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mplication rate. With thorough preoperative planning and meticulous technique, performing this procedure in carefully selected patients may be associated with acceptable PROMs.

Dystonia in pantothenate kinase-associated neurodegeneration (PKAN) is progressive despite medication. Deep brain stimulation (DBS) was reported to effectively provide symptom relief. No consensus exists in candidate and target selection for DBS. We aimed to demonstrate effectiveness of subthalamic DBS (STN-DBS) placement in pediatric patients with PKAN.

We reviewed consecutive series of pediatric patients diagnosed with PKAN and treated with STN-DBS from 2016 to 2019 in our institution. Each case was described in detail. Preoperative and postoperative Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) were assessed to evaluate functional improvement at follow-up.

Seven pediatric patients were included. Mean age of initial onset was 0.6 ± 0.5 years and presentation to clinics was 6.6 ± 1.3 years. Mean preoperative BFMDRS was 73.3 ± 3.5. Following STN-DBS, for mean follow-up duration of 13.0 ± 10.7 months, mean BFMDRS was 37.3 ± 12.6, translating to score improvement of 36.0 ± 12.9 (P<0.001), and percentage improvement of 49.0 ± 18.0%.

This case series demonstrated that STN-DBS is an effective symptom-based treatment for pediatric patients with PKAN.

This case series demonstrated that STN-DBS is an effective symptom-based treatment for pediatric patients with PKAN.

The introduction of carotid stenting (CAS) has led to numerous comparative trials with carotid endarterectomy (CEA).

The objective of the study was to review real-world volumes, outcomes, and complications following CEA versus CAS over an extended period to identify durable changes in practice.

Data were extracted from the National Inpatient Sample. Trends were assessed by annual percent change (APC), and adjusted risk ratios were calculated across the last 5years of the study period.

During 1997-2015, 199,330 symptomatic and 1,995,637 asymptomatic patients underwent carotid revascularization. In symptomatic patients, CEA declined (1997-2004; APC=-7.68%, P < 0.001) and CAS rose (1997-2008; APC= 15.48%, P < 0.001) during the first decade, subsequently becoming more muted. In asymptomatic patients, CEA decreased, whereas CAS initially increased (1997-2006; APC= 20.27%, P < 0.001) and then decreased (2007-2015; APC=-4.52%, P < 0.001). Routine discharge after symptomatic revascularization decltinue to favor CEA in real-world practice.People deceive for different reasons, from avoiding interpersonal conflicts to preserving, protecting, and nurturing interpersonal relationships, and to obtaining social status and power. A growing body of research highlights the role of personality in both deception detection and production, with a particular focus on high Dark Triad (DT) traits (Narcissism, Machiavellianism and Psychopathy), for their shared tendency to engage in unethical self-benefitting behaviors, despite negative consequences for others. The main goal of the current scoping review was to bring together the studies investigating self-reported and performance-based deception production and detection performances, as presented in individuals characterized by high DT traits and point out the possible contribution of DT to deception research. To do so, we identified the relevant studies documenting the similarities and discrepancies between the three personality traits and presented their results, based on the procedure used for deception assessment subjective or objective measurements for production / detection. Then, we discussed possible explanatory mechanisms for inter-individual differences in lie detection / production and argue for the contribution of DT to deception research beyond the typical personality models, particularly for the antisocial character of deception.Narrative coherence reflects parents' ability to provide a believable, clear, relevant, and internally consistent story about their child. Parents demonstrating more narrative coherence have been theorized to show higher parental sensitivity, but this has not been examined in a normative sample, nor across the transition to parenthood, and only once in fathers. The aim of this study was to examine stability and change in narrative coherence across the transition to parenthood in mothers and fathers, as well as the relation between pre- and postnatal narrative coherence and postnatal parental sensitivity. The sample consisted of 105 primiparous expecting parents. Narrative coherence was measured at 36-weeks pregnancy and when the child was 4 months old, using the Five Minute Speech Sample procedure. Parental sensitivity was observed in three episodes. Results demonstrated that narrative coherence was moderately stable (correlations) across the transition to parenthood in fathers only. Both mothers' and fathers' narrative coherence improved over time. Furthermore, mothers and fathers were overall equally coherent, and maternal and paternal narrative coherence were positively interrelated during pregnancy only. Lastly, our findings showed weak evidence for the theorized link between narrative coherence and parental sensitivity only postnatal narrative coherence predicted paternal sensitivity, only during free play. Our findings give new insight in the development of narrative coherence across the transition to parenthood, and how it relates to actual parenting. More research is needed to confirm our findings and further explore this topic.Previous research has found that the better-than-average effect exists in moral issues specifically, people believe that they are more moral than others. The present study demonstrated the better-than-average effect in judging moral and immoral behaviors in oneself. In Study 1 (N = 233), we found that the better-than-average effect is observed in estimating the intention of immoral behaviors in oneself, which means that people are likely to perceive themselves having less intention to conduct immoral behaviors than others. In Study 2 (N = 52), we confirmed the better-than-average effect in the estimation of their intention both on moral and immoral issues. selleck chemicals In Study 3 (N = 153), we manipulated actors (self vs. others) and the level of morality (very immoral, immoral, moral, or very moral) and investigated the interaction between the actors and the level of morality on two types of causal attribution (i.e., dispositional or situational attribution). We found that when participants were asked to imagine having engaged in immoral behaviors, they made less dispositional attributions. In contrast, when participants were asked to imagine having in moral behaviors, they made less situational attributions. Our results showed that a systematic asymmetry of causal attribution resulted in the better-than-average effect, which varied with levels of morality.This study investigates the patterns of relations between beliefs, emotions, and job satisfaction in 249 Italian in-service teachers. Participants were assessed on their growth and fixed mindsets, self-efficacy beliefs, emotions associated with various components of their professional engagement and job satisfaction. Mediational analyses shed light on the mediating role of teaching and role emotions in the relation between beliefs and job satisfaction. Specifically, teachers' high self-efficacy beliefs positively impact on job satisfaction if negative teaching and role emotions are contained at low levels.Lynch Syndrome is characterized by phenotypic and genotypic heterogeneity. Despite scarce evidence, individuals with an EPCAM deletion appear to have a comparable risk of colorectal cancer (CRC) as MSH2 mutation carriers, but a lower risk of extracolonic cancer (such as endometrial cancer) unless the deletion extends close to the promoter of MSH2. A genotype-phenotype correlation is yet to be established for EPCAM alterations. In this report, we describe a family with EPCAM deletion characterized by a particularly aggressive phenotype and extracolonic cancer. We present a family with 5 members carrying an EPCAM deletion encompassing exons 8 and 9. Three female family members presented CRC at the ages of 32, 44 and 60 (mucinous moderately and well-differentiated adenocarcinoma); in two of them metachronous colon cancers and advanced adenomas were diagnosed in the intensive surveillance program. Two female patients (42 and 63 years-old) presented with gastric cancer (GC). Two patients presented with small bowel cancer at 51 and 60 years-old - the first one presented a metachronous jejunal cancer at 68 years. Only one family member was submitted to hemithyroidectomy due a right-lobe Hürthle cell carcinoma at 56 years-old. This report illustrates the existence of intrafamilial clinical heterogeneity among carriers of this EPCAM alteration, and hence the difficulty in predicting phenotype for EPCAM-associated Lynch syndrome.GRIN2A encodes for the 2A subunit of N-methyl-D-aspartate receptors. Pathogenic variants in GRIN2A have been associated with a wide spectrum of neurodevelopmental disorders ranging from speech disorders and/or self-limiting epilepsy (childhood epilepsy with centrotemporal spikes) to severe and disabling phenotypes (atypical childhood epilepsy with centrotemporal spikes, epileptic encephalopathy with continuous spike-wave during sleep, Landau-Kleffner syndrome and infantile-onset epileptic encephalopathy). Here we describe a family with two affected sisters with atypical childhood epilepsy with centrotemporal spikes and their mildly affected mother carrying a novel N-terminal null variant in GRIN2A gene. These familial cases corroborate previous studies showing that loss-of-function GRIN2A variants are associated with milder phenotypes, possibly due to haploinsufficiency.

Arginine metabolites are associated with cardiovascular and all-cause mortality in several patient groups. We investigated whether arginine metabolites are associated with mortality in patients with critical illness and whether associations are independent of other factors affecting prognosis in an Intensive Care Unit (ICU).

1155 acutely unwell adult patients admitted to a mixed medical-surgical ICU were studied. Arginine, asymmetric dimethyl-l-arginine (ADMA), monomethyl-l-arginine (MMA), symmetric dimethyl-l-arginine (SDMA) and l-homoarginine were measured in a plasma sample collected at admission to ICU by liquid chromatography tandem mass spectrometry. Risk of death score was calculated using data submitted to the Australia and New Zealand Intensive Care Society.

In this cohort, 163 patients (14.1%) died. ADMA (odds ratio=1.159 (1.033-1.300) per 0.1μmol/L increment, p=0.012), homoarginine (odds ratio=0.963 (0.934-0.992), p=0.013) and risk of death score (odds ratio=1.045 (1.037-1.053) per 1% incremetality in critically ill patients.Solid-tumor cell invasion typically occurs by collective migration of attached cell-cohorts, yet we show here that indirect cell-interactions through the substrate can also drive invasiveness. We have previously shown that well-spaced, invasive cancer cells push-into and indent gels to depths of 10 µm, while closely adjacent, non-contacting cancer cells may reach up to 18 µm, potentially relying on cell-cell interactions through the gel-substrate. To test that, we developed finite element models of indenting cells, using experimental gel mechanics, cell mechanostructure, and force magnitudes. We show that under 50-350 nN of combined traction and normal forces, a stiff nucleus-region is essential in facilitating 5-10 µm single-cell indentations, while uniformly soft cells attain 1.6-fold smaller indentations. We observe that indentation depths of cells in close proximity (0.5-50 µm distance) increase relative to well-spaced cells, due to additive, continuum mechanics-driven contributions. Specifically, 2-3 cells applying 220 nN normal forces gained up to 3% in depth, which interestingly increased to 7.