Notes

Comparability of fresh DXA technique, Shimazu SONIALVISION G4, using GE-Lunar Natural born player.
Background Early infancy and childhood are critical periods in the establishment of lifelong weight trajectories. Parents and early family environment have a strong effect on children's health behaviors that track into adolescence, influencing lifelong risk of obesity. Objective We aimed to identify developmental trajectories of body mass index (BMI) from early childhood to adolescence and to assess their early individual and family predictors. Methods This was a secondary analysis of the Millennium Cohort Study and included 17,165 children. Weight trajectories were estimated using growth mixture modeling based on age- and gender-specific BMI Z-scores, followed by a bias-adjusted regression analysis. Results We found four BMI trajectories Weight Loss (69%), Early Weight Gain (24%), Early Obesity (3.7%), and Late Weight Gain (3.3%). Weight trajectories were mainly settled by early adolescence. Lack of sleep and eating routines, low emotional self-regulation, child-parent conflict, and low child-parent closeness in early childhood were significantly associated with unhealthy weight trajectories, alongside poverty, low maternal education, maternal obesity, and prematurity. Conclusions Unhealthy BMI trajectories were defined in early and middle-childhood, and disproportionally affected children from disadvantaged families. This study further points out that household routines, self-regulation, and child-parent relationship are possible areas for family-based obesity prevention interventions.Objectives There is an increasing interest in cannabinoid-based products for the treatment of refractory pediatric epilepsy. However, a licensed cannabidiol (CBD) product was first approved for use by the European regulatory authorities in 2019. We aimed to obtain knowledge about clinical experience and attitudes toward cannabinoid use for epilepsy treatment among neuropediatricians in Scandinavia and Germany in the era before a CBD-product was commercially licensed and available. Study design An internet-based questionnaire (Survey Monkey) was distributed by email to members of neuropediatric societies in Sweden, Germany, Denmark, and Norway between February and April 2018. One reminder email was sent. Results Eighty-six responded. Only 10 of 86 (12%) respondents had personal experience with off-label prescription of cannabinoid-based products, mainly for severe refractory pediatric epilepsies like Dravet syndrome and Lennox-Gastaut syndrome. However, 49 respondents (57%) had been exposed to relatives of patients that had requested or wanted to discuss cannabinoid therapy, and 32 (37%) respondents knew about cannabinoid self-medication. Iruplinalkib The knowledge regarding cannabinoid-based therapy among the respondents was overall limited. Main reasons for not prescribing cannabinoid-based therapy were concerns about law regulations and lack of an available product. Conclusion Off-label cannabinoid-based therapy for pediatric epilepsy was not widely prescribed by neuropediatricians in Scandinavia and Germany in 2018.Objective The purpose of this article was to demonstrate related characteristics of intensive care unit (ICU) admission after an unscheduled revisit by febrile children visiting the emergency department (ED). Method We performed a retrospective study in a tertiary medical center from 2010 to 2016. Patients whose chief complaint was fever and who were admitted to the ICU following a 72-h return visit to the ED were included, and we selected patients who were discharged from the same emergency department for comparison. Results During the study period, 54 (0.03%) patients met the inclusion criteria, and 216 patients were selected for the matched control group. Regarding clinical variables on initial ED visit, visiting during the night shift (66.7 vs. 46.8%, p = 0.010), shorter length of 1st ED stay (2.5 ± 2.63 vs. 3.5 ± 3.44 h, p = 0.017), and higher shock index (SI) (1.6 ± 0.07 vs. 1.4 ± 0.02, p = 0.008) were associated with ICU admission following a return visit. On the return ED visit, we found that clinical variables such as elevated heart rate, SI, white blood cell count, and C-reactive protein level were all associated with ICU admission. Furthermore, elevated SI and pediatric age-adjusted (SIPA) values were observed in the study group in both the initial (42.2 vs. 20.1%, OR2.3 (1.37-4.31), p = 0.002) and return ED visits (29.7 vs. 6.9%, OR 4.6 (2.42-8.26), p less then 0.001). Conclusion For children who visited the emergency department with a febrile complaint, elevated SIPA values on the initial ED visit were associated with ICU admission following an unscheduled ED revisit within 72 h.Objective 11β-hydroxylase deficiency (11βOHD) is a rare autosomal recessive disorder caused by mutations in the CYP11B1 gene. It is characterized by virilization, hypertension, and significant final height impairment. In this study, we aim to investigate the clinical and molecular characteristics of four unrelated Chinese patients with 11βOHD disorder. Methods The clinical information of four 11βOHD patients were carefully reviewed. Genetic analysis was performed using next-generation sequencing (NGS) based panel analysis. NGS coverage depth was analyzed to detect exonic copy-number variants (CNVs) on patient 1. Quantitative PCR (qPCR) was subsequently performed to confirm the CNVs detected from the NGS coverage depth analysis. Results The mean age of the patients at diagnosis was 4.7 years (range, 2.0-9.3 years). Two genetically female patients (patients 1 and 2) with 11βOHD presented severe virilization of external genitalia and were raised as males. Two genetically male patients (patients 3 and 4) presented precocious puberty. Additionally, patients 1, 3, and 4 presented with hypertension. In patient 4, unilateral adrenal mass was detected and removed at the age of 9 years. Interestingly, the height of patient 4 (174.4 cm, +6.7 SD) wasn't impaired and reached his mid-parental height (173 cm). Three novel variants in the CYP11B1 gene (c.1150_1153del, c.217C>T, and c.400G>C) were identified by NGS. Various bioinformatics tools revealed potential pathogenic effects for the novel variants, and evolutionary-conservation revealed that the novel missense variant affected an amino acid that is highly conserved among species. Furthermore, NGS coverage depth analysis and qPCR identified a novel heterozygous deletion of exons 1-6 in patient 1. Conclusion Our study expands the spectrum of mutations of the CYP11B1 gene in Chinese population. In addition, We reported the first case of a patient with classical 11βOHD disorder, whose final height wasn't compromised.
Website: https://www.selleckchem.com/products/iruplinalkib.html