Conservation of function without conservation of amino acid sequence in intrinsically disordered transcriptional activation domains.

Protein function is canonically believed to be more conserved than amino acid sequence, but this idea is only well supported in folded domains, where highly diverged sequences can fold into equivalent 3D structures. In contrast, intrinsically disordered protein regions (IDRs) do not fold into a stable 3D structure, thus it remains unknown when and how function is conserved for IDRs that experience rapid amino acid sequence divergence. As a model system for studying the evolution of IDRs, we examined transcriptional activation domains, the regions of transcription factors that bind to coactivator complexes.

Mixed methods to evaluate knowledge, attitudes and practices (KAP) towards rabies in central and remote communities of Moramanga district, Madagascar.

Control of dog-mediated rabies relies on raising awareness, access to post-exposure prophylaxis (PEP) and mass dog vaccination. To assess rabies awareness in Moramanga district, Madagascar, where rabies is endemic, two complementary quantitative and qualitative approaches were carried out in 2018. In the quantitative approach, a standardized questionnaire was administered to 334 randomized participants living in 170 households located less than 5 km from the anti-rabies treatment center (ARTC) located in Moramanga city (thereafter called the central area), and in 164 households located more than 15 km away from the ARTC in two rural communes (thereafter called the remote area).

Early formative objective structured clinical examinations for students in the pre-clinical years of medical education: A non-randomized controlled prospective pilot study.

Background: The value of formative objective structured clinical examinations (OSCEs) during the pre-clinical years of medical education remains unclear. We aimed to assess the effectiveness of a formative OSCE program for medical students in their pre-clinical years on subsequent performance in summative OSCE.

Methods: We conducted a non-randomized controlled prospective pilot study that included all medical students from the last year of the pre-clinical cycle of the Université Paris-Cité Medical School, France, in 2021.

A decade of progress in juvenile idiopathic arthritis treatments and outcomes in Canada: results from ReACCh-Out and the CAPRI registry.

Objective: To assess changes in juvenile idiopathic arthritis (JIA) treatments and outcomes in Canada, comparing 2005-2010 and 2017-2021 inception cohorts.

Methods: Patients enrolled within three months of diagnosis in the Research in Arthritis in Canadian Children Emphasizing Outcomes (ReACCh-Out) and the Canadian Alliance of Pediatric Rheumatology Investigators Registry (CAPRI) cohorts were included. Cumulative incidences of drug starts and outcome attainment within 70 weeks of diagnosis were compared with Kaplan-Meier survival analysis and multivariable Cox regression.

Immunomodulatory Therapy for MIS-C.

Context: Studies comparing initial therapy for multisystem inflammatory syndrome in children (MIS-C) provided conflicting results.

Objective: To compare outcomes in MIS-C patients treated with intravenous immunoglobulin (IVIG), glucocorticoids, or the combination thereof.

Data Sources: Medline, Embase, CENTRAL and WOS, from January 2020 to February 2022.

Developing a Framework of Cost Elements of Socioeconomic Burden of Rare Disease: A Scoping Review.

Background And Objective: Rare diseases place a significant burden on patients, families, the healthcare system, and society. Evidence on the socioeconomic burden of rare disease is limited and mostly reflects diseases where treatments are available. We developed a framework encompassing recommended cost elements for studies of the socioeconomic burden of rare diseases.

Hyper inflammatory syndrome following COVID-19 mRNA vaccine in children: A national post-authorization pharmacovigilance study.

Background: Multisystem inflammatory syndrome in children (MIS-C) is the most severe clinical entity associated with pediatric SARS-CoV-2 infection with a putative role of the spike protein into the immune system activation. Whether COVID-19 mRNA vaccine can induce this complication in children is unknown. We aimed to assess the risk of hyper-inflammatory syndrome following COVID-19 mRNA vaccine in children.

Promoting the Participation of Children and Adolescents With Disabilities in Sports, Recreation, and Physical Activity.

The benefits of physical activity are likely universal for all children, including children and adolescents with disabilities (CWD). The participation of CWD in physical activity, including adaptive or therapeutic sports and recreation, promotes inclusion, minimizes deconditioning, optimizes physical functioning, improves mental health as well as academic achievement, and enhances overall well-being. Despite these benefits, CWD face barriers to participation and have lower levels of fitness, reduced rates of participation, and a higher prevalence of overweight and obesity compared with typically developing peers.

Osteoporotic Fractures and Vertebral Body Reshaping in Children With Glucocorticoid-treated Rheumatic Disorders.

Context: Osteoporotic fractures are an important cause of morbidity in children with glucocorticoid-treated rheumatic disorders.

Objective: This work aims to evaluate the incidence and predictors of osteoporotic fractures and potential for recovery over six years following glucocorticoid (GC) initiation in children with rheumatic disorders.

Methods: Children with GC-treated rheumatic disorders were evaluated through a prospective inception cohort study led by the Canadian STeroid-induced Osteoporosis in the Pediatric Population (STOPP) Consortium.

Imported schistosomiasis in Paris region of France: A multicenter study of prevalence and diagnostic methods.

Background: There are few data on imported schistosomiasis - especially in children. The objectives of the present study were to estimate the prevalence of imported schistosomiasis in at-risk children in the greater Paris region of France and to compare diagnostic methods.

Method: Children at risk of schistosomiasis who consulted or were hospitalized in four hospitals in the greater Paris region were prospectively included.

Systematic Severe Acute Respiratory Syndrome Coronavirus 2 Screening at Hospital Admission in Children: A French Prospective Multicenter Study.

To assess the relevance of systematic severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) screening of all children admitted to hospital, we conducted a prospective multicenter study including 438 consecutive hospitalized children. A symptom-based SARS-CoV-2 testing strategy failed to identify 45% (95% confidence interval, 24%-68%) of hospitalized children infected by SARS-CoV-2. To limit intrahospital transmission, a systematic screening of children admitted to hospital should be considered.

A Canadian evaluation framework for quality improvement in childhood arthritis: key performance indicators of the process of care.

Background: The evaluation of quality of care in juvenile idiopathic arthritis (JIA) is critical for advancing patient outcomes but is not currently part of routine care across all centers in Canada. The study objective is to review the current landscape of JIA quality measures and use expert panel consensus to define key performance indicators (KPIs) that are important and feasible to collect for routine monitoring in JIA care in Canada.

Methods: Thirty-seven candidate KPIs identified from a systematic review were reviewed for inclusion by a working group including 3 pediatric rheumatologists.

Plasmodium vivax severe imported malaria in two migrants in France.

Background: With less than one severe case per year in average, Plasmodium vivax is very rarely associated with severe imported malaria in France. Two cases of P. vivax severe malaria occurred in patients with no evident co-morbidity.

Successful extracorporeal membrane oxygenation transport of a 4-month-old brain-dead infant for organ donation: A case report.

A 4-month-old infant was declared brain-dead 2 days after being initiated on venoarterial ECMO for a refractory septic shock. All brain death diagnostic criteria were fulfilled according to French law, and parental consent was given for organ donation. The hospital where ECMO was initiated had no authorization for organ procurement, and the donor was then transferred to the local referral center for child organ recovery with our mobile ECMO team to maintain organ perfusion.

Increasing Wellness Through Physical Activity in Children With Chronic Disease and Disability.

Children with chronic medical conditions face many challenges when considering sport participation. Compared with their healthy counterparts, they are often discouraged from physical activity or sports participation because of real or perceived limitations imposed by their condition. Prescribed exercise should be based on the demands of the sport, the effect of the disease on performance, and the potential for exercise-induced acute or chronic worsening of the illness or disability.

Increasing Canadian paediatricians' awareness and use of the new Canadian Physical Activity and Sedentary Behaviour Guidelines for ages 0 to 17 years.

Objective: To examine changes in the awareness, use, feasibility, and barriers associated with the Canadian Physical Activity and Sedentary Behaviour Guidelines for Children and Youth ages 0-17 years in a sample of Canadian paediatricians, and to assess tools/resources developed by the Canadian Paediatric Society (CPS).

Methods: Practicing paediatricians who were members of the CPS were invited to complete an on-line survey in February 2013. In response to survey findings, the CPS developed and promoted tools/resources to increase and improve paediatricians' counselling of the guidelines.

Canadian 24-Hour Movement Guidelines for the Early Years (0-4 years): An Integration of Physical Activity, Sedentary Behaviour, and Sleep.

Background: The Canadian Society for Exercise Physiology convened representatives of national organizations, research experts, methodologists, stakeholders, and end-users who followed rigorous and transparent guideline development procedures to create the Canadian 24-Hour Movement Guidelines for the Early Years (0-4 years): An Integration of Physical Activity, Sedentary Behaviour, and Sleep. These novel guidelines for children of the early years embrace the natural and intuitive integration of movement behaviours across the whole day (24-h period).

Methods: The development process was guided by the Appraisal of Guidelines for Research and Evaluation (AGREE) II instrument.

Congenital pulmonary airway malformations: state-of-the-art review for pediatrician's use.

Unlabelled: Congenital pulmonary airway malformations or CPAM are rare developmental lung malformations, leading to cystic and/or adenomatous pulmonary areas. Nowadays, CPAM are diagnosed prenatally, improving the prenatal and immediate postnatal care and ultimately the knowledge on CPAM pathophysiology. CPAM natural evolution can lead to infections or malignancies, whose exact prevalence is still difficult to assess.

Validation of the Standardized Universal Pain Evaluations for Rheumatology Providers for Children and Youth (SUPER-KIDZ).

Study Design Longitudinal observational clinimetric study with repeated measures. Background No validated multidimensional pain measure for children and youth with juvenile idiopathic arthritis exists. Objective To determine the test-retest reliability, construct validity, and responsiveness of English and French versions of the Standardized Universal Pain Evaluations for Rheumatology Providers for Children and Youth (SUPER-KIDZ).

Growth and weight gain in children with juvenile idiopathic arthritis: results from the ReACCh-Out cohort.

Background: With modern treatments, the effect of juvenile idiopathic arthritis (JIA) on growth may be less than previously reported. Our objective was to describe height, weight and body mass index (BMI) development in a contemporary JIA inception cohort.

Methods: Canadian children newly-diagnosed with JIA 2005-2010 had weight and height measurements every 6 months for 2 years, then yearly up to 5 years.