics were related to each health indicator: BMI z-score; waist circumference; waist to height ratio; blood pressure; glycemia; HDL cholesterol; non-HDL cholesterol; triglycerides., Results: This study included 1296 children (mean age ± standard deviation: 12.1 ± 3.5 years; BMI z-score: 3.55 ± 1.29; 95.3% with obesity). Hours spent sleeping (estimated β = -0.10; 95% CI [-0.15, -0.05], p = 0.0001), hours per week of organized physical activity (estimated β = -0.32; 95% CI [-0.53, -0.11], p = 0.0026), daily sugared drink intake (estimated β = 0.06; 95% CI [0.01, 0.10], p = 0.0136) and maternal BMI (estimated β = 0.03; 95% CI [0.02, 0.04], p < 0.0001) were associated with BMI z-score (adj. R 2  = 0.2084), independent of other individual and family characteristics. Physical activity, total sugared drink intake and sleep duration were associated with glycemia and non-HDL cholesterol, independent of child BMI z-score. However, irrespective of obesity severity, little of the variance (0.86-11.1%) in cardiometabolic health indicators was explained by individual modifiable health behaviors., Conclusions: Physical activity, total sugared drink intake and hours spent sleeping were related to anthropometric and some cardiometabolic health indicators in children entering pediatric weight management programs. This highlights the importance of these modifiable health behaviors on multiple health indicators in children with obesity., (© 2021. The Author(s), under exclusive licence to Springer Nature Limited.)

Congenital adrenal hyperplasia (CAH), if untreated, carries high morbidity and mortality. A higher incidence of CAH is expected in countries where consanguinity is common, such as in the countries of the WHO Eastern Mediterranean Region (EMRO). CAH is managed through lifelong treatment with fludrocortisone and hydrocortisone. In this analysis, performed in the 22 EMRO countries and territories plus Algeria, we review which countries offer a neonatal screening programme for CAH and describe the barriers and opportunities to access oral fludrocortisone and oral and injectable hydrocortisone. Neonatal CAH screening was only available nationally in Qatar, Kuwait and partially in Lebanon and Saudi Arabia. We reviewed the national lists of essential medicines (NEMLs) and found that 13/23 (57%) countries included fludrocortisone and 18/23 (78%) included oral hydrocortisone. Fludrocortisone was not included by any of the low-income countries and oral hydrocortisone was only included by one low-income country. We then contacted paediatric endocrinologists in each country to assess perceived availability of these medicines. Overall, there was a relatively good consistency between inclusion of fludrocortisone and hydrocortisone in the NEML and their actual availability in a country. We propose several mechanisms to improve access, including prequalification by the WHO, a common registration process for groups of countries, pooled procurement, working with local pharmaceutical companies, special access status for medicines not yet registered in a country and compounding. We suggest that access to medicines requires a collaboration between health professionals, families of patients, health authorities, pharmaceutical companies and the WHO., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2021. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Clinical practice guidelines for pediatric weight management highlight the importance of family-based behavioral strategies to enhance health behaviors. Little is known, however, of how clinics implement these programs. The study objectives were to (1) describe how Canadian pediatric weight management clinics deliver care and (2) evaluate change in services over time. Methods: The CANadian Pediatric Weight management Registry (CANPWR) is a multisite prospective cohort study of participants enrolled in a Canadian pediatric weight management clinic. Clinical program characteristics ( e.g. , referral process, inclusion criteria, funding, program characteristics, patient interaction methods, and follow-up) were collected at the start and end of the CANPWR recruitment period (2015-2019). Results: Entrance into the nine clinics varied with limiting criteria based on geographic proximity, age, weight status, and presence of health conditions. The clinics varied in size (50-220 new patients/year). The planned length of intervention varied widely, from 10 weeks to open-ended (median 2 years). Behavior modification strategies were delivered with a mix of individual and group-based sessions and most were delivered in person, complemented by use of virtual care. Over time, more clinics saw patients under the age of 5 years and all clinics defined a program length. Conclusion: Although all clinics offered family-based behavioral weight management services, these varied considerably, especially in program entrance criteria, size of clinic, and the length of intervention. The influence of the variability in delivery of services on health outcomes will be addressed in future studies.

Objectives: Post-thyroidectomy hypocalcemia is a common complication that causes increased morbidity. This review aims to identify the factors that predict occurrence of hypocalcemia after total thyroidectomy in children and adolescents., Methods: Comprehensive searches of English language pediatric (≤18 years of age) articles were performed in Medline, CINAHL, EMBASE, Web of Science and the Cochrane Library. Studies published between January 1, 1970 to August 20, 2020 regarding risk factors and strategies to prevent hypocalcemia were included if the study: 1. included only pediatric patients who were ≤18 years of age, 2. included only patients who had total, subtotal or completion thyroidectomy, 3. defined hypocalcemia as serum total calcium of <2.0 mmol/L (8 mg/dL) or ionized calcium of <1.0 mmol/L. The quality of included papers was assessed using the Newcastle-Ottawa scale. Results of all included studies were summarised. Meta-analyses were performed if appropriate., Results: Five studies with a total of 477 patients between 0 and 18 years, who had total/subtotal/completion thyroidectomy, were included. Overall rates of transient hypocalcemia were higher than permanent hypocalcemia (transient n = 104, 22%; permanent n = 48, 10%). Two studies found intraoperative parathyroid hormone (PTH) useful in predicting hypocalcemia. While two single institution cohort studies reported that neck dissection, male sex and a lower Parathyroid Gland Remaining In Situ score were associated with hypocalcemia, two other studies, including one population-based cohort study, reported that age at surgery, patient sex, hospital volume, type of thyroid disease, number of identified parathyroid glands, parathyroid auto-transplantation, operation time, thyroid specimen weight, and lymph node dissection were not risk factors for hypocalcemia., Conclusion: Intraoperative PTH may be useful in predicting hypocalcemia in children after total thyroidectomy. No consistent clinical risk factors or preventative methods were identified in the pediatric literature. High-quality pediatric research is urgently required to address this knowledge gap., (Copyright © 2021 Elsevier B.V. All rights reserved.)

Background: Electronic learning (e-learning) is a widely accessible, low-cost option for learning remotely in various settings that allows interaction between an instructor and a learner., Objective: We describe the development of a free and globally accessible multilingual e-learning module that provides education material on topics in pediatric endocrinology and diabetes and that is intended for first-line physicians and health workers but also trainees or medical specialists in resource-limited countries., Methods: As complements to concise chapters, interactive vignettes were constructed, exemplifying clinical issues and pitfalls, with specific attention to the 3 levels of medical health care in resource-limited countries. The module is part of a large e-learning portal, ESPE e-learning, which is based on ILIAS (Integriertes Lern-, Informations- und Arbeitskooperations-System), an open-source web-based learning management system. Following a review by global experts, the content was translated by native French, Spanish, Swahili, and Chinese-speaking colleagues into their respective languages using a commercial web-based translation tool (SDL Trados Studio)., Results: Preliminary data suggest that the module is well received, particularly in targeted parts of the world and that active promotion to inform target users is warranted., Conclusions: The e-learning module is a free globally accessible multilingual up-to-date tool for use in resource-limited countries that has been utilized thus far with success. Widespread use will require dissemination of the tool on a global scale., (©Evangelia Kalaitzoglou, Edna Majaliwa, Margaret Zacharin, Carine de Beaufort, Jean-Pierre Chanoine, Conny van Wijngaard-DeVugt, Ervin Sperla, Annemieke M Boot, Stenvert L S Drop. Originally published in JMIR Formative Research (http://formative.jmir.org), 05.11.2020.)

It is established that pediatric hematopoietic stem cell transplant (HSCT) recipients have a lower rate of chronic graft-versus-host disease (cGvHD) compared to adults. Our group has previously published immune profiles changes associated with cGvHD of clinically well-defined adult and pediatric HSCT cohorts. Since all analyses were performed by the same research group and analyzed using identical methodology, we first compared our previous immune profile analyses between adults and children. We then performed additional analyses comparing the T cell populations across age groups, and a sub-analysis of the impact of the estimated pubertal status at time of HSCT in our pediatric cohort. In all analyses, we corrected for clinical covariates including total body irradiation and time of onset of cGvHD. Three consistent findings were seen in both children and adults, including elevations of ST2 and naive helper T (Th) cells and depression of NK reg cells. However, significant differences exist between children and adults in certain cytokines, B cell, and T reg populations. In children, we saw a broad suppression of newly formed B (NF-B) cells, whereas adults exhibited an increase in T1-CD21 lo B cells and a decrease in T1-CD24 hi CD38 hi B cells. Prepubertal children had elevations of aminopeptidase N (sCD13) and ICAM-1. T reg abnormalities in children appeared to be primarily in memory T reg cells, whereas in adults the abnormalities were in naïve T reg cells. In adults, the loss of PD1 expression in naïve T reg and naïve Th cells was associated with cGvHD. We discuss the possible mechanisms for these age-related differences, and how they might theoretically impact on different therapeutic approaches to cGvHD between children and adults., (Copyright © 2020 Cuvelier, Li, Drissler, Kariminia, Abdossamadi, Rozmus, Chanoine, Ng, Mostafavi, Brinkman and Schultz.)

Cerebral edema (CE) is a rare but potentially fatal complication of diabetic ketoacidosis (DKA) in children with type 1 diabetes. CE is frequently mentioned as being more common in young children. The primary objective of this study was to review the evidence suggesting that younger age is a risk factor for the development of CE during DKA. The secondary objective was to assess if younger children are at a higher risk of DKA and severe DKA. A literature review was performed, and studies which reported the frequency of CE, DKA and severe DKA in children <3 and 3 to 5 years of age were included. Among the 6 studies reporting the frequency of CE that were identified, 5 good-quality studies found no significant association between younger age and higher risk of CE. Twenty-seven studies (DKA frequency: 11.3% to 54%) reported DKA frequency as a function of age. Most published studies found a higher frequency of DKA in children <5 years of age (20/25 studies), and in particular in those in the first 2 to 3 years of life (8/8 studies). There was inconclusive evidence to determine whether the severity of DKA was influenced by age. In conclusion, the commonly held view that CE is more common in younger children is not supported by the existing literature. Published data suggest that DKA (and possibly severe DKA) is more common in very young children. Regardless of age, all children with DKA should be monitored carefully for the development of CE., (Copyright © 2019 Canadian Diabetes Association. Published by Elsevier Inc. All rights reserved.)

Aim: To document normative data on penile size and anogenital distances of apparently normal term male infants in Sagamu, Nigeria., Methods: This cross-sectional study was carried out on 303 term male infants within the first 72 hours of life. Stretched penile length, penile width and three measures of anogenital distances were recorded: anogenital distance-1, anogenital distance-2 and anoscrotal distance., Results: The mean (±SD) stretched penile length and penile width were 39.4 ± 4.2 mm and 11.0 ± 1.0 mm, respectively. The mean values for anogenital distance-1, anogenital distance-2 and the anoscrotal distance were 48.7 ± 3.9 mm, 42.8 ± 4.6 mm and 25.5 ± 3.9 mm, respectively. Only penile width and anogenital distance-1 significantly correlated with all the anthropometric parameters but anogenital distance-1 had the strongest correlation (p < 0.01 for all)., Conclusion: The observed mean stretched penile length was greater than previously reported. Local references are preferable in interpreting anogenital measurements in newborns to avoid diagnostic errors. There is need for a consensus on the definition of micropenis., (©2019 Foundation Acta Paediatrica. Published by John Wiley & Sons Ltd.)

Background: Children with acute lymphocytic leukemia (ALL) are enrolled in advanced treatment protocols involving high doses of glucocorticoids (GCs). Current protocols do not advocate tapering of GCs doses postinduction phase. Prolonged administration of supra-physiologic doses of GCs can induce transient suppression of the hypothalamic pituitary adrenal axis (HPA). Timely recognition of adrenal insufficiency is important in order to ensure that children at risk receive the necessary treatment and follow-up including stress coverage during illness and surgical procedures. Methods: 21 newly diagnosed patients with ALL aged 3-10 years old were prospectively enrolled in the study over a 2-year period. All enrolled patients received high doses of GCs as part of a chemotherapy treatment protocol. The HPA axis was assessed prior to the induction phase of chemotherapy and 1-2 weeks after un-tapered discontinuation of GCs. Results: All children had normal HPA axis at baseline. Postinduction 1 mcg ACTH stimulation test result was normal (cortisol > 500 nmol/L) in 75% of children and partially responsive in 25% (cortisol 300-500 nmol/L). None of the participants demonstrated clinically significant adrenal insufficiency following abrupt cessation of GCs. Conclusion: All children in our cohort had either normal or subnormal cortisol response during a low dose ACTH stimulation test 1 to 2 weeks following abrupt discontinuation of GCs, suggesting that any inhibition of the HPA axis is of short duration. We suggest that future studies investigate the timing of adrenal function recovery following GC discontinuation as well as whether tapering of the GC should be recommended.

Pediatric specialists are often unavailable in low- and middle-income countries. As part of multiple professional associations' efforts to improve access to endocrine expertise globally, a pediatric endocrine teleconsultation network was established on a store-and-forward teleconsultation platform to facilitate focused, language-appropriate advice that can be kept for future reference while bypassing real-time video-conferencing, and obviating the need for a scheduled appointment. User information was recorded, and quality statistics on network performance and qualitative evaluation by referring physicians were analyzed. Over a 3-year period, 81 referrers (88% from Haiti) and 13 pediatric endocrinologists registered onto the network and discussed 47 pediatric endocrine cases, exchanging a total of 412 messages for a median of 7 messages (IQR 5, 11) per case. Diagnoses spanned the spectrum of pediatric endocrine disorders. According to referrers, an appropriate expert was consulted and an answer provided sufficiently quickly in 100% of cases. The answer was well-adapted to their environment in 86%, and referrers were able to follow the advice given in 72%. All but one referrer found the advice helpful, it clarified the diagnosis in 88%, assisted with management in 93%, improved patient's symptoms in 77%, improved function in 77%, and was considered cost-saving in 50%. Perceived benefits of the consultations were academic instruction, setting-adapted advice beyond the scope of guidelines or textbooks, and advancement in the diagnostic process. Pediatric endocrine remote store-and-forward consultations in low- and middle-income countries may provide a reasonable alternative to face-to-face visits, providing clinical and educational benefit, and a potential for cost-saving., (Copyright © 2019 von Oettingen, Craven, Duperval, Sine St. Surin, Eveillard, Saint Fleur, Van Vliet, Chanoine and Louis.)

Objective: The incidence of disorders of sexual development (DSD) is unknown in sub-Saharan Africa. We describe the characteristics and incidence of DSD in a cohort of infants born in Ghana., Design: Trained research assistants performed systematic genital examination at birth. All infants with suspected abnormal genitalia were further examined by a paediatric endocrinologist., Setting: Komfo Anokye Teaching Hospital, Kumasi, Ghana., Patients: Consecutive infants born in a single centre over a 1-year period (May 2014 to April 2015)., Main Outcome Measures: Incidence of DSD. Micropenis was defined as a stretched length <2.1 cm and clitoromegaly as a clitoral length >8.6 mm., Results: We examined 9255 infants (93% of all live births) within 72 hours of birth. Twenty-six neonates had a DSD. Nineteen infants had DSD without genital ambiguity: isolated micropenis (n=2), hypospadias (n=7), cryptorchidism (n=4) and clitoromegaly (n=6). Seven infants had DSD with ambiguity: clitoromegaly with a uterus on ultrasound and elevated 17-hydoxyprogesterone, suggesting XX DSD due to congenital adrenal hyperplasia (CAH)(n=4) and micropenis, hypospadias and gonads in a bifid scrotum or in the inguinal region, consistent with XY DSD (n=3)., Conclusion: The incidence of atypical genitalia was 28/10,000 (95% CI 17/10 000 to 39/10 000) live births. The incidence of CAH was 4.3/10 000 (95% CI 1.2/10 000 to 11.1/10 000) and was strongly associated with consanguinity., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Paediatric obesity management remains generalised to dietary and exercise modifications with an underappreciation for the contributions of eating behaviours and appetitive traits in the development of obesity., Objectives: To determine whether treatment-seeking children and adolescents with obesity cluster into phenotypes based on known eating behaviours and appetitive traits ("eating correlates") and how socio-demographic and clinical characteristics associate with different phenotypes., Methods: A cross-sectional, multi-centre questionnaire was administered between November 2015 and March 2017 examining correlates of eating in children and adolescents attending weight-management programmes in Canada. Latent profile analysis was used to cluster participants based on seven eating correlate scores obtained from questionnaires. Analysis of variance (ANOVA) was used to determine phenotype differences on socio-demographic and clinical characteristics. Multinomial logistic regression models assessed relative risk of specific characteristics associating with a disordered eating phenotype., Results: Participants were 247 children and adolescents (45.3% male, mean BMI z-score = 3.4 ± 1.0 kg/m 2 ) from six paediatric weight management centres in Canada. Seven eating correlates clustered into three distinct phenotypes: (1) loss of control eating, emotional eating, external eating, hyperphagia, impulsivity ("Mixed-Severe"; n = 42, 17%), (2) loss of control eating, emotional eating, external eating, hyperphagia ("Mixed-Moderate"; n = 138, 55.9%), and (3) impulsivity ("Impulsive"; n = 67; 27.1%). Social functioning scores and body esteem were significantly different across groups, with the Mixed-Severe participants having the poorest social functioning and lowest body esteem. Low body esteem indicated a greater risk of being in a multi-correlate group compared to the Impulsive group, while poor social function had a greater risk of clustering in the Mixed-Severe than Impulsive phenotype., Conclusions: Distinct eating phenotypes were found in treatment-seeking children and adolescents with obesity. Empirical evidence is needed, but these data suggest that tailored treatment approaches could be informed by these classifications to improve weight-management outcomes.

Although prolonged engagement in paediatric weight management (PWM) is associated with positive treatment outcomes, little is currently known about enablers of long-term programme attendance. The purpose of our study was to explore families' reasons for and facilitators of their continued attendance at health services for PWM. Semi-structured, individual interviews were conducted with parents of children (10-17 year old; body mass index ≥85th percentile) who completed the active phase of treatment in one of four Canadian multidisciplinary clinics for PWM. Interview data were recorded digitally, transcribed verbatim and analysed thematically. Parents' (n = 40) reasons for continued clinic attendance included ongoing concerns (eg, parental concern about their child's health), actual and expected benefits from treatment (eg, lifestyle improvements) and perceived quality of care (eg, structured, comprehensive, tailored health services). Several logistical and motivational factors contributed to continued attendance, including flexible work schedules, flexible appointment times, financial resources and children's motivation for treatment. Helping families address treatment barriers and ensuring that weight management services meet families' treatment expectations are promising strategies to enhance retention in PWM to optimize health outcomes., (© 2019 World Obesity Federation.)

Background: Previous studies suggest significant ethnic and racial differences in clitoral sizes and anogenital distances in the newborn. This study aimed to document normative data on clitoral sizes and anogenital distances of apparently normal term female infants in Sagamu., Methods: The study was a multi-center, cross-sectional descriptive research carried out among 317 female term infants within the first 72 h of life. Interviewer-based questionnaire was applied to obtain sociodemographic data, pregnancy and birth history. A sliding digital caliper was used for measurement. Data analysis was with SPSS version 20.0., Results: The mean clitoral length was 6.7 ± 1.6 mm while the mean clitoral width was 5.6 ± 0.8 mm. The mean fourchette-clitoris distance, anus-clitoris distance and anus-fourchette distance were 21.9 ± 2.1 mm, 35.5 ± 2.5 mm and 17.0 ± 2.6 mm respectively. The anus-clitoris and anus-fourchette distances significantly correlated with the anthropometric parameters while the clitoral measurements did not., Conclusion: The mean values recorded in this study were higher than observed in most previous studies. This simple, affordable and non-invasive evaluation could aid early diagnosis and treatment of female infants with potentially harmful conditions such as congenital adrenal hyperplasia., Competing Interests: Competing interestsThe authors declare that they have no competing interests., (© The Author(s). 2019.)

Objectives: As a follow-up to the CHIPS trial (Control of Hypertension In Pregnancy Study) of 'less tight' (versus 'tight') control of maternal blood pressure in pregnancy, CHIPS-Child investigated potential developmental programming of maternal blood pressure control in pregnancy, by examining measures of postnatal growth rate and hypothalamic-pituitary adrenal (HPA) axis activation., Methods: CHIPS follow-up was extended to 12 ± 2 months corrected post-gestational age for anthropometry (weight, length, head/waist circumference). For eligible children with consent for a study visit, we collected biological samples (hair/buccal samples) to evaluate HPA axis function (hair cortisol levels) and epigenetic change (DNA methylation analysis of buccal cells). The primary outcome was 'change in z-score for weight' between birth and 12 ± 2 mos. Secondary outcomes were hair cortisol and genome-wide DNA methylation status., Results: Of 683 eligible babies, 183 (26.8%) were lost to follow-up, 83 (12.2%) declined, 3 (0.4%) agreed only to ongoing contact, and 414 (60.6%) consented. 372/414 (89.9%) had weight measured at 12mos. In 'less tight' (vs. 'tight') control, the primary outcome was similar [-0.26 (-0.53, +0.01); p = 0.14, p adjusted  = 0.06]; median (95% confidence interval) hair cortisol (N = 35 samples) was lower [-496 (-892, -100) ng/g; p = 0.02], and buccal swab DNA methylation (N = 16 samples) was similar. No differences in growth rate could be demonstrated up to 5 years., Conclusions: Results demonstrate no compelling evidence for developmental programming of growth or the HPA axis. Clinicians should look to the clinical findings of CHIPS to guide practice. Researchers should seek to replicate these findings and extend outcomes to paediatric blood pressure and neurodevelopment., (Copyright © 2018 The Authors. Published by Elsevier B.V. All rights reserved.)

Background: Country-specific data on outcomes of treatment with recombinant human growth hormone are lacking. We present such data for children treated with growth hormone in Canada., Methods: We describe characteristics and outcomes of 850 children (mean age at baseline 8.5 yr) treated with growth hormone constituting the Canadian cohort of the multinational phase IV prospective observational Genetics and Neuroendocrinology of Short-stature International Study (GeNeSIS). The diagnosis associated with short stature was as determined by the investigator. Auxological data were evaluated yearly until near-adult height. Adverse events were assessed in all growth-hormone-treated patients., Results: The diagnosis ascribed as the cause of short stature was growth hormone deficiency in 526 children (61.9%), predominantly organic rather than idiopathic, particularly congenital pituitary abnormalities and intracranial tumours. All diagnostic groups with sufficient patients for analysis had increased height velocity standard deviation score (SDS) and height SDS during growth hormone treatment. For patients who reached near-adult height ( n = 293), the mean height SDS was within the normal range for about 80% of patients with organic growth hormone deficiency ( n = 131) or idiopathic growth hormone deficiency ( n = 50), 50% of patients with idiopathic short stature ( n = 10) and 46% of patients with Turner syndrome ( n = 79). Eleven deaths were reported, 7 in patients with organic growth hormone deficiency. Serious adverse events considered related to growth hormone treatment ( n = 19) were isolated except for medulloblastoma recurrence ( n = 2) and adenoidal hypertrophy ( n = 2)., Interpretation: Growth hormone treatment was effective and had a good safety profile in Canadian children. Growth hormone dosages were lower than in the US and global GeNeSIS cohorts, and a greater proportion of treated Canadian children had organic growth hormone deficiency., Study Registration: ClinicalTrials.gov, no. NCT01088412., Competing Interests: Competing interests: Cheri Deal has been involved with phase III and/or phase IV studies with Eli Lilly and Company, Serono, Versartis and OPKO Health, invited lectureships sponsored by Novo Nordisk, Eli Lilly and Company, Pfizer, EMD Serono and Merck Serono, ad hoc consulting with EMD Serono, Novo Nordisk, Merck Serono, Versartis, OPKO Health and Zafgen, and the Grant for Growth Innovation Scientific Advisory Board: Merck. She was a member of the Genetics and Neuroendocrinology of Short Stature International Study International Advisory Board. She has received honoraria and meeting expenses from Eli Lilly and Company. Susan Kirsch and Nan Jia are employees of Eli Lilly and Company. Jean-Pierre Chanoine has received honoraria (speaker fees and advisory board membership) from Eli Lilly and Company. Elizabeth Cummings has received grant support from Eli Lilly and Company and Sanofi. Elizabeth Rosolowsky has received grant support from Eli Lilly and Company and has served on the advisory boards for Insulet and Medtronic. Christopher Child is an employee and stockholder of Eli Lilly and Company. No other competing interests were declared., (Copyright 2018, Joule Inc. or its licensors.)

Background: There is increasing recognition of the value of "real-world evidence" in evaluating health care services. Registry-based, observational studies conducted in clinical settings represent a relevant model to achieve this directive. Starting in 2010, we undertook a longitudinal, observational study (the CANadian Pediatric Weight management Registry [CANPWR]), which is embedded in 10 multidisciplinary, pediatric weight management clinics across Canada. The objective of this paper was to share the lessons our team learned from this multi-centre project., Methods: Data sources included a retrospective review of minutes from 120 teleconferences with research staff and investigators, notes taken during clinical site visits made by project leaders, information from quality control processes to ensure data accuracy and completeness, and a study-specific survey that was sent to all sites to solicit feedback from research team members (n = 9). Through an iterative process, the writing group identified key themes that surfaced during review of these information sources and final lessons learned were developed., Results: Several key lessons emerged from our research, including the (1) value of pilot studies and central research coordination, (2) need for effective and regular communication, (3) importance of consensus on determining outcome measures, (4) challenge of embedding research within clinical practice, and (5) difficulty in recruiting and retaining participants. The sites were, in spite of these challenges, enthusiastic about the benefits of participating in multi-centre collaborative studies., Conclusion: Despite some challenges, multi-centre observational studies embedded in pediatric weight management clinics are feasible and can contribute important, practical insights into the effectiveness of health services for managing pediatric obesity in real-world settings.

Background: Exergaming is potentially useful to promote physical activity in children; however, long-term effectiveness is unclear. MobileKids Monster Manor (MKMM) is a mobile exergame developed with the help of young advisors. The game wirelessly transmits physical activity data from an accelerometer to a mobile device. Players' steps are redeemed for in-game rewards, for example, new characters., Objective: First, to evaluate whether increased physical activity previously observed in a 1-week intervention is sustained over a 2-week intervention and 1-week follow-up, and second, to compare impact in schools within different socioeconomic environments., Methods: Thirty-seven elementary school students participated in a 4-week randomized controlled study (1-week baseline; 2-week intervention [with only the Game group receiving MKMM]; and 1-week follow-up). All participants wore a Tractivity ® accelerometer throughout. Linear mixed models were applied to assess sustainability; a second 42-children-based dataset and age-/sex-adjusted linear regression models were used to compare effect across socioeconomic environments., Results: In the first week of intervention, the Game group compared to the Control group showed a greater increase in physical activity (of 1,758 steps/day [95% confidence interval, CI = 133-3,385] and 31 active minutes/day [95% CI = 4-59]), relative to baseline (13,986 steps/day; 231 active minutes/day). However, this was not sustained in the second intervention week or follow-up. The school within a lower socioeconomic status environment showed lower baseline activity and the 1-week intervention resulted in a greater increase relative to baseline (3,633 steps/day more [95% CI = 1,281-5,985])., Conclusion: MKMM could be a useful short-term physical activity promotion tool; however, effectiveness may decrease as novelty diminishes.

Objective: To explore parents' recommendations to enhance enrollment in multidisciplinary clinical care for managing pediatric obesity., Study Design: Data for this interpretative description study were collected through individual, semistructured interviews that were audiorecorded, transcribed verbatim, and analyzed thematically. Parents (n = 79) were recruited from 4 multidisciplinary weight management clinics in Canada located in Edmonton, Hamilton, Montreal, and Vancouver., Results: Most interviewed parents had children with obesity (body mass index ≥95th percentile; 84.2%), were female (87.3%), had postsecondary education (69.6%), and were white (75.9%). Parents' recommendations referred to enrollment opportunities, information about obesity services, motivation for treatment, and accessibility to obesity services. Specifically, parents recommended to increase referral options and follow-up contacts with families during the enrollment process, inform referring physicians and families about the availability and characteristics of obesity services, enhance families' motivation for treatment, prevent families from getting discouraged, make services more appealing to families, and address accessibility issues (eg, offering multiple options for appointment times, providing support for transportation)., Conclusions: Parents' recommendations support the need for family-centered approaches to enhance enrollment; however, their feasibility, acceptability, and effectiveness remain to be tested empirically., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Background: Access to essential medicines in pediatric endocrinology and diabetes is limited in resource-limited countries. The World Health Organization (WHO) maintains two non-binding lists of essential medicines (EMLs) which are often used as a template for developing national EMLs., Methods: We compared a previously published master list of medicines for pediatric endocrinology and diabetes with the WHO EMLs and national EMLs for countries within the WHO African region. To better understand actual access to medicines by patients, we focused on diabetes and surveyed pediatric endocrinologists from 5 countries and assessed availability and true cost for insulin and glucagon., Results: Most medicines that are essential in pediatric endocrinology and diabetes were included in the national EMLs. However, essential medicines, such as fludrocortisone, were present in less than 30% of the national EMLs despite being recommended by the WHO. Pediatric endocrinologists from the 5 focus countries reported significant variation in terms of availability and public access to insulin, as well as differences between urban and rural areas. Except for Botswana, glucagon was rarely available. There was no significant relationship between Gross National Income and the number of medicines included in the national EMLs., Conclusions: Governments in resource-limited countries could take further steps to improve EMLs and access to medicines such as improved collaboration between health authorities, the pharmaceutical industry, patient groups, health professionals, and capacity-building programs such as Paediatric Endocrinology Training Centres for Africa., (© 2018 S. Karger AG, Basel.)

Background: High-dose steroid administration is no longer recommended in the treatment of acute traumatic brain injury (TBI) as it failed to prove beneficial in improving patients' outcome. However, a masked benefit of steroid administration in TBI management was that it provided corticosteroid replacement therapy in patients with TBI-related central adrenal insufficiency., Case Presentation: We report the case of a 12-year-old boy who suffered a severe TBI from a motor vehicle accident that resulted in complete deficiency of anterior pituitary function. Central adrenal insufficiency was not ruled out by a near normal response to a low-dose ACTH test performed on D11., Conclusion: Consideration should be given to the empirical treatment of TBI pediatric patients with stress doses of corticosteroids if injury to the hypothalamus or pituitary gland is possible until a formal assessment of the hypothalamic-pituitary-adrenal axis can be made.

The Arab societies have the highest prevalence of consanguineous marriages; this results in an increased incidence of autosomal recessive conditions. There are different trends of family marriages across the globe and also the teachings of major religions differ from each other. The culture of family marriages is no more limited in any specific part of world due to rapid and mass migration of people secondary to wars or economic reasons. The endocrine conditions are relatively less discussed in the medical literature as well as in genetic counselling programs. The aim of this review is to highlight endocrine conditions associated with consanguineous marriages; it also discusses the cultural and religious trends of family marriages; the barriers and scarcity of good counselling programs., (Copyright© of YS Medical Media ltd.)

Objective: To explore parents' reasons for discontinuing tertiary-level care for paediatric weight management., Methods: Participants were parents of 10-17year olds (body mass index [BMI] ≥85th percentile) who were referred for paediatric weight management. Parents were recruited from three Canadian sites (Vancouver, BC; Edmonton, AB; Hamilton, ON) and were eligible if their children attended at least one clinical appointment and subsequently discontinued care. Data were collected using semi-structured individual interviews that were digitally recorded, transcribed, and analysed using an inductive manifest content analysis., Results: Parents (n=29) of children [mean age: 14.7±1.8years; mean BMI percentile: 98.9±1.6; n=17 (58.6%) boys] were primarily female (n=26; 89.7%), Caucasian (n=22; 75.9%), and had a university degree (n=23; 79.3%). Reasons for discontinuing care were grouped into three categories: (i) family factors (e.g., perceived lack of progress, lack of family support, children's lack of motivation), (ii) logistical factors (e.g., monetary costs, distance, scheduling), and (iii) health services factors (e.g., unmet expectations of care, perceived limited menu of services, no perceived need for further support)., Conclusions: A range of multi-level factors influenced attrition from tertiary-level paediatric weight management. Our data suggest that experimental research is needed to examine whether addressing reasons for attrition can enhance families' retention in care and ultimately improve health outcomes for children living with obesity., (Copyright © 2016 Asia Oceania Association for the Study of Obesity. Published by Elsevier Ltd. All rights reserved.)

Background: Few studies have evaluated the effect of adherence to a lifestyle intervention on adolescent health outcomes. The objective of this study was to determine whether adolescent and parental adherence to components of an e-health intervention resulted in change in adolescent body mass index (BMI) and waist circumference (WC) z-scores in a sample of overweight/obese adolescents., Methods: In total, 159 overweight/obese adolescents and their parents participated in an 8-month e-health lifestyle intervention. Each week, adolescents and their parents were asked to login to their respective website and to monitor their dietary, physical activity, and sedentary behaviours. We examined participation (percentage of webpages viewed [adolescents]; number of weeks logged in [parents]) and self-monitoring (number of weeks behaviors were tracked) rates. Linear mixed models and multiple regressions were used to examine change in adolescent BMI and WC z-scores and predictors of adolescent participation and self-monitoring, respectively., Results: Adolescents and parents completed 28% and 23%, respectively, of the online component of the intervention. Higher adolescent participation rate was associated with a decrease in the slope of BMI z-score but not with change in WC z-score. No association was found between self-monitoring rate and change in adolescent BMI or WC z-scores. Parent participation was not found to moderate the relationship between adolescent participation and weight outcomes., Conclusions: Developing strategies for engaging and promoting supportive interactions between adolescents and parents are needed in the e-health context. Findings demonstrate that improving adolescents' adherence to e-health lifestyle intervention can effectively alter the weight trajectory of overweight/obese adolescents.

Background: Information sharing in chronic conditions such as disorders of/differences in sex development (DSD) is essential for a comprehensive understanding by parents and patients. We report on a qualitative analysis of communication skills of fellows undergoing training in paediatric endocrinology. Guidelines are created for the assessment of communication between health professionals and individuals with DSD and their parents., Methods: Paediatric endocrinology fellows worldwide were invited to study two interactive online cases (www.espe-elearning.org) and to describe a best practice communication with (i) the parents of a newborn with congenital adrenal hyperplasia and (ii) a young woman with 46,XY gonadal dysgenesis. The replies were analysed regarding completeness, quality, and evidence of empathy. Guidelines for structured assessment of responses were developed by 22 senior paediatric endocrinologists worldwide who assessed 10 selected replies. Consensus of assessors was established and the evaluation guidelines were created., Results: The replies of the fellows showed considerable variation in completeness, quality of wording, and evidence of empathy. Many relevant aspects of competent clinical communication were not mentioned; 15% (case 1) and 17% (case 2) of the replies were considered poor/insufficient. There was also marked variation between 17 senior experts in the application of the guidelines to assess communication skills. The guidelines were then adjusted to a 3-level assessment with empathy as a separate key item to better reflect the qualitative differences in the replies and for simplicity of use by evaluators., Conclusions: E-learning can play an important role in assessing communication skills. A practical tool is provided to assess how information is shared with patients with DSD and their families and should be refined by all stakeholders, notably interdisciplinary health professionals and patient representatives., (© 2017 S. Karger AG, Basel.)

Acylation of ghrelin is mediated by ghrelin O- acyltansferase (GOAT). Exogenous acylated ghrelin (AG) stimulates growth hormone (GH) and food intake. In non-pregnant (NP) animals, the GOAT-ghrelin-GH axis prevents hypoglycemia caused by caloric restriction (CR). In humans, maternal malnutrition challenges glucose metabolism, which is a key determinant of fetal health. To clarify the role of AG and GH, we compared effects of CR on the GOAT-ghrelin-GH axis in pregnant (P) and NP mice. C57BL/6 wild type (WT) and GOAT knock-out (KO) P and NP mice were freely fed (FF) or subjected to 50% CR for one week. CR was started in P mice on Day 10.5 after conception. We measured body composition, blood glucose, plasma ghrelin and GH, stomach, hypothalamus and pituitary GOAT and ghrelin expression, and liver glycogen content and Pck1 expression. GOAT and AG were undetectable in KO. In NP mice, CR did not affect blood glucose (-1.3 mmol/l, p>0.05) in WT but was lowered (-1.8 mmol/l, p<0.0001) in KO. GH and Pck1 mRNA expression increased in WT but not in KO. In P mice, CR markedly lowered glucose (-2.7 mmol/l; p<0.0001) in WT and caused fatal hypoglycemia in KO, despite similarly elevated GH in WT and KO mice. KO animals are more prone to hypoglycemia than WT. GH, which is high in P animals, does not prevent hypoglycemia caused by CR during pregnancy. Our data suggest a specific role of AG in the regulation of gluconeogenesis to maintain euglycemia during pregnancy when energy availability is limited., (© Georg Thieme Verlag KG Stuttgart · New York.)

Background: Accurately determining energy requirements is key for nutritional management of pediatric obesity. Recently, a portable handheld indirect calorimeter, MedGem (MG) has become available to measure resting energy expenditure (REE). Our work aims to determine the clinical validity and usefulness of MG to measure REE in overweight and obese adolescents., Methods: Thirty-nine overweight and obese adolescents (16 male (M): 23 female (F), 15.2 ± 1.9 y, BMI percentile: 98.6 ± 2.2%) and 15 normal weight adolescents (7M: 8F, age 15.2 ± 2.0 y, BMI percentile: 39.2 ± 20.9%) participated. REE was measured with both MG and standard indirect calorimeter (VMax) in random order., Results: MG REE (1,600 ± 372 kcal/d) was lower than VMax REE (1,727 ± 327 kcal/) in the overweight and obese adolescents. Bland Altman analysis (MG -VMax) showed a mean bias of -127 kcal/d (95% CI = -72 to -182 kcal/d, P < 0.001), and a proportional bias existed such that lower measured REE by VMax was underestimated by MG, and higher measured REE by VMax were overestimated by MG., Conclusion: MG systematically underestimates REE in the overweight and adolescent population, thus the MG portable indirect calorimeter is not recommended for routine use. Considering that it is a systematic underestimation of REE, MG may be clinically acceptable, only if used with caution.

Background: Genital measurements are a useful adjunct in the early detection of various endocrine conditions including hypopituitarism and disorders of sexual differentiation. Standards for genital sizes have been published but racial/ethnic differences exist. This study was done to establish norms for genital sizes in term Ghanaian male newborns., Methods: This was a cross-sectional study of all apparently well full-term newborns of postnatal age < 48 h and birth weight between 2.5 and 4.0 kg delivered at Komfo Anokye Teaching Hospital within the study period. Anthropometric and genital parameters were documented for study subjects as well as parental socio-demographic indices., Results: A total of 644 male newborns were recruited between May and September 2014. The mean penile length (MPL) was 3.3 ± 0.5 cm and the mean penile width (MPW) was 1.05 ± 0.1 cm. An inverse relationship was found between maternal age and MPL (correlation coefficient -0.062, 95% CI -0.121 to -0.002; p  = 0.04). MPL was also significantly different ( p  = 0.04) by mode of delivery, with babies delivered by caesarean section having the lowest MPL. MPL correlated positively with both gestational age ( p  = 0.04) and birth length ( p  < 0.001), while MPW correlated proportionally with birth weight and length ( p  < 0.001 for both)., Conclusions: Using the conventional definition of micropenis as stretched penile length (SPL) < 2.5 standard deviation (SD) below the mean and macropenis as an SPL > 2.5 SD, a Ghanaian term newborn may warrant investigation if he has an MPL < 2.1 cm or > 4.4 cm.

Background: Data on normative clitoral sizes in newborns is relatively sparse and racial/ethnic differences have also been reported. This study was performed to establish norms for clitoral size in term Ghanaian female newborns., Methods: This was a cross-sectional study of all apparently well full-term newborns of postnatal age < 48 h and birth weight between 2.5 and 4.0 kg delivered at Komfo Anokye Teaching Hospital between May and September, 2014. Anthropometric and genital parameters were documented for study subjects as well as parental socio-demographic indices., Results: In 612 newborn females studied, the mean (±SD) clitoral length (MCL) and the mean (±SD) clitoral width (MCW) were 4.13 ± 1.6 mm and 4.21 ± 1.1 mm, respectively. MCL was inversely related to birth weight ( r  = -0.62; p < 0.001 ) while MCW was inversely related to both gestational age ( r  = -0.1; p = 0.02 ) and birth weight ( r  = -0.54; p < 0.001 ). Babies with a clitoris that was completely covered by the labia majora had relatively lower clitoral sizes ( p  < 0.001) than those who had a partially covered or prominent clitoris. Neither MCL nor MCW differed significantly by birth length or maternal tribe., Conclusions: Clitoral size varies with birth weight and gestational age. Babies with a completely covered clitoris are unlikely to warrant detailed clitoral measurements for clitoromegaly.

Background: Anogenital distance (AGD) is a simple noninvasive measure of foetal androgen exposure. This study was done to generate normative data on AGD in Ghanaian newborns., Methods: AGD was measured in 644 male and 612 female term newborns; including the distance between the anterior base of the penis and the centre of the anus, the posterior base of the penis and the centre of the anus, and the posterior base of the scrotum and the centre of the anus (ASD) in males and the distance from the anus to the fourchette (AF) and from the anus to the base of the clitoris in females. Other anthropometric and parental socio-demographic indices were also documented., Results: AGD was sexually dimorphic; with a mean ± SD ASD and AF of 25.5 ± 5.1 and 13.6 ± 2.7 mm, respectively. There was a significant correlation between AGD and birth weight, birth length, and occipitofrontal circumference (p < 0.05). ASD was significantly longer (p < 0.001) in newborns (83/644; 12.9%) of mothers who had ingested herbs during pregnancy., Conclusion: AGD was approximately twice as long in males compared to females and can serve as a useful indicator of androgen exposure. Measurements of AGD also need to factor in anthropometric parameters, which are important correlates of AGD., (© 2017 S. Karger AG, Basel.)

Paediatric endocrinology and diabetes is a paediatric specialty with less common conditions and higher cost medicines. Access to medicines for our specialty in low and middle income countries remains limited. We analysed the content of the WHO (children and adults) and of all available national Model Lists of Essential Medicines (EMLs) for Mexico, the Caribbean, Central and South America from a paediatric endocrinology and diabetes standpoint. A master list of medicines deemed necessary in paediatric endocrinology and diabetes was established and compared with the WHO and national EMLs, taking into account the gross national income. The WHO EMLs, which are largely recognised as an international benchmark and drive the content of the national EMLs, included many but not all medicines present on our master list. Interestingly, several national EMLs from richer countries included medicines that were not present in the WHO EMLs. Our analysis suggests that these medicines could be considered by the WHO for inclusion in their EMLs, which may promote the adoption of more medicines by individual countries. We also propose several changes to the WHO and national EMLs that could facilitate access to medicines in our specialty: age cut-off for a child using physical maturity rather than a set age limit; greater standardisation of the formatting of the national EMLs for easier comparison and collaborations between countries; greater emphasis on age-specificity and population-specificity for some medicines; and formatting of the EMLs in a disease-focused manner rather than as individual medicines., Competing Interests: Competing interests: None declared.

Objective: Although parents are uniquely positioned to offer first-hand insights that can be used to improve health services for managing pediatric obesity, their perspective is underexplored. Our objective was to characterize parents' recommendations for enhancing tertiary-level health services for managing pediatric obesity in Canada., Methods: Semistructured, one-on-one interviews were conducted with parents of children who initiated treatment at 1 of 4 Canadian tertiary-level, multidisciplinary weight management clinics. Parent perspectives were elicited regarding the strengths and weaknesses of the health services they received as well as areas for potential improvement. Interviews were audio-recorded and transcribed verbatim. We used qualitative description as the methodological framework and manifest content analysis as the analytical strategy., Results: Parents (n = 65; 88% female; 72% Caucasian; 74% with at least some postsecondary education; 52% >$50,000 CDN household income) provided a range of recommendations that were organized according to health care: 1) accessibility, 2) content, and 3) delivery. The most common recommendations included increasing scheduling options (44%; n = 29), tailoring services to families' needs and circumstances (29%; n = 19), placing greater emphasis on physical activity (29%; n = 19), altering program duration (29%; n = 19), incorporating interactive elements (25%; n = 16), information provision (25%; n = 16), and providing services at sites closer to participants' homes (24%; n = 15)., Conclusions: Parents' recommendations to enhance health services for managing pediatric obesity concerned modifiable factors related to accessibility, content, and delivery of care. Further research is needed to evaluate whether implementing suggested recommendations improves clinically relevant outcomes including attrition, quality of care, and success in weight management., (Copyright © 2016 Academic Pediatric Association. Published by Elsevier Inc. All rights reserved.)

Background: Few children with obesity who are referred for weight management end up enroled in treatment. Factors enabling enrolment are poorly understood. Our purpose was to explore reasons for and facilitators of enrolment in paediatric weight management from the parental perspective., Methods: Semi-structured interviews were conducted with parents of 10- to 17-year-olds who were referred to one of four Canadian weight management clinics and enroled in treatment. Interviews were audio-recorded and transcribed verbatim. Manifest/inductive content analysis was used to analyse the data, which included the frequency with which parents referred to reasons for and facilitators of enrolment., Results: In total, 65 parents were interviewed. Most had a child with a BMI ≥95th percentile (n = 59; 91%), were mothers (n = 55; 85%) and had completed some post-secondary education (n = 43; 66%). Reasons for enrolment were related to concerns about the child, recommended care and expected benefits. Most common reasons included weight concern, weight loss expectation, lifestyle improvement, health concern and need for external support. Facilitators concerned the referral initiator, treatment motivation and barrier control. Most common facilitators included the absence of major barriers, parental control over the decision to enrol, referring physicians stressing the need for specialized care and parents' ability to overcome enrolment challenges., Conclusions: Healthcare providers might optimize enrolment in paediatric weight management by being proactive in referring families, discussing the advantages of the recommended care to meet treatment expectations and providing support to overcome enrolment barriers., (© 2016 John Wiley & Sons Ltd.)

Physical inactivity is increasing among children globally and has been directly linked to the growing problems of overweight and obesity. We aim to assess the impact of a new mobile exergame, MobileKids Monster Manor (MKMM), in a school-based setting. MKMM, developed with input from youth to enhance physical activity, is wirelessly connected to an accelerometer-based activity monitor. Forty-two healthy students (11.3 ± 1.2 years old and 0.28 ± 1.29 body-mass index [BMI] z-score) participated in a randomized 4-week crossover study to evaluate the game intervention. The two study arms consisted of week-long baseline, game intervention/control, washout, and control/game intervention phases. All participants were required to wear an activity monitor at all times to record steps and active minutes for the study duration. MKMM was used during each arm's respective intervention week, during which children were asked to play the game at their convenience. When children were exposed to the game, an increase compared with the control phase of 2,934 steps per day (p = 0.0004, 95% CI 1,434-4,434) and 46 active minutes per day (p = 0.001, 95% CI 20-72) from baseline (12,299 steps/day and 190 active minutes/day) was observed. A linear regression model showed that MKMM yielded a greater increase in steps and active minutes per day among children with a higher BMI z-score, showing 10 percent more steps per day and 14 percent more active minutes per day relative to baseline, per unit increase in BMI z-score. In conclusion, MKMM increased steps and active minutes in a school-based environment. This suggests that mobile exergames could be useful tools for schools to promote physical activity and combat obesity in adolescents.