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Since the original guidelines were published, there has been additional work done to characterize the pathophysiology of cystic fibrosis-related diabetes (CFRD), including the role of genes related to type 2 diabetes, the role of inflammation, and the potential role of the basic CF transmembrane conductance regulator (CFTR) chloride channel defect. [A] The pathophysiology of CFRD is complex and includes the loss of pancreatic islet cells leading to both insulin and glucagon deficiency, fluctuating insulin resistance, the requirement for high caloric intake, gut abnormalities including delayed gastric emptying, altered intestinal motility, and liver disease. Factors specific to CF which impact glucose metabolism include the loss of total islets leading to both insulin and glucagon deficiency, chronic and acute inflammation and infection which cause fluctuating insulin resistance, a requirement for high caloric intake because of increased energy expenditure and malabsorption, risk of life-threatening malnutrition, and gut abnormalities including delayed gastric emptying, altered intestinal motility, and liver disease. Yes No No Insulin deficiency Nearly complete Partial, variable Severe, not complete Insulin sensitivity Somewhat decreased Severely decreased Somewhat decreased Ketones Yes Rare Rare Usual treatment Insulin Diet, oral meds, insulin Insulin Microvasular complications Yes Yes Yes Macrovascular complications Yes Yes No Metabolic syndrome No Yes No Cause of death Cardiovascular Cardiovascular Pulmonary Diagnostic criteria for CFRD and abnormal glucose tolerance The diagnostic criteria for CFRD were updated in 2010 in North America by the CFRD Guidelines Committee in a position statement co-sponsored by the American Diabetes Association (ADA) and the Cystic Fibrosis Foundation, and endorsed by the Pediatric Endocrine Society.

Recommendations: Regular self-monitoring of glucose (using accurate fingerstick blood glucose [BG] measurements, with or without continuous glucose monitoring [CGM] or intermittently scanned CGM [isCGM]), is essential for diabetes management for all children and adolescents with diabetes (A). ○ Each child should have access to technology and materials for self-monitoring of glucose measurements to test enough to optimize diabetes care (B). ○ Diabetes center personnel should advocate to nations, states, and health care funders to ensure that children and adolescents with diabetes have adequate glucose monitoring supplies (E). ○ When fingerstick BGs are used, testing may need to be performed 6 to 10 times per day to optimize intensive control. For children, adolescents, and young adults aged ≤25 years we recommend individualized targets, aiming for the lowest achievable HbA1c without undue exposure to severe hypoglycemia balanced with quality of life and burden of care (E). For children, adolescents, and young adults ≤25 years who have access to comprehensive care a target of HbA1c of <53 mmol/mol (7.0%) is recommended (E). ○ A higher HbA1c goal (in most cases <58 mmol/mol [7.5%]) is appropriate in the following contexts: inability to articulate symptoms of hypoglycemia, hypoglycemia unawareness/history of severe hypoglycemia, lack of access to analog insulins, advanced insulin delivery technology, ability to regularly check BG, and CGM (E), and individuals who are “high glycators,” in whom an at-target HbA1c would reflect a significantly lower mean glucose than 8.6 mmoL/L (155 mg/dL) (E). ○ A lower goal (6.5%) or 47.5 mmol/mol may be appropriate if achievable without excessive hypoglycemia, impairment of quality of life, and undue burden of care (E). ○ A lower goal may be appropriate during the honeymoon phase of type 1 diabetes (E). ○ For patients who have elevated HbA1c, a step-wise approach to improve glycemic control is advised including individualized attention to: dose adjustments (E), personal factors limiting achievement of the target (E), assessment of the psychological effect of goal setting on the individual (E), and incorporation of available technology to improve glucose monitoring and insulin delivery modalities (E). Importantly, recent data suggest that lowering HbA1c targets is associated with a decreased mean HbA1c on a population and individual level without an increased frequency of severe hypoglycemia, even in children who achieve HbA1c levels <53 mmol/mol (7.0%).

Many adolescents may experience a deterioration in metabolic control—attributable to one or several of the following: endocrine changes leading to increased insulin resistance (B) erratic meal and exercise patterns (C) poor adherence to treatment regimens (C) eating disorders (C) hazardous and risk-taking behaviors (C/E) It is essential to develop appropriate communication skills to facilitate teaching and education, and recognize the need for privacy and confidentiality for this age group (E). In the context of type 1 diabetes, many adolescents may experience a deterioration in metabolic control often attributable to erratic meal and exercise patterns, poor adherence to treatment regimens, hazardous and risk-taking behaviors, disordered eating behaviors, other mental health issues, and endocrine changes associated with puberty, leading to greater insulin resistance. Understand that attending to the developmental needs of young people may be just as important for quality of life as diabetes-specific treatment. When there is conflict between the needs of diabetes management and the adolescent's social development and peer activities, a stepwise approach with interim achievable goals can be used to bridge competing priorities Providing well-directed education to help understand the physiological changes of puberty, their effect on insulin dose, difficulties of weight control and dietary regulation.

Introduction: Growth hormone (GH) therapy is used to treat a variety of growth disorders in childhood/adolescence. Its efficacy is thought to be dependent on patients' adherence to their treatment regimen. Methods: PubMed was searched using the keywords ‘growth hormone', ‘child'[Mesh], ‘adolescent'[Mesh], and ‘patient compliance'[Mesh]. Results: Most studies of adherence to paediatric GH therapy have used either issued/encashed GH prescriptions or questionnaires. Estimates of prevalence of non-adherence vary from 5-82%, depending on the methods and definitions used. Different studies have variously demonstrated an association (or lack thereof) between adherence and age, socioeconomic status, treatment duration, injection device used and injection-giver. A number of interventions have been proposed to improve adherence, including offering a choice of injection device, but none are supported by trials. Poor adherence is associated with reduced height velocity and likely increased economic costs; evidence for other effects is circumstantial. Conclusion: Adherence to paediatric GH therapy is suboptimal, which may partially explain why the mean final height attained is below that of the general population. Analysis of the causes of non-adherence is complicated by conflicting evidence from different studies. Multifactorial interventions are most likely to be successful in improving adherence. We make recommendations for further research.

Depression and diabetes distress Disordered eating behaviors Cognitive functioning and school performance Parental distress, coping, and demographic factors Stress and coping Factors affecting quality of life, including resiliency Psychosocial and behavioral intervention studies to improve glycemic control, diabetes management behaviors, and psychosocial functioning EXECUTIVE SUMMARY AND RECOMMENDATIONS The following summary and recommendations build upon the previous ISPAD Guidelines and are consistent with the latest statements and guidelines issued by the American Diabetes Association, Australia (APEG—Clinical Practice Guidelines, ), Canada and the United Kingdom ). Young people with diabetes are at increased risk for mild decrements in general cognitive ability, information processing skills, executive functions, and academic achievement, especially if there is a background of early diabetes onset, severe hypoglycemia or chronic hyperglycemia (B). [...]it is recommended that assessment of developmental progress in all domains of functioning (ie, physical, intellectual, academic, emotional and social development) should be conducted on a routine basis (A, B, E). Identification of overall psychosocial well-being and diabetes-specific quality of life, as well as psychosocial adjustment problems, depression, eating disorders, diabetes distress, and other psychiatric disorders should be performed at planned intervals and by appropriately trained mental health professionals (B, E). Family conflict is associated with lower regimen adherence and poor glycemic control (B, C). [...]the interdisciplinary team should assess general family functioning (conflict, cohesion, adaptability, parental psychopathology) and diabetes-related functioning (communication, parental involvement and support, roles and responsibilities for self-management behaviors) especially during periods of transition (eg, at diagnosis, at start of a new treatment plan, early adolescence) and when there is an evidence of cultural, language or family problems or difficulties in adjustment to diabetes (A, B, E).

Matching of insulin dose to carbohydrate intake on intensive insulin regimens allows greater flexibility in carbohydrate intake and meal-times, with improvements in glycemic control and quality of life (A). The use of the glycemic index provides additional benefit to glycemic control over that observed when total carbohydrate is considered alone (B). The impact of diabetes on eating behavior must not be underestimated and may cause psychological disturbance. [...]experienced professionals should facilitate dietary and lifestyle changes. Achieve a balance between food intake, metabolic requirements, energy expenditure, and insulin action profiles to attain optimum glycemic control.

Closed-loop systems link continuous glucose measurements to insulin delivery. We aimed to establish whether closed-loop insulin delivery could control overnight blood glucose in young people. We undertook three randomised crossover studies in 19 patients aged 5–18 years with type 1 diabetes of duration 6·4 years (SD 4·0). We compared standard continuous subcutaneous insulin infusion and closed-loop delivery (n=13; APCam01); closed-loop delivery after rapidly and slowly absorbed meals (n=7; APCam02); and closed-loop delivery and standard treatment after exercise (n=10; APCam03). Allocation was by computer-generated random code. Participants were masked to plasma and sensor glucose. In APCam01, investigators were masked to plasma glucose. During closed-loop nights, glucose measurements were fed every 15 min into a control algorithm calculating rate of insulin infusion, and a nurse adjusted the insulin pump. During control nights, patients' standard pump settings were applied. Primary outcomes were time for which plasma glucose concentration was 3·91–8·00 mmol/L or 3·90 mmol/L or lower. Analysis was per protocol. This trial is registered, number ISRCTN18155883. 17 patients were studied for 33 closed-loop and 21 continuous infusion nights. Primary outcomes did not differ significantly between treatment groups in APCam01 (12 analysed; target range, median 52% [IQR 43–83] closed loop vs 39% [15–51] standard treatment, p=0·06; ≤3·90 mmol/L, 1% [0–7] vs 2% [0–41], p=0·13), APCam02 (six analysed; target range, rapidly 53% [48–57] vs slowly absorbed meal 55% [37–64], p=0·97; ≤3·90 mmol/L, 0% [0–4] vs 0% [0–0], p=0·16]), and APCam03 (nine analysed; target range 78% [60–92] closed loop vs 43% [25–65] control, p=0·0245, not significant at corrected level; ≤3·90 mmol/L, 10% [2–15] vs 6% [0–44], p=0·27). A secondary analysis of pooled data documented increased time in the target range (60% [51–88] vs 40% [18–61]; p=0·0022) and reduced time for which glucose concentrations were 3·90 mmol/L or lower (2·1% (0·0–10·0) vs 4·1% (0·0–42·0); p=0·0304). No events with plasma glucose concentration lower than 3·0 mmol/L were recorded during closed-loop delivery, compared with nine events during standard treatment. Closed-loop systems could reduce risk of nocturnal hypoglycaemia in children and adolescents with type 1 diabetes. Juvenile Diabetes Research Foundation; European Foundation for Study of Diabetes; Medical Research Council Centre for Obesity and Related Metabolic Diseases; National Institute for Health Research Cambridge Biomedical Research Centre.

Individual variability in human gender-related behaviour is influenced by many factors, including androgen exposure prenatally, as well as self-socialization and socialization by others postnatally. Many studies have looked at these types of influences in isolation, but little is known about how they work together. Here, we report that girls exposed to high concentrations of androgens prenatally, because they have the genetic condition congenital adrenal hyperplasia, show changes in processes related to self-socialization of gender-related behaviour. Specifically, they are less responsive than other girls to information that particular objects are for girls and they show reduced imitation of female models choosing particular objects. These findings suggest that prenatal androgen exposure may influence subsequent gender-related behaviours, including object (toy) choices, in part by changing processes involved in the self-socialization of gendered behaviour, rather than only by inducing permanent changes in the brain during early development. In addition, the findings suggest that some of the behavioural effects of prenatal androgen exposure might be subject to alteration by postnatal socialization processes. The findings also suggest a previously unknown influence of early androgen exposure on later processes involved in self-socialization of gender-related behaviour, and thus expand understanding of the developmental systems regulating human gender development.

Anogenital distance (AGD) in animals is a sensitive biomarker of fetal endocrine disruption and the associated testicular dysgenesis syndrome (TDS). However, AGD in human infants with cryptorchidism and hypospadias, which are potential manifestations of TDS during childhood, is not clearly described. Our aim was to compare AGD in boys with cryptorchidism or hypospadias against normative data. Boys with isolated cryptorchidism (n = 71, age 13.4 ± 5.8 months) or hypospadias (n = 81, age 11.4 ± 6.2 months) were recruited from a tertiary center for measurement of AGD and penile length; they were compared with 487 healthy full-term boys from a birth cohort by deriving age-specific standard deviation scores (SDS). Boys with cryptorchidism were older (p = 0.048) compared with boys with hypospadias. Boys with hypospadias had shorter mean AGD and penile length SDS than healthy boys (both p < 0.0001). Mean AGD and penile length SDS values in boys with cryptorchidism were longer than mean values in boys with hypospadias (both p < 0.01) and shorter than mean values in healthy boys (both p < 0.0001). Mean penile length SDS decreased as the severity of hypospadias increased (ptrend = 0.078). In the study population, AGD and penile length were reduced in boys with hypospadias or cryptorchidism relative to normative data derived from a longitudinal birth cohort. The findings support the use of AGD as a quantitative biomarker to examine the prenatal effects of exposure to endocrine disruptors on the development of the male reproductive tract.

Background: Anogenital distance (AGD) is sexually dimorphic in rodents anil humans, being 2- to 2. 5-fold greater in males. It is a reliable marker of androgen and antiandrogen effects in rodent reproductive toxicologic studies. Data on AGD in humans are sparse, with no longitudinal data collected during infancy. Objective: This study was designed to determine AGD from birth to 2 years in males and females and relate this to other anthropometric measures. Materials and Methods: Infants were recruited from the Cambridge Baby Growth Study. AGD was measured from the center of the anus to the base of the scrotum in males and to the posterior fourchette in females. Measurements were performed at birth and at 3, 12, 18, and 24 months of age. Results: Data included 2,168 longitudinal AGD measurements from 463 male and 426 female full-term infants (median = 2 measurements per infant). Mean AGD (± SD) at birth was 19.8 ± 6.1 mm in males and 9.1 ± 2.8 mm in females (p < 0.0001). AGD increased up to 12 months in both sexes and in a sex- dimorphic pattern. AGD was positively correlated with penile length at birth (r = 0.18, p = 0.003) and the increase in AGD from birth to 3 months was correlated with penile growth (r = 0.20, p = 0.001). Conclusion: We report novel, longitudinal data for AGD during infancy in a large U.K. birth cohort. AGD was sex dimorphic at all ages studied. The availability of normative data provides a means of utilizing this biological marker of androgen action in population studies of the effects of environmental chemicals on genital development.