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Neuronal ceroid lipofuscinosis type 2 (CLN2) disease is a rare, lysosomal storage disorder that causes pediatric onset neurodegenerative disease. It is characterized by mutations in the TPP1 gene. Symptoms begin between 2 and 4 years of age with loss of previously acquired motor, cognitive, and language abilities. Cerliponase alfa, a recombinant human TPP1 enzyme, is the only approved therapy. We report the first presymptomatic cerliponase alfa intraventricular treatment in a familial case of CLN2 related to a classical TPP1 variant. Sister 1 presented with motor, cognitive, and language decline and progressive myoclonic epilepsy since the age of 3 years, evolved with severe diffuse encephalopathy, received no specific treatment, and died at 11 years. Sister 2 had a CLN2 presymptomatic diagnosis and has been treated with cerliponase since she was 12 months old. She is now 6 years 8 months and has no CLN2 symptom except one generalized seizure 1 year ago. No serious adverse event has occurred. Repeated Wechsler Preschool and Primary Scale of Intelligence, Fourth Edition standardized index scores are heterogeneous in the extremely low to low average ranges. Mean length of utterances, a global index of sentence complexity, showed a delay, but a gradual improvement. The reported case enhances the major contribution of presymptomatic diagnosis and significant middle‐term treatment benefit for patients with CLN2. [ABSTRACT FROM AUTHOR]

We document and discuss the implications of a striking feature of modern American policing: the stasis of police labor forces. Using an original employment dataset assembled through public records requests, we show that, after the first few years on a job, officers rarely change employers, and intermediate officer ranks are filled almost exclusively through promotion rather than lateral hiring. Policing is like a sports league, if you removed trades and free agency and left only the draft in place. We identify both nonlegal and legal causes of this phenomenon--ranging from geographic monopolies to statutory and collectively bargained rules about pensions, rank, and seniority--and discuss its normative implications. On the one hand, job stability may encourage investment in training and expertise by agencies and officers alike; it may also attract some high-quality candidates, including candidates from underrepresented backgrounds, to the profession. On the other hand, low labor mobility can foster sclerosis in police departments, entrenching old ways of policing. Limited outside options may lead officers to stay in positions that suit them poorly, decreasing morale and productivity and potentially contributing to the scale of policing harms. In turn, the lack of labor mobility makes it all the more important to police officers to retain the jobs they have. This encourages them to insist on extensive labor protections and to enforce norms like the "blue wall of silence," which exacerbate the problem of police misconduct. We suggest reforms designed to confer the advantages of labor mobility while ameliorating its costs. [ABSTRACT FROM AUTHOR]

Aims Pulmonary vein isolation (PVI) is a well-established strategy for the treatment of paroxysmal atrial fibrillation (PAF). Despite randomized controlled trials and real-world data showing the promise of pulsed-field ablation (PFA) for this treatment, long-term efficacy and safety data demonstrating single-procedure outcomes off antiarrhythmic drugs remain limited. The aim of the FARA-Freedom Study was to evaluate the long-term efficacy and safety of PFA using the pentaspline catheter for PAF. Methods and results FARA-Freedom, a prospective, non-randomized, multicentre study, enrolled patients with PAF undergoing de novo PVI with PFA, who were followed for 12 months with weekly transtelephonic monitoring and a 72-h Holter ECG at 6 and 12 months. The primary safety endpoint was a composite of device- or procedure-related serious adverse events out to 7 days post-ablation and PV stenosis or atrioesophageal (AE) fistula out to 12 months. Treatment success is a composite of acute PVI and chronic success, which includes freedom from any documented atrial tachyarrhythmia longer than 30 s, use of antiarrhythmic drugs or cardioversion after a 3-month blanking period, or use of amiodarone or repeat ablation at any time. The study enrolled 179 PAF patients (62 ± 10 years, 39% female) at 13 centres. At the index procedure, all PVs were successfully isolated with the pentaspline PFA catheter. Procedure and left atrial dwell times, with a 20-min waiting period, were 71.9 ± 17.6 and 41.0 ± 13.3 min, respectively. Fluoroscopy time was 11.5 ± 7.4 min. Notably, monitoring compliance was high, with 88.4 and 90.3% with weekly events and 72-h Holter monitors, respectively. Freedom from the composite primary effectiveness endpoint was 66.6%, and 41 patients had atrial tachyarrhythmia recurrence, mostly recurrent atrial fibrillation (31 patients). The composite safety endpoint occurred in two patients (1.1%), one tamponade and one transient ischaemic attack. There was no coronary spasm, PV stenosis, or AE fistula. There were four cases of transient phrenic nerve palsy, but all resolved during the index procedure. Conclusion In this prospective, non-randomized, multicentre study, PVI using a pentaspline PFA catheter was effective in treating PAF patients despite rigourous endpoint definitions and high monitoring compliance and demonstrated favourable safety. Registration Clinical Trials.gov Identifier: NCT05072964 (sponsor: Boston Scientific Corporation). [ABSTRACT FROM AUTHOR]

Current psychopathology models have evolved toward dimensional models, in which symptoms and diseases are at the extremes of dimensions. Despite these new dimensional proposals, classifications and third-person approach have shown limitations. Their extraordinary evolution nevertheless underlines the contributions of developmental and psychodynamic frameworks. Developmental contributions have made it possible to evolve from disorders centered on a first-person perspective. Complementarily to the first-person/third-person perspectives, we advocate a second-person perspective, based on intersubjectivity. This perspective reverses the intuitive trend to focus our interventions on the most specific symptoms and syndromes, and advocates instead interventions on a "p" general factor that are both generalized and highly targeted. The implications are (1) to intervene as early as possible, (2) to base the definition of our therapeutic targets on an intersubjective perspective, (3) to identify and enhance children's and parents' strengths. These empirically informed directions are not in the current mainstream of psychopathology frameworks, and need to be developed. [ABSTRACT FROM AUTHOR]

Aims Pulsed field ablation (PFA) is a new, non-thermal ablation modality for pulmonary vein (PV) isolation in patients with atrial fibrillation (AF). The multi-centre EUropean Real World Outcomes with Pulsed Field AblatiOn in Patients with Symptomatic AtRIAl Fibrillation (EU-PORIA) registry sought to determine the safety, efficacy, and learning curve characteristics for the pentaspline, multi-electrode PFA catheter. Methods and results All-comer AF patients from seven high-volume centres were consecutively enrolled. Procedural and follow-up data were collected. Learning curve effects were analysed by operator ablation experience and primary ablation modality. In total, 1233 patients (61% male, mean age 66 ± 11years, 60% paroxysmal AF) were treated by 42 operators. In 169 patients (14%), additional lesions outside the PVs were performed, most commonly at the posterior wall (n = 127). Median procedure and fluoroscopy times were 58 (interquartile range: 40–87) and 14 (9–21) min, respectively, with no differences due to operator experience. Major complications occurred in 21/1233 procedures (1.7%) including pericardial tamponade (14; 1.1%) and transient ischaemic attack or stroke (n = 7; 0.6%), of which one was fatal. Prior cryoballoon users had less complication. At a median follow-up of 365 (323–386) days, the Kaplan–Meier estimate of arrhythmia-free survival was 74% (80% for paroxysmal and 66% for persistent AF). Freedom from arrhythmia was not influenced by operator experience. In 149 (12%) patients, a repeat procedure was performed due to AF recurrence and 418/584 (72%) PVs were durably isolated. Conclusion The EU-PORIA registry demonstrates a high single-procedure success rate with an excellent safety profile and short procedure times in a real-world, all-comer AF patient population. [ABSTRACT FROM AUTHOR]

Objective: To study longitudinal changes in tuber and whole-brain perfusion in children with tuberous sclerosis complex (TSC) using arterial spin labeling (ASL) perfusion MRI and correlate them with pathological EEG slow wave activity and neurodevelopmental outcomes. Methods: Retrospective longitudinal cohort study of 13 children with TSC, 3 to 6 serial ASL-MRI scans between 2 months and 7 years of age (53 scans in total), and an EEG examination performed within 2 months of the last MRI. Tuber cerebral blood flow (CBF) values were calculated in tuber segmentation masks, and tuber:cortical CBF ratios were used to study tuber perfusion. Logistic regression analysis was performed to identify which initial tuber characteristics (CBF value, volume, location) in the first MRI predicted tubers subsequently associated with EEG slow waves. Whole-brain and lobar CBF values were extracted for all patient scans and age-matched controls. CBF ratios were compared in patients and controls to study longitudinal changes in whole-brain CBF. Results: Perfusion was reduced in tubers associated with EEG slow waves compared with other tubers. Low tuber CBF values around 6 months of age and large tuber volumes were predictive of tubers subsequently associated with EEG slow waves. Patients with severe developmental delay had more severe whole-brain hypoperfusion than those with no/mild delay, which became apparent after 2 years of age and were not associated with a higher tuber load. Conclusions: Dynamic changes in tuber and brain perfusion occur over time. Perfusion is significantly reduced in tubers associated with EEG slow waves. Whole-brain perfusion is significantly reduced in patients with severe delay. Key Points: • Tubers associated with EEG slow wave activity were significantly more hypoperfused than other tubers, especially after 1 year of age. • Larger and more hypoperfused tubers at 6 months of age were more likely to subsequently be associated with pathological EEG slow wave activity. • Patients with severe developmental delay had more extensive and severe global hypoperfusion than those without developmental delay. [ABSTRACT FROM AUTHOR]

Background: Recently, a novel cryoballoon ablation catheter has demonstrated acute safety and efficacy in de novo pulmonary vein isolation (PVI) procedures in patients with paroxysmal atrial fibrillation (PAF). However, there are limited studies demonstrating the long-term efficacy. The aim of this study was to evaluate the long-term safety and efficacy of this novel cryoballoon in treating PAF. Methods: This was a non-randomized, prospective, multicentre study enrolling 58 consecutive patients. Cryoablation was delivered for 180 s if time to isolation was ≤ 60 s. Otherwise a 240-s cryoablation was performed. One centre performed pre- and post-ablation high-density mapping (n = 9) to characterize lesion formation. After a 3-month blanking period, recurrence was defined as having any documented, symptomatic episode(s) of AF or atrial tachycardia. All patients were followed for 1 year. Results: Acute PVI was achieved in 230 of 231 pulmonary veins (99.6%) with 5.3 ± 1.6 cryoablations per patient (1.3 ± 0.7 cryoablations per vein). Forty-three (77%) patients remained arrhythmia-free at 1-year follow-up. Four patients (6.9%) experienced phrenic nerve injury (3 resolved during the index procedure; 1 resolved at 6 months). One serious adverse device event was reported: femoral arterial embolism event occurring 2 weeks post-index procedure. For patients who underwent high-density mapping, cryoablation was antral with 50% of the posterior wall ablated. Conclusions: Initial multicentre clinical experience with a novel cryoballoon has demonstrated safety and efficacy of PVI in patients with PAF. Ablation with this cryoballoon provides a wide, antral lesion set with significant debulking of the posterior wall of the left atrium. [ABSTRACT FROM AUTHOR]

Maternal input addressed to children after an early hospitalization (EH) was longitudinally compared to maternal input directed to typically developing children (TD), at 6, 9, 12, 18 and 24 months of age. The data were analyzed with the CHILDES tools for (a) word-tokens (b) word-types (c) Mean Length of Utterances (MLU) and (d) questions in which the mothers request a response or a turn. Nonsignificant differences between groups were found. Only questions were significantly higher for EH mothers than for TD mothers across the five sessions. Lexical Correspondence Analysis (LCA) on these two datasets show qualitative differences between the two groups. Depression and anxiety at 12 months and children attachment behaviour assessed at 18 months do not play a role from EH mothers. These findings suggest that EH mothers have a specific conversational style encouraging their infants and toddlers to request salient information or to perform an action. [ABSTRACT FROM AUTHOR]

Aim: The aim of the study was to assess the emotional and behavioural functioning of siblings of children treated with long term non‐invasive ventilation (NIV). Methods: Parents of children treated with NIV completed the Child Behaviour Checklist and a qualitative questionnaire for each sibling, aged 1.5–18 years old. Results: The parents of 49 ventilated children were questioned about 79 siblings. For the siblings aged 1.5–5, mean total T score was 57 ± 22 (range 28–92), and five siblings (31%) were in the clinical range. For the siblings aged 6–18, mean total T score was 49 ± 12 (range 26–71), and six siblings (10%) were in the clinical range. Siblings felt responsible for their affected sibling (31%) and involved with his/her illness (52%), with 31% being worried about him/her. A change in behaviour was observed in 19% of the siblings since the initiation of NIV; 26% were impacted by the use of the NIV device. Conclusions: The majority of siblings of children treated with NIV do not present significant emotional and behavioural problems. They feel deeply responsible for their affected sibling and involved in his/her illness and treatment, highlighting the importance to involve the siblings in the care of the affected child. [ABSTRACT FROM AUTHOR]

Objectives: When for an RCT heterogeneous treatment effects are inductively obtained, significant complications are introduced. Special loss functions may be needed to find local, average treatment effects followed by techniques that properly address post-selection statistical inference. Methods: Reanalyzing a recidivism RCT, we use a new form of classification trees to seek heterogeneous treatment effects and then correct for "data snooping" with novel inferential procedures. Results: There are perhaps increases in recidivism for a small subset of offenders whose risk factors place them toward the right tail of the risk distribution. Conclusions: A legitimate but partial account for uncertainty might well reject the null hypothesis of no heterogenous treatment effects. An equally legitimate but far more complete account of uncertainty for this study fails to reject the null hypothesis of no heterogeneous treatment effects. [ABSTRACT FROM AUTHOR]

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Aims: Pulmonary vein isolation (PVI) has become a cornerstone of the invasive treatment of atrial fibrillation. Severe complications are reported in 1-3% of patients. This study aims to compare complications and follow-up outcome of PVI in patients with atrial fibrillation.Methods and Results: The data were extracted from the Netherlands Heart Registration. Procedural and follow-up outcomes in patients treated with conventional radiofrequency (C-RF), multielectrode phased RF (Ph-RF), or cryoballoon (CB) ablation from 2012 to 2017 were compared. Subgroup analysis was performed to identify variables associated with complications and repeat ablations. In total, 13 823 patients (69% male) were included. The reported complication incidence was 3.6%. Patients treated with C-RF developed more cardiac tamponades (C-RF 0.8% vs. Ph-RF 0.3% vs. CB 0.3%, P ≤ 0.001) and vascular complications (C-RF 1.7% vs. Ph-RF 1.2% vs. CB 1.3%, P ≤ 0.001). Ph-RF was associated with fewer bleeding complications (C-RF: 1.0% vs. Ph-RF: 0.4% vs. CB: 0.7%, P = 0.020). Phrenic nerve palsy mainly occurred in patients treated with CB (C-RF: 0.1% vs. Ph-RF: 0.2% vs. CB: 1.5%, P ≤ 0.001). In total, 18.4% of patients were referred for repeat ablation within 1 year. Female sex, age, and CHA2DS2-VASc were independent risk factors for cardiac tamponade and bleeding complications, with an adjusted OR for female patients of 2.97 (95% CI 1.98-4.45) and 2.02 (95% CI 1.03-4.00) respectively.Conclusion: The reported complication rate during PVI was low. Patients treated with C-RF ablation were more likely to develop cardiac tamponades and vascular complications. Female sex was associated with more cardiac tamponade and bleeding complications. [ABSTRACT FROM AUTHOR]

We study how local labour market conditions and information about job availability affect recidivism after incarceration. We exploit daily variations in the quality of the labour market at the time of release from prison. We combine individual-level administrative data on former inmates in France to county-level daily data on new job vacancies, and on newspaper coverage of job creation and destruction. Our analysis provides two new findings. First, media coverage of job creation reduces recidivism, suggesting that policies promoting access to information about employment opportunities can contribute to reducing recidivism. Second, we show that there is heterogeneity in what kinds of jobs affect recidivism: in France, former inmates do not respond to overall job creation, but better opportunities in manufacturing jobs at release reduce recidivism rates. [ABSTRACT FROM AUTHOR]

The objective of the study was to examine the process of mother to infant trauma transmission among traumatized mothers in humanitarian contexts. We investigated the impact of mothers' post-traumatic stress disorder symptoms on the quality of the dyadic interaction by conducting a microanalysis of mother-infant interactions at specific moments when trauma was recalled, compared to more neutral moments. Twenty-four mother-infant dyadic interactions of traumatized mothers and children aged from 1.5 to 30 months Central Africa, Chad, and Cameroon were videotaped during three sequences: a neutral initial session (baseline) exploring mothers' representations of the infant and of their bonding; a second sequence, "the traumatic narration," in which mothers were asked to talk about the difficult events they had experienced; and a third sequence focusing on a neutral subject. Three minutes of each sequence were coded through a specific grid for microanalysis [based on the scales developed at Bobigny Faculty of Medicine and the work of (1)], according to different communication modalities (touch, visual, and vocal), for both the mother and the child. Impact of traumatic event (IES-R), the level of depression and anxiety (HAD) were investigated in order to have a holistic understanding of the trauma transmission mechanism. The data analysis highlighted significant differences in mothers, children and their interaction during the "traumatic narration": mothers touched and looked at the infant less, looked more absent and smiled less, and looked less at the interviewer; infants looked less at the interviewer, and sucked the breast more. The mother-child interaction "infant self-stimulation—mother looks absent" and "Infant sucks the breast—mother looks absent" occurred more often during the mothers' traumatic narrations. The "absence" of the mother during trauma recall seems to have repercussions on infants' behavior and interaction; infants show coping strategies that are discussed. We found no significant associations between interaction and infant gender and age, the severity of traumatic experience, mothers' depression and anxiety symptoms, and the country of residence. The results of the microanalysis of interaction can shed light on the fundamental role of intermodal exchanges between mother and infant in trauma transmission during mothers' trauma reactivation. [ABSTRACT FROM AUTHOR]

Aim: To examine how children with sleep-disordered breathing express their own sleep through drawing.Methods: Children hospitalised for a sleep study in a sleep laboratory of a tertiary hospital were asked to draw a human figure and themselves while asleep. Characteristics of the two drawings were analysed and compared along with a descriptive analysis of some drawings.Results: Children with sleep-disordered breathing and an associated disorder, n = 34, age 5-11 years, participated in the study. The size of the human figure, the colours used, the orientation of the sheet, the type of drawing strokes and the objective quality of the drawing were comparable between the two drawings. On the sleep drawing, 71% of the children drew a bed, 15% drew themselves asleep, 19% represented snoring and 12% night elements. Sixty-two per cent of the children preferred the human drawing to the sleep drawing. A descriptive analysis of 12 drawings showed the influence of the associated disorder on the two drawings.Conclusion: This study showed how the associated disease of children with sleep-disordered breathing infiltrated their imaginary life. The sleep drawing gave useful information about representation, fears and wishes in relation to the associated disease and the child's sleeping. [ABSTRACT FROM AUTHOR]

This paper aims at the detection and characterization of partial discharges (PD) in GIS under HVDC constraints in the presence of a protrusion/metallic particle on the high voltage conductor. It is shown that the use of the IEC 60270 standard does not allow for the detection of PDs. For that purpose, a very accurate steady-state current measurement and a high frequency measurement of the current and emitted light associated with the PDs are performed. The results evidence a close correlation between the PD and the steady-state current and light emission. The transition between pulse and pulseless forms of corona discharges is also evidenced. [ABSTRACT FROM AUTHOR]

36779624 3 Younis A, Zilberman I, Krywanczyk A. Effect of pulsed-field and radiofrequency ablation on heterogeneous ventricular scar in a swine model of healed myocardial infarction. D Activation map of the left ventricle after seven overlapping PFA applications showing the absence of isochronal crowding This is the first description of successful ablation with PFA for scar-related VT. [Extracted from the article]

Summary: Objective: We aimed to assess a cohort of young patients with Dravet syndrome (DS) for intellectual disability (ID) and autism spectrum disorder (ASD) using standardized tools and parental questionnaires to delineate their specific profiles. Methods: We included 35 patients with DS aged 24 months to 7 years, excluding patients with a developmental age (DA) <18 months (n = 5). We performed specific tests adapted for ID (Psychoeducational Profile, Third Edition [PEP‐3]), in addition to the Child Development Inventory (CDI) and Vineland Adaptive Behavior Scales, Second Edition (VABS‐II) questionnaires. We used 2 standardized tools for ASD: the Autism Diagnostic Observation Schedule, Second Edition (ADOS‐2) and the Autism Diagnostic Interview‐Revised (ADI‐R). We compared the with parental questionnaires and the VABS‐II, and with ASD characteristics. Results: PEP‐3 subscales showed pathologic development in all but one patient (97%): ID in 23 of 30 (77%), and borderline cognitive functioning in 6 of 30 (22%). Eleven patients (39%) had ASD and 2 (7%) had a Social Communication Disorder (SCD) diagnosis. We found no difference between PEP‐3 and CDI categorization except for fine motor skills. We found significant negative correlations between ADOS‐2 and PEP‐3 for the majority of scores. For patients aged older than 50 months, 2 groups emerged (ASD/no ASD) with significant difference in DA. The logistic regression for ASD diagnosis explained by VABS‐II showed a significant effect for Socialization, Motor Skills, and Adaptive Behavior. Significance: We found a high prevalence of ID in patients with DS. ID is characterized by expressive and comprehensive communication deficits in addition to visuospatial difficulties. ASD showed a specific profile with a relative preservation of social skills, emphasizing a possible underdiagnosis. Parental questionnaires can provide a good assessment of cognitive profile and might allow the difficulty of addressing cognitive scales in DS to be overcome. The profile of ID and ASD should help to establish early adapted rehabilitation programs and emphasizes the global need for care beyond seizures in DS and other developmental epileptic encephalopathies. [ABSTRACT FROM AUTHOR]

We explore how television broadcasting of unrelated criminal justice events affects sentencing. Exploiting as-good-as-random variation in news content before a verdict, we find that sentences are 3 months longer when the verdict is reached after coverage of crime. Sentences increase with media exposure to crime, not crime itself, and the effect tapers off quickly. Our results suggest that professional experience and expertise mitigate the effect of irrelevant external information. This paper highlights the influence of noise in the news cycle: media can temporarily influence decisions by changing what is top of the mind rather than signaling deeper changes in offending or societal concerns. [ABSTRACT FROM AUTHOR]

Aim: We evaluated the impact that having a child with sleep-disordered breathing had on their parents, including their own sleep quality.Methods: Questionnaires were completed by 96 parents of 86 children referred for a sleep study or control of continuous positive airway pressure (CPAP) or noninvasive ventilation (NIV) at the sleep laboratory of the Necker Hospital, Paris, France, between October 2015 and January 2016. The questionnaires evaluated anxiety and depression, family functioning, the parents' quality of life, daytime sleepiness and sleep quality.Results: The children had a mean age of seven ±five years and most of the responses (79%) came from their mothers. These showed that 26% of parents showed moderate-to-severe anxiety, 8% moderate-to-severe depression, 6% complex family cohesion, 59% moderate-to-severe daytime sleepiness and 54% poor sleep quality. Anxiety was higher in mothers than in fathers (p < 0.001). The questionnaire scores did not differ according to the child's age, the results of the sleep studies or the CPAP or NIV treatment. The symptoms seem to be more commonly related to the child's underlying disease than their sleep-disordered breathing.Conclusion: The parents of children referred to a sleep laboratory reported frequent anxiety, daytime sleepiness and poor sleep quality. [ABSTRACT FROM AUTHOR]

Propionic acidemia is the result of a deficiency in propionyl-CoA carboxylase activity. Chronic neurologic and cognitive complications frequently occur, but the psychiatric evolution of the disorder is not well documented. We conducted a pedopsychiatric evaluation of 19 children, adolescents and young adults, aged between 2 and 25 years, using ADI-R, CARS-T, as well as ADOS when autism spectrum disorder was suspected. Previous psychometric examinations were also taken into consideration. Thirteen patients had an IQ < 80. Two patients presented with autism and two additional patients with other autism spectrum disorders. Five patients did not fulfill diagnostic criteria for autism spectrum disorder but showed difficulties indicative of a broader autism phenotype (BAP). Four other patients had severe anxiety manifestations related to their disease. Two patients presented with acute psychotic episodes. The number of decompensations in the first 3 years of life was lower in patients with autism spectrum disorder or related symptoms. These patients were also older when they were assessed (median age of 15 years old versus 11 years old). There was no significant correlation between 3-hydroxypropionate levels during the first 6 years of life and autism spectrum disorder diagnosis. In conclusion, autism spectrum disorder is frequent in patients with propionic acidemia. These patients should undergo in-depth psychiatric evaluation and be screened for autism spectrum disorder. Further studies are needed to understand the underlying mechanisms. [ABSTRACT FROM AUTHOR]

Highlights The kinematics of hand movements (spatial use, curvature, acceleration, and velocity) of infants with their mothers in an interactive setting are significantly associated with age in cohorts of typical and at-risk infants differ significantly at 5-6 months of age, depending on the context: relating either with an object or a person. Environmental and developmental factors shape the developmental trajectories of hand movements in different cohorts: environment for infants with VIMs; stage of development for premature infants and those with West syndrome; and both factors for infants with orality disorders. The curvature of hand movements specifically reflects atypical development in infants with West syndrome when developmental age is considered. We aimed to discriminate between typical and atypical developmental trajectory patterns of at-risk infants in an interactive setting in this observational and longitudinal study, with the assumption that hand movements (HM) reflect preverbal communication and its disorders. We examined the developmental trajectories of HM in five cohorts of at-risk infants and one control cohort, followed from ages 2 to 10 months: 25 West syndrome (WS), 13 preterm birth (PB), 16 orality disorder (OD), 14 with visually impaired mothers (VIM), 7 early hospitalization (EH), and 19 typically developing infants (TD). Video-recorded data were collected in three different structured interactive contexts. Descriptors of the hand motion were used to examine the extent to which HM were associated with age and cohort. We obtained four principal results: (i) the kinematics of HM (spatial use, curvature, acceleration, and velocity) were significantly associated with age in all cohorts; (ii) HM significantly differed at 5-6 months of age in TD infants, depending on the context; (iii) environmental and developmental factors shaped the developmental trajectories of HM in different cohorts: environment for VIM, development for PB and WS, and both factors for OD and; (iv) the curvatures of HM showed atypical development in WS infants when developmental age was considered. These findings support the importance of using kinematics of HM to identify very early developmental disorders in an interactive context and would allow early prevention and intervention for at-risk infants. [ABSTRACT FROM AUTHOR]

This retrospective observational study conducted in Necker Hospital for Sick Children, France (January 2018-June 2020) evaluated a potential temporal association between admissions for suicide behaviours in children and adolescents and the national COVID-19 lockdown (March-May 2020). During the study period, 234 patients were admitted for suicide behaviours (28% male; mean age 13.4 years). Using Poisson regression, we found a significant decrease in the incidence of admissions for suicide behaviour during the lockdown (adjusted incidence rate ratio: 0.46; 95% CI 0.24 to 0.86). This association might result from reduced help-seeking and decreased hospital admission rates during the lockdown, as well as cognitive and environmental factors. Further multicentre studies should be conducted to confirm these findings and investigate whether a compensatory rise in admissions for suicide behaviour occurred in the postlockdown period. [ABSTRACT FROM AUTHOR]

Economic theories of punishment focus on determining the levels that provide maximal social material payoffs. In other words, these theories treat punishment as a public good. Several parameters are key to calculating optimal levels of punishment: total social costs, total social benefits, and the probability that offenders are apprehended. However, levels of punishment are often determined by aggregating individual decisions. Research in behavioral economics, psychology, and neuroscience shows that individuals appear to treat punishment as a private good (cold glow). This means that individual choices may not respond appropriately to the social parameters. We present a simple theory and show in a series of experiments that individually chosen punishment levels can be predictably too high or too low relative to those that maximize social material welfare. Our findings highlight the importance of the psychology of punishment for understanding social outcomes and for designing social institutions. [ABSTRACT FROM AUTHOR]

West syndrome (WS) is a rare epileptic encephalopathy with early onset and a high risk of autistic outcome. The PréAut grid assesses this risk following WS onset by taking into account synchrony and emotion in interactions and by evaluating the baby's active desire to engage in pleasant interactions (especially the infant's early active behaviors that encourage being gazed at or kissed by the mother or to share joy with her). We followed a sample of 25 WS patients prospectively from disease onset and assessed whether the PréAut grid before 9 months, and the checklist for autism in toddlers (CHAT) at 18 and 24 months predicted autism or intellectual disability (ID) outcomes at 4 years. We found that the PréAut grid at 9 months (sensitivity = 0.83; specificity = 1) had similar prediction parameters as the CHAT at 18 months (sensitivity = 0.90; specificity = 0.83) and 24 months (sensitivity = 0.92; specificity = 1). WS patients with a positive PréAut screening at 9 months had a risk of having autism or ID at 4 years, which is 38 times that of children with a negative PréAut grid [OR = 38.6 (95 % CI 2.2-2961); p = 0.006]. We conclude that the PréAut grid could be a useful tool for the early detection of autism or ID risk in the context of WS. Further research is needed to assess the PréAut grid in other contexts (e.g. infants at high-risk for non-syndromic autism). [ABSTRACT FROM AUTHOR]

BACKGROUND: A distinct velocity pattern represented by a "notch" is observed during the time interval between the end of the systolic and the onset of the early diastolic velocity wave on longitudinal myocardial velocity curve. The origin of the post-systolic velocity notch (PSN) has not been resolved. METHODS: The high frame rate color tissue Doppler imaging of the apical longitudinal axis was performed in 32 healthy subjects. RESULTS: The time delays of the PSN onset at the posterior aortic wall (AW), the mid anteroseptal wall (MAS) and the posterior mitral annulus (MA) relatively to the anterior aortic annulus (AA) were found to be significantly longer than zero (5.1 ± 2.2, 6.0 ± 2.3, 6.8 ± 2.8 ms;p < 0.001). The amplitude was the highest at the AA when compared to the AW, the MAS and the MA (4.77 ± 1.28 vs. 2.88 ± 1.11, 2.15 ± 0.73, 2.44 ± 1.17 cm/s; p < 0.001). A second PSN spike was identifiable in 10/32 (31%) of the studied subjects at the AA. Of these, 9 (28%) exhibited a second PSN spike at the AW, 3 (9%) at the MAS and no one at the MA. CONCLUSION: The AA represents the site of the earliest onset and maximal amplitude of the PSN on the longitudinal velocity curve suggesting its mechanism to be that of an energy release at the instant of the aortic valve closure causing an apically directed acceleration of the myocardium. A substantial number of healthy subjects exhibit a second PSN spike predominantly at the level of the AA. Its mechanism remains to be elucidated. [ABSTRACT FROM AUTHOR]

This study focused on parents’ satisfaction with the special education and care services proposed to their child with autism spectrum disorders (ASD). Data were collected in three regions of France, using a questionnaire designed for the purpose of this study. Among the 530 families contacted, 212 filled in the questionnaire (response rate = 40.8%). Results showed that parents were globally satisfied with providers’ involvement and motivation, but they felt they were not involved enough in their child’s individualized program, that communication with providers was insufficient and that the services lacked ASD’s specific tools and interventions. Among all families interviewed, parents of adolescents were the most unsatisfied and we hypothesized that this could be due to the specific issues regarding developmental changes and concern about the future at this period of life. Congruently with the literature, variables related to parental overall satisfaction were a regular communication with professionals, a specific, regularly updated individual program in which parents are associated, and specialized tools and interventions. The implications of these findings are discussed as well as future directions for clinicians to improve service delivery and allow the persons with ASD and their families to be more involved in the services. [ABSTRACT FROM PUBLISHER]

Providing abandoned children the necessary medical and psychological care as possible after their institutionalization may minimize developmental delays. We describe psychomotor development in infants admitted to an orphanage in Khartoum, Sudan, assessed at admission and over an 18-month follow-up. Psychological state and psychomotor quotients were determined using a simplified Neonatal Behavior Assessment Scale (NBAS), the Brunet–Lezine and Alarm distress baby (ADBB) scale. From May–September 2005, 151 children were evaluated 2, 4, 9, 12 and 18 months after inclusion. At admission, ∼15% of children ≤1 month had a regulation impairment according to the NBAS, and 33.8% presented a distress state (ADBB score >5). More than 85% (129/151) recovered normal psychomotor development. The results of the program reinforce the importance of early detection of psychological disorders followed by rapid implementation of psychological case management to improve the development of young children in similar institutions and circumstances. [ABSTRACT FROM PUBLISHER]

The article presents a case study of a 65 year old male patient who received a DDDR pacemaker for high grade atrioventricular block with recurrent syncope who presented to physicians with sudden onset fatigue which occurred during cycling. A discussion of an electrocardiogram which was conducted on the patient, and of factors which could have led to an apparent pacemaker malfunction in the patient, is presented.

Background: Trimethylaminuria (TMAU) is a metabolic disorder characterized by the excessive excretion of the malodorous compound trimethylamine (TMA). The diagnosis of TMAU is challenging because this disorder is situated at the boundary between biochemistry and psychiatry. Here, we used nuclear magnetic resonance spectroscopy to assess TMAU in 13 patients. We also sequenced the FMO3 gene in 11 of these patients. Treatment with vitamin B2 was prescribed.Results: Two patients (aged 3 and 9 years at the initial consultation) had a particularly unpleasant body odor, as assessed by their parents and the attending physicians. The presence of high urine TMA levels confirmed the presence of a metabolic disorder. The two (unrelated) children carried compound heterozygous variants in the FMO3 gene. In both cases, vitamin B2 administration decreased TMA excretion and reduced body odor. The 11 adults complained of an unpleasant body odor, but the physicians did not confirm this. In all adult patients, the urine TMA level was within the normal range reported for control (non-affected) subjects, although two of the patients displayed an abnormally high proportion of oxidized TMA. Seven of the 9 tested adult patients had a hypomorphic variant of the FMO3 gene; the variant was found in the homozygous state, in the heterozygous state or combined with another hypomorphic variant. All 11 adults presented a particular psychological or psychiatric phenotype, with a subjective perception of unpleasant odor.Conclusions: The results present the clinical and biochemical data of patients complaining of unpleasant body odor. Contrary to adult patients, the two children exhibited all criteria of recessively inherited trimethylaminuria, suspected by parents in infancy. B2 vitamin treatment dramatically improved the unpleasant body odor and the ratio of TMA/Cr vs TMAO/Cr in the urine in the children. Other patients presented a particular psychological or psychiatric phenotype. [ABSTRACT FROM AUTHOR]

An abstract of the article "Apically directed postsystolic motion of the basal anteroseptal wall during stress-echo" by A. Ouss and P. A. Van der Wouw is presented.

An abstract of the article "The Temporal Relationship Between Mitral and Aortic Valves Opening and Closure and the Myocardial Velocity Curve" by A. Ouss and colleagues is presented.

An abstract of the article "Apically directed post-systolic motion of the non-ischemic myocardium" by A. Ouss and P. A. Van der Wouw is presented.

An abstract of the article "The delay between ECG and spectral Doppler signal is PRF-dependent" by A. Ouss and P. A. Van der Wouw is presented.