Notes

Wolfram-like syndrome : an additional encounter of your unusual illness in youngsters.
buy Luminespib of rapamycin (mTOR) regulates cell proliferation, growth and survival, and is activated in cancer and neurological disorders, including epilepsy. The rapamycin derivative ("rapalog") everolimus, which allosterically inhibits the mTOR pathway, is approved for the treatment of partial epilepsy with spontaneous recurrent seizures (SRS) in individuals with tuberous sclerosis complex (TSC). In contrast to the efficacy in TSC, the efficacy of rapalogs on SRS in other types of epilepsy is equivocal. Furthermore, rapalogs only poorly penetrate into the brain and are associated with peripheral adverse effects, which may compromise their therapeutic efficacy. Here we compare the antiseizure efficacy of two novel, brain-permeable ATP-competitive and selective mTORC1/2 inhibitors, PQR620 and PQR626, and the selective dual pan-PI3K/mTORC1/2 inhibitor PQR530 in two mouse models of chronic epilepsy with SRS, the intrahippocampal kainate (IHK) mouse model of acquired temporal lobe epilepsy and Tsc1GFAP CKO mice, a well-characterized mouse model of epilepsy in TSC. During prolonged treatment of IHK mice with rapamycin, everolimus, PQR620, PQR626, or PQR530; only PQR620 exerted a transient antiseizure effect on SRS, at well tolerated doses whereas the other compounds were ineffective. In contrast, all of the examined compounds markedly suppressed SRS in Tsc1GFAP CKO mice during chronic treatment at well tolerated doses. Thus, against our expectation, no clear differences in antiseizure efficacy were found across the three classes of mTOR inhibitors examined in mouse models of genetic and acquired epilepsies. The main advantage of the novel 1,3,5-triazine derivatives is their excellent tolerability compared to rapalogs, which would favor their development as new therapies for TORopathies such as TSC.
Breakfast is commonly described as the most important meal of the day; however, we have little information regarding its relevance for childhood overweight and obesity in southern mainland China. This study aimed to assess the association between breakfast preferences, consumption location and overweight and obesity for children in Shenzhen.

Among 6126 children (median age 7.2 years) at primary schools, 3504 were finally included after a questionnaire survey and physical examination. Ten commonly consumed foods/beverages for breakfast in southern China were exposure variables, and consumption location, total energy demographic and other dietary characteristics were covariates in logistic regression to determine the effect of breakfast preferences on overweight and obesity, estimating odds ratios (ORs) and 95% confidence intervals (CIs).

Probability of overweight/general obesity was inversely associated with children eating plain congee for breakfast (adjusted OR 0.689, 95% CI 0.566-0.838), restricted to breakfast in China. However, frequent consumption of steamed rice roll for breakfast and especially eating out could contribute to overweight and obesity. Thus, we need to promote a healthier breakfast pattern among children in southern China to reduce the odds of obesity.Jean Rodier (1920-2003), distinguished researcher and scientist, directed the Toxicology Department of Hygiene Institute of Rabat under the French Protectorate. From 1946, he developed numerous lines of research in occupational health, in particular on Manganism, a neurological disorder that impacted miners in his home country of Morocco. His many papers on Manganism, only one of which was published in English, describe field and laboratory research studies that focused its prevention and management.Microgramma vacciniifolia is broadly used in folk medicine but safety information is unavailable. Therefore, we evaluated the toxicity of a saline extract and a lectin-rich fraction of M. #link# vacciniifolia rhizome. The extract showed hemolytic activity on mice erythrocytes at 1000 μg/mL, whereas the fraction promoted hemolysis (8.57-26.15%) at all tested concentrations (10-1000 μg/mL). Acute toxicity test in mice indicated an LD50 of >5000 mg/kg. Hematological alterations and increased serum alkaline phosphatase level were observed in the treated animals. Transaminases and urea levels increased in the groups treated with the extract or fraction at 5000 mg/kg. Leukocyte infiltration was observed in the liver of extract-treated animals and in the liver and lungs of mice treated with the fraction. The kidneys of animals treated with the fraction at 5000 mg/kg presented hydropic degeneration. link2 The extract and fraction did not induce oxidative stress in the liver and did not show genotoxicity, as examined by micronucleus and comet assays. In conclusion, the preparations were not lethal to mice but caused some signs of toxicity, mainly the fraction. The results indicated the need to evaluate the toxicity of M. vacciniifolia rhizome in other models and in chronic assays.
To use medical claims data to determine patterns of healthcare utilization in children with intellectual and developmental disabilities, including frequency of service utilization, conditions that require hospital care, and costs.

Medicaid administrative claims from 4 states (Iowa, Massachusetts, New York, and South Carolina) from years 2008-2013 were analyzed, including 108 789 children (75 417 male; 33 372 female) under age 18years with intellectual and developmental disabilities. Diagnoses included cerebral palsy, autism, fetal alcohol syndrome, Down syndrome/trisomy/autosomal deletions, other genetic conditions, and intellectual disability. Utilization of emergency department (ED) and inpatient hospital services were analyzed for2012.

Children with intellectual and developmental disabilities used both inpatient and ED care at 1.8 times that of the general population. Epilepsy/convulsions was the most frequent reason for hospitalization at 20 times the relative risk of the general population. Other fnce for developing outpatient care strategies that anticipate common clinical problems in intellectual and developmental disabilities and ensure responsive management before hospital care is needed.
To evaluate the cost-effectiveness of treating young children with chronic hepatitis C virus (HCV) with new direct-acting antivirals.

A state-transition model of chronic HCV was developed to conduct a cost-effectiveness analysis comparing treatment at age 6years vs delaying treatment until age 18years. Model inputs were derived from recently conducted systematic reviews, published literature, and government statistics. Medical care costs were obtained from linked population level laboratory and administrative data (Ontario, Canada). Outcomes are expressed in expected quality-adjusted life-years and costs (CAD$). Analysis included a base-case to estimate the expected value and one-way and probabilistic sensitivity analyses to evaluate the impact of uncertainty of the model inputs.

After 20years, treating 10 000 children early would prevent 330 cases of cirrhosis, 18 cases of hepatocellular carcinoma, and 48 liver-related deaths. The incremental cost-effectiveness ratio of early treatment compared to delayed treatment was approximately $12 690/quality-adjusted life-years gained and considered cost-effective. Model results were robust to variation in fibrosis progression rates, disease state-based costs, treatment costs, and utilities.

Delaying treatment until age 18years results in an increased lifetime risk of late-stage liver complications. Early treatment in children is cost effective. Our work supports clinical and health policies that broaden HCV treatment access to young children.
Delaying treatment until age 18 years results in an increased lifetime risk of late-stage liver complications. Early treatment in children is cost effective. Our work supports clinical and health policies that broaden HCV treatment access to young children.
To evaluate whether quality improvement (QI) capacity-building in affiliated primary care practices could increase well care visit uptake.

Partners For Kids (PFK) is an accountable care organization caring for pediatric Medicaid beneficiaries in Ohio. PFK QI specialists recruited practices to develop QI projects around increasing well care visit rates (proportion of eligible children with well care visits during calendar year) for children aged 3-6years and adolescents. The QI specialists supported practice teams in implementing interventions and collecting data through monthly or bimonthly practice visits.

Ten practices, serving more than 26 000 children, participated in QI projects for a median of 8.5months (IQR 5.3-17.6). Well care visit rates in the QI-engaged practices significantly improved from 2016 to 2018 (P<.001 for both age groups). Over time, well care visit rates for 3- to 6-year-old children increased by 11.8% (95% CI 5.4%-18.2%) in QI-engaged practices, compared with 4.1% (95% CI 0.1%-7.4%) in non-engaged practices (P=.233). For adolescents, well care visit rates increased 14.3% (95% CI -2.6% to 31.2%) compared with 5.4% (95% CI 1.8%-9.0%) in QI-engaged vs non-engaged practices over the same period (P=.215). Although not statistically significant, QI-engaged practices had greater magnitudes of rate increases for both age groups.

Through practice facilitation, PFK helped a diverse group of community practices substantially improve preventive visit uptake over time. QI programs in primary care can reach patients early to promote preventive services that potentially avoid costly downstream care.
Through practice facilitation, PFK helped a diverse group of community practices substantially improve preventive visit uptake over time. QI programs in primary care can reach patients early to promote preventive services that potentially avoid costly downstream care.
To assess the national prevalence of inadequate sleep among school-age children and its relationship with childhood flourishing.

This cross-sectional study analyzed 49 050 parental responses from the 2016-2017 National Survey of Children's Health for school-age children. Inadequate sleep duration was defined as <9hours for 6- to 12-year-olds and <8hours for 13- to 17-year-olds on an average weeknight. Five markers of flourishing were examined individually and as a combined measure. Logistic regression was used with complex survey design and applied weights.

Inadequate sleep was found in 36.4% of 6- to 12-year-olds and in 31.9% of 13- to 17-year-olds. Compared with children with adequate sleep, 6- to 12-year-olds with inadequate sleep had increased odds of not showing interest and curiosity in learning (aOR, 1.61; 95% CI, 1.34-1.94), not caring about doing well in school (aOR, 1.45; 95% CI, 1.23-1.71), not doing homework (aOR, 1.44; 95% CI, 1.24-1.68), and not finishing tasks (aOR, 1.18; 95% CI, 1.03-1.35). Children aged 13-17years with inadequate sleep had increased odds of not doing homework (aOR, 1.36; 95% CI, 1.17-1.58), not staying calm and in control when challenged (aOR, 1.34; 95% CI, 1.16-1.54), not showing interest and curiosity in learning (aOR, 1.34; 95% CI, 1.14-1.58), not finishing tasks (aOR, 1.20; 95% CI, 1.03-1.40), and not demonstrating the combined flourishing measure (aOR, 1.35; 95% CI, 1.17-1.56).

Nationally representative data show that one-third of school-age children have inadequate sleep. Inadequate sleep is associated with decreased flourishing. These data will help inform sleep policies and optimize child development.
Nationally representative data show that one-third of school-age children have inadequate sleep. Inadequate sleep is associated with decreased flourishing. link3 These data will help inform sleep policies and optimize child development.
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