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Background: Ataxia telangiectasia (AT) is a genetic multisystem disorder, presenting with progressive ataxia, immune deficiency, and propensity toward malignancy. Endocrine abnormalities (growth retardation, reproductive dysfunction, and diabetes) have been described, however detailed information regarding this aspect is lacking. We aimed to characterize endocrine anomalies and growth patterns in a large cohort of AT patients. Methods: Retrospective study comprising all 52 patients (aged 2–26.2 y) followed at a national AT Clinic. Anthropometric and laboratory measurements were extracted from the charts. Results: Median height-SDS was already subnormal during infancy, remaining negative throughout follow up to adulthood. Height-SDS was more impaired than weight-SDS up to age 4 y, thereafter weight-SDS steadily decreased, resulting in progressively lower BMI-SDS. IGF-I-SDS was low (−1.53 ± 1.54), but did not correlate with height-SDS. Gonadal failure was present in all 13 females older than 10 y but only in one male. Two patients had diabetes and 10 had dyslipidemia. Vitamin D deficiency was observed in 52.2% of the evaluated patients. Conclusion: Our results suggest a primary growth abnormality in AT, rather than secondary to nutritional impairment or disease severity. Sex hormone replacement should be considered for female patients. Vitamin D levels should be followed and supplementation given if needed.

Background Data regarding glycemic control in children and adolescents with a dual diagnosis of type 1 diabetes mellitus (T1DM) and attention‐deficit/hyperactivity disorder (ADHD) are limited. Objective To compare various aspects of diabetes control among youth with T1DM, between those with and without ADHD. Methods In this cross‐sectional study of youth with T1DM, 39 had ADHD (mean age 14.1 ± 2.8 years) and 82 did not (control group, mean age 12.6 ± 3.3 years). Health‐related quality of life was assessed by a Diabetes Quality of Life (DQOL) questionnaire submitted to their parents. Glycemic data were downloaded from glucometers, pumps, and continuous glucose monitoring systems. HbA1c levels, hospitalizations, and severe hypoglycemic and diabetes ketoacidosis events were retrieved from the medical files. Results Compared to the control group mean HbA1c level of the ADHD group was higher: 8.3 ± 1.1% versus 7.7 ± 1.0% (p = 0.005) and the percent of time that glucose level was in the target range (70–180 mg/dl) was lower: 48 ± 17% versus 59 ± 14% (p = 0.006). Mean glucose and glucose variability were higher in the ADHD group. Youth with ADHD who were not pharmacologically treated had worse HbA1c and more hospitalizations than those who were treated. DQOL did not differ between the control group, the treated ADHD group, and the untreated ADHD‐Group. Conclusions Dual diagnosis of T1DM and ADHD during childhood leads to worse diabetes control, which is more pronounced in the context of untreated ADHD. Healthcare providers should be aware of the difficulties facing youth with T1DM and ADHD in coping with the current intensive treatment of diabetes.

Growth retardation is an established complication of anorexia nervosa (AN). However, findings concerning final height of AN patients are inconsistent. The aim of this study was to assess these phenomena in female adolescent inpatients with AN. We retrospectively studied all 211 female adolescent AN patients hospitalized in an inpatient eating disorders department from 1/1/1987 to 31/12/99. Height and weight were assessed at admission and thereafter routinely during hospitalization and follow-up. Final height was measured in 69 patients 2-10 years after discharge. Pre-morbid height data was available in 29 patients. Patients' height standard deviation scores (SDS) on admission (-0.285±1.0) and discharge (-0.271±1.02) were significantly (p<0.001) lower than expected in normal adolescents. Patients admitted at age ≤13 years, or less than 1 year after menarche, were more severely growth-impaired than patients admitted at an older age, (p = 0.03). Final height SDS, available for 69 patients, was -0.258±1.04, significantly lower than expected in a normal population (p = 0.04), and was more severely compromised in patients who were admitted less than 1 year from their menarche. In a subgroup of 29 patients with complete growth data (pre-morbid, admission, discharge, and final adult height), the pre-morbid height SDS was not significantly different from the expected (-0.11±1.1), whereas heights at the other time points were significantly (p = 0.001) lower (-0.56±1.2, -0.52±1.2, and -0.6±1.2, respectively). Our findings suggest that whereas the premorbid height of female adolescent AN patients is normal, linear growth retardation is a prominent feature of their illness. Weight restoration is associated with catch-up growth, but complete catch-up is often not achieved.

Survivors of childhood cancer are at high risk for developing non-melanoma skin cancer and therefore are firmly advised to avoid or minimize sun exposure and adopt skin protection measures. We aimed to compare sun exposure and protection habits in a cohort of pediatric patients with a history of malignancy to those of healthy controls. Case-control study of 143 pediatric patients with a history of malignancy (aged 11.2±4.6 y, Male = 68, mean interval from diagnosis 4.4±3.8 y) and 150 healthy controls (aged 10.4±4.8 y, Male = 67). Sun exposure and protection habits were assessed using validated questionnaires. Patients and controls reported similar sun exposure time during weekdays (94±82 minutes/day vs. 81±65 minutes/day; p = 0.83), while during weekends patients spent significantly less time outside compared to controls (103±85 minutes/day vs. 124±87 minutes/day; p = 0.02). Time elapsed from diagnosis positively correlated with time spent outside both during weekdays (r = 0.194, p = 0.02) and weekends (r = 0.217, p = 0.01), and there was a step-up in sun exposure starting three years after diagnosis. There was no significant difference regarding composite sun protection score between patients and controls. Age was positively correlated with number of sunburns per year and sun exposure for the purpose of tanning, and was negatively correlated with the use of sun protection measures. Although childhood cancer survivors are firmly instructed to adopt sun protection habits, the adherence to these instructions is incomplete, and more attention should be paid to improve these habits throughout their lives. Since sunlight avoidance may results in vitamin D deficiency, dietary supplementation will likely be needed.

Klotho is an aging-modulating protein expressed mainly in the kidneys and choroid plexus, which can also be shed, released into the circulation and act as a hormone. Klotho deficient mice are smaller compared to their wild-type counterparts and their somatotropes show marked atrophy and reduced number of secretory granules. Recent data also indicated an association between klotho levels and growth hormone (GH) levels in acromegaly. We aimed to study the association between klotho levels and GH deficiency (GHD) in children with growth impairment. Prospective study comprising 99 children and adolescents (aged 9.0 ± 3.7 years, 49 male) undergoing GH stimulation tests for short stature (height-SDS = -2.1 ± 0.6). Klotho serum levels were measured using an α-klotho ELISA kit. Klotho levels were significantly lower (p<0.001) among children with organic GHD (n = 11, 727 ± 273 pg/ml) compared to both GH sufficient participants (n = 59, 1497 ± 754 pg/ml) and those with idiopathic GHD (n = 29, 1645 ± 778 pg/ml). The difference between GHS children and children with idiopathic GHD was not significant. Klotho levels positively correlated with IGF-1- standard deviation scores (SDS) (R = 0.45, p<0.001), but were not associated with gender, pubertal status, age or anthropometric measurements. We have shown, for the first time, an association between low serum klotho levels and organic GHD. If validated by additional studies, serum klotho may serve as novel biomarker of organic GHD.

Freeform/Key Words: adolescents, menstruation, polycystic ovary syndrome, AMH, oligomenorrhea

Background Metabolic bone disease is a common manifestation of celiac disease (CD). We aimed to assess fracture risk among children and adolescents with CD compared with a matched group. Methods This registry-based cohort study included 2372 children with CD who were matched 1:5 to 11,860 children without CD. Demographic and clinical data were obtained from the electronic database of Meuhedet, a health maintenance organization. Fracture events at ages 1–18 years were identified by coded diagnoses. Results The overall fracture incidence rate was 256 per 10,000 patient-years (PY) in the CD group and 165 per 10,000 PY in the comparison group ( p  < 0.001). The hazard ratio (HR) to have a fracture was 1.57 (95% CI 1.43–1.73, p  < 0.001) for the CD group compared to the matched group. The HR for multiple fractures was 1.67 (95% CI 1.38–2.01, p  < 0.001). Analysis of the pre- and post-diagnosis periods separately showed that the HR for fractures in the pre-diagnosis period was 1.64 (95% CI 1.42–1.88, p  < 0.001) for the CD group compared to the matched group, and 1.52 (95% CI 1.26–1.71, p  < 0.001) in the period from diagnosis to the end of the follow-up period. Conclusions Children with CD had increased fracture risk both preceding and following the diagnosis of CD. Impact One manifestation of celiac disease (CD) is metabolic bone disease, including osteoporosis and impaired bone mineralization. We found increased fracture risk among children with CD, both preceding the CD diagnosis and during the years following the diagnosis. Recognition of the high risk of fractures in this population may help promote prevention. Further studies are needed to evaluate changes in bone quantity and quality after initiation of a gluten-free diet, and to identify those at risk for persistent metabolic bone disease.

Leptin and interleukin-6 (IL-6) are inversely correlated and associated with decreased survival in critically ill patients. We investigated changes in leptin, IL-6, and troponin in children undergoing open-heart surgery, hypothesizing that IL-6 and troponin will increase after cardiopulmonary bypass (CPB) and will be negatively correlated with leptin. Serial blood samples were collected from 21 patients 24 hours before and up to 48 hours after surgery. Leptin levels decreased by 50% during CPB ( P < .001), then gradually increased, reaching baseline levels 12 hours after surgery. The IL-6 levels increased ( P < .001) during CPB, peaking 2 hours after surgery and remaining slightly elevated at 24 hours after surgery ( P < .001). Leptin and IL-6 were negatively correlated ( R = −0.448, P < .001). Troponin levels increased during CPB ( P < .001). Postoperative leptin and troponin were inversely correlated ( r = −0.535, P < .001). Patients with modest elevations in troponin levels (<20 μg/L) had a shorter aortic clamp and CPB time ( P < .01), lower IL-6 peak levels ( P = .03), and shorter duration of ventilation and inotropic support compared with patients with peak troponin levels greater than 20 μg/L. Lower leptin and higher IL-6 levels correlated with troponin, a marker of myocardial injury. Because leptin may have cardioprotective effects, the postoperative drop in its levels may further contribute to myocardial dysfunction.

To analyse the risk of fractures among children with attention-deficit/hyperactivity disorder (ADHD) compared with matched children without ADHD; and to evaluate the impact of pharmacological treatment. This registry-based cohort study included 31,330 children diagnosed with ADHD and a comparison group of 62,660 children matched by age, sex, population sector and socioeconomic status. Demographic and clinical information was extracted from the electronic database of Meuhedet, a health maintenance organization. Fracture events between 2–18 years of age were identified by coded diagnoses. The overall fracture incidence rate was 334 per 10,000 patient-years (PY) in the ADHD group and 284 per 10,000 PY in the comparison group (p < 0.001). Among boys, the fracture incidence rates were 388 per 10,000 PY and 327 per 10,000 PY (p < 0.001), for the respective groups. Among girls, the rates were lower in both groups compared to boys, but higher in the ADHD compared to the matched group (246 vs 203 per 10,000 PY, p < 0.001). Among the children with ADHD, the hazard ratios (HR) to have a fracture were similar in boys (1.18, 95%CI 1.15–1.22, p < 0.001) and girls (1.22, 95%CI 1.16–1.28, p < 0.001). Children with ADHD were also at increased risk for two and three fractures; the hazard ratios (HRs) were 1.32 (95%CI 1.26–1.38, p < 0.001) and 1.35 (95%CI 1.24–1.46, p < 0.001), respectively. In a multivariable model of the children with ADHD, pharmacological treatment was associated with reduced fracture risk (HR 0.90, 95%CI 0.82–0.98, p < 0.001) after adjustment for sex, resident socioeconomic status and population sector.   Conclusion : Children with ADHD had greater fracture risk than a matched group without ADHD. Pharmacological treatment for ADHD may decrease this risk. What is Known: • Children with attention-deficit/hyperactivity disorder (ADHD) may be more prone to injuries and fractures than children without ADHD. What is New: • Children with ADHD were 1.2 times more likely to have a fracture than children with similar characteristics, without ADHD. The increased risk for fractures was even greater for two and three fractures (hazard ratios 1.32 and 1.35, respectively). • Our study suggests a positive effect of pharmacological treatment for ADHD in reducing fracture risk.

The COVID-19 pandemic led to fundamental changes in daily routines of children. Our aim was to evaluate the incidence and characteristics of fractures among Israeli children during 2020 compared with 2015–2019. Demographic, clinical data, and incidence rates of fractures in individuals aged < 18 years were derived from the electronic database of Meuhedet Health Services, which provides healthcare services to 1.2 million people in Israel. We further subdivided the year to five periods according to government regulations of lockdown and isolation at each period. Fracture sites were determined according to ICD9 definitions. During 2020, 10,701 fractures occurred compared with 12,574 ± 599 fractures per year during 2015–2019 ( p -value < 0.001). Fracture rates were lower during all periods in 2020. The largest decline was observed during the first lockdown for both boys (56% decline, 95% confidence interval [CI] 52–60%) and girls (47% decline CI 41–53%). While the fracture rate declined for most age groups, the largest decline was recorded for the age group 11–14 years, with significant reduction rates of 66% (CI 59–71%) for boys and 65% (CI 54–73%) for girls. The most prominent declines were of fractures of the hand bones of both boys and girls (64% and 59%, respectively).  Conclusions: Our data showed a significant decrease in fracture rate in 2020 compared to the previous 5 years, as well as differences between periods within that year. What is New: •The COVID-19 pandemic led to fundamental change in daily routines of children with significant decrease in school attendance and sport activities. •Consequent to these public health measures, the incidence rate of pediatric fractures decreased significantly. What is New: •This study demonstrates declines in fracture rates during lockdown periods, with only partial reversing of the trends between the lockdown periods. •The most pronounced decline was observed during the first lockdown period. •The decline was most prominent in children aged 11-14 years; there was no significant change in fracture incidence of children aged <3 years.