Notes

The Current and also Potential Position regarding Radiation Therapy inside the Era regarding Auto T-cell Repair.
Hajdu-Cheney Syndrome (HCS) is a rare genetic autosomal dominant disorder, characterized by distinctive facial features, acroosteolysis, and severe osteoporosis. Very rarely HCS is associated with polycystic kidney disease, splenomegaly or Crohn's disease (CD). It is caused by gain-of-function mutations in NOTCH2 gene. Treatment with bisphosphonates or denosumab is reported to result in BMD increase.

We report a mutation in exon 34 of NOTCH2 gene, in a Greek pedigree, with diverse phenotypes among members.

The 48-year-old mother had a history of a T12 vertebral fracture, postpartum at the age of 21 and two subsequent uneventful full-term pregnancies and never received treatment. Her 29-year-old son, presented with severe osteoporosis and multiple morphological vertebral fractures. Her 21-year-old daughter had recurrent vertebral fractures starting at 10 years of age. At 17 years, she developed severe CD, resistant to treatment with biologic agents, and functional hypothalamic hypogonadism. One male pedigree died of cystic fibrosis. ML349 All subjects bore the typical facial characteristics and acroosteolysis, while none had splenomegaly or renal defects. Zoledronate infusion led to BMD increase.

Mutation in c.6758 G > A (NM_008163.1), leading to a Trp2253Ter replacement. This mutation has been reported as possibly pathogenic (SCV000620308), but not in association with HCS.

Bone involvement can present with diverse severity in the same pedigree, ranging from low BMD to multiple fragility fractures. Antiresorptive therapy improves BMD, but its anti-fracture efficacy remains to be shown. The presence of CD might indicate the significant role of NOTCH2 signaling in different tissues.
Bone involvement can present with diverse severity in the same pedigree, ranging from low BMD to multiple fragility fractures. Antiresorptive therapy improves BMD, but its anti-fracture efficacy remains to be shown. The presence of CD might indicate the significant role of NOTCH2 signaling in different tissues.
The purpose of this study was to investigate the association of anthropometric indices with continuous metabolic syndrome (cMetS) risk score components in a large population-based sample of children and adolescents.

This multi-centric study was performed on 3843 students aged 7-18years who were selected by multistage, stratified cluster sampling method from 30 provinces of Iran. Demographic, anthropometric and biochemical factors were obtained and standardized residuals (z-scores) were calculated for MetS components. A structural equation modeling approach was applied to evaluate the relationships among the study variables and to implement the subsequent structural modeling.

The mean age of the participants (52.3% boys) was 12.4 ± 3.05years. Standardized scores of body mass index (ZBMI) and waist circumference (ZWC) had a direct effect on standardized scores of mean arterial pressure (ZMAP) (0.23 and 0.24 in boys and 0.22 and 0.23 in girls, respectively) and triglyceride (ZTG) (0.07 and 0.04 in boys and 0.02 and 0.06 in girls, respectively), but the effect of ZWC was stronger than ZBMI on these variables. Age, socioeconomic status and sedentary behaviors showed a positive direct effect on ZWC (0.01, 0.05 and 0.07 in boys and 0.05, 0.08 and 0.002 in girls, respectively). These variables induced indirect effects on cMets risk score components through ZWC.

The magnitude of association between WC and continuous metabolic syndrome risk score components was higher compared to BMI in school-aged children, emphasizing on paying more attention to central obesity in childhood.

Level V, cross-sectional descriptive study.
Level V, cross-sectional descriptive study.
We compared parent's perceived child's silhouette, and investigated predictors of their dissatisfaction.

Participants were 4930 mother-child dyads enrolled at a Portuguese birth cohort. Parents' perceptions of child's current and desired silhouette was assessed and dissatisfaction with child's silhouette was defined as the discrepancy between these ratings (current-desired body). Multinomial logistic regressions, adjusted for potential confounders, were performed.

Mothers were more dissatisfied with child's silhouette, compared to fathers, in all weight categories. Mothers and fathers of girls were more dissatisfied, preferring thinner silhouettes (OR = 2.77, 95% CI 2.19; 3.51 and OR = 2.08, 95% CI 1.18; 3.66, respectively), compared to parents of boys. Lower birth weight increased maternal desire for a heavier child silhouette. Younger (< 20years) and less educated (≤ 9years of schooling) mothers were more dissatisfied with their child's silhouette, preferring heavier children (OR = 1.65, 95% CI 1.10; 2.48 and OR = 1.73, 95% CI 1.42; 2.09, respectively). Parents' own dissatisfaction was also associated with child's silhouette dissatisfaction.

Sociodemographic characteristics and parents' dissatisfaction with their own silhouette influenced their dissatisfaction with child's silhouette and should be considered when developing obesity interventions.

Level III, case-control analytic study.
Level III, case-control analytic study.Previous studies have shown that placebo repetitive transcranial magnetic stimulation (rTMS) was effective on post-stroke motor rehabilitation. However, the placebo effect has not been systematically assessed. Therefore, this meta-analysis was conducted to resolve this issue and explore potential influencing factors further. PubMed, Embase, web of science and the Cochrane Library were searched for published randomised controlled trials (RCTs) with placebo rTMS treatment of stroke recovery until May 2019. The placebo effect size (Hedges' g) was estimated using the motor outcome of pre- and post- placebo rTMS treatment. Meta-regression analysis was also performed to explore potential influencing factors for the placebo effect. link2 Twenty-six placebo-controlled trials (including 381 patients in placebo group) were selected. Effect size results (Hedges' g = 0.466, 95% CI 0.207-0.726; P  less then  0.05) showed a medium and significant placebo rTMS effect on improving post-stroke motor recovery. The mean ratio of the effect size of sham to real stimulation was 56%. Meta-regression analysis did not find significant result except for the treatment sessions, which was significantly correlated with the placebo effect size (r = 0.465, p = 0.031). In the follow-up observations (1, 2 and 3 months), the sham rTMS groups manifested gradually increased motor improvement, which was similar to the real group, but the amplitude was lower, which was sustained for at least 3 months. Placebo effect of rTMS on post-stroke motor recovery was medium but significant. Regarding different sham styles, the number of stimulation sessions had an impact on the effect.In the suboptimal-choice task, birds systematically choose the leaner but informative option (suboptimal) over the richer but non-informative option (optimal). The task has two variations. In the standard task, the optimal option includes two terminal link stimuli. In the original task, it includes a single terminal link stimulus. Two models, the temporal information account (Cunningham and Shahan, J Exp Psychol Anim Learn Cogn 441-22, 2018) and the ∆-∑ hypothesis (González et al., J Exp Anal Behav 113591-608, 2020), presuppose that these procedures are equivalent, but no formal comparison is available. Here we test whether or not these procedures are functionally equivalent. One group of pigeons was trained with the standard procedure, another group with the original procedure, and a third group was trained with a hybrid of the other two (i.e., the two options were the optimal links of the standard and original procedures). Our findings indicate that the number of terminal link stimuli in the optimal option is inconsequential vis-à-vis choice. Moreover, our findings also indicate that latencies to respond are a sensitive metric of value and choice. As predicted by the Sequential Choice Model, we were able to predict simultaneous choices from the latencies of sequential choices and observed a substantial shortening of latencies during simultaneous choices.
The purpose of this review is to provide an update on the data regarding periodontitis and its association with cardiovascular disease (CVD), as well as a closer look at the connection between periodontitis and valvular heart disease.

The body of literature that demonstrates an association between periodontitis and CVD is growing, and there is increasing evidence that periodontal disease (PD) can have negative cardiovascular effects. Far less data is available for the management of periodontitis specifically in those with valvular heart disease. However, recent studies suggest that routine preoperative dental evaluation practices may not be necessary in all patients and, similar to changes in antibiotic prophylaxis, may only be indicated for a select group of individuals. There is a strong association between PD and CVD, although a causal relationship is yet to be elucidated. Further data is needed in this regard, as well as in determining the appropriate management of PD in those with valvular heart disease.
The body of literature that demonstrates an association between periodontitis and CVD is growing, and there is increasing evidence that periodontal disease (PD) can have negative cardiovascular effects. Far less data is available for the management of periodontitis specifically in those with valvular heart disease. However, recent studies suggest that routine preoperative dental evaluation practices may not be necessary in all patients and, similar to changes in antibiotic prophylaxis, may only be indicated for a select group of individuals. There is a strong association between PD and CVD, although a causal relationship is yet to be elucidated. Further data is needed in this regard, as well as in determining the appropriate management of PD in those with valvular heart disease.The healthy context paradox-an unexpected pattern in which victims' psychological adjustment worsens as the overall level of victimization in a classroom or school declines-implies that reducing the frequency of bullying or victimization incidents does not do enough to help victims of bullying. In light of this finding, it is imperative to identify protective factors that alleviate victimization-related distress in the peer ecology. The current study examines classroom-level peer victimization and peer-defending behaviors as moderators of the association between individual-level victimization and psychological adjustment. These classroom-level moderators were tested with a sample of 1373 adolescents (40% girls, Mage 14 years) from 54 classrooms in South Korean middle schools. Consistent with past findings documenting the healthy context paradox, the results of multilevel modeling indicated that victimized youth experienced a lower level of depressive symptoms in classrooms where victimization was more common. Most importantly, bullied students reported fewer depressive symptoms, on average, in classrooms with relatively high levels of bully-oriented (i.e., confronting the bully), rather than victim-oriented (i.e., comforting the victim), defending behavior. link3 These findings provide a more nuanced understanding of the role of peers' defending behaviors toward bullied adolescents and have significant implications for anti-bullying interventions.
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