Introduction This cross-sectional study examined the relationships among English learning beliefs (ELBs), English learning self-efficacy (ELSE), English classroom enjoyment (ECE), and English autonomous learning ability (EALA) among secondary school students. Methods A convenience sample of 1,939 secondary school students was recruited from 11 provincial-level regions in China. Mediation and moderated mediation analyses were conducted using Hayes' PROCESS macro (Models 4 and 8) with 5,000 bootstrap samples, controlling for age, school stage, family residence, only-child status, and monthly family income. Results After controlling for covariates, ELBs were positively associated with EALA (B = 0.731, 95% CI [0.701, 0.761]). A significant indirect association between ELBs and EALA via ELSE was observed (indirect association = 0.377; 95% bootstrap CI: [0.340, 0.414]). ECE significantly moderated the association between ELBs and ELSE (B = 0.036, 95% CI [0.017, 0.054]), such that the positive association was stronger at higher ECE levels. The index of moderated mediation was significant (index = 0.016, 95% bootstrap CI [0.007, 0.024]), indicating that the indirect association between ELBs and EALA through ELSE was stronger at higher levels of ECE. Discussion These findings highlight the relevance of learning beliefs, self-efficacy, and classroom enjoyment to secondary school students' autonomous English learning.
Background Augmentative and Alternative Communication (AAC) refers to a range of communication methods used by children or adults to enhance or replace oral communication. Studies show many children with communication needs who require AAC do not have access to appropriate supports, and that embedding AAC interventions in the classroom is challenging. This means many children who use AAC face barriers to meaningful participation, engagement, and independence. Understanding the experiences and perspectives of AAC users and those who support the use of AAC in the classroom can provide actionable knowledge to guide service improvement such that AAC users can be empowered to achieve and participate in school. Methods A comprehensive literature search of the following electronic databases will be completed: PubMed, CINAHL Complete, EMBASE, EBSCO and Scopus. A manual search of the grey literature will also be carried out. Qualitative studies and mixed-methods designs that include qualitative data on the perspectives and experiences of AAC users, their families, and educators and healthcare professionals who support AAC users in school settings for children 3-18 years will be analysed. Methodological quality of included papers will be appraised using the Critical Appraisal Skills Programme (CASP) checklist for qualitative research by two independent reviewers. The Grading of Recommendations Assessment, Development, and Evaluation (GRADE) - Confidence in the Evidence from Reviews of Qualitative research (CERQual) approach will be employed to assess how much confidence to place in the findings of the qualitative evidence synthesis. Qualitative content analysis will be undertaken using constructs from Normalisation Process Theory. Conclusion This qualitative evidence synthesis will summarise findings from the empirical international literature to provide a deeper understanding of the experiences of AAC use from a range of stakeholder perspectives. Findings will inform the development of pathways to support implementation of AAC for children with communication needs within schools. Prospero Registration No. CRD420251229480.
Abstract AIMS: We report a cohort of YCMB cases homogeneously treated with HDCT in two Italian institutions, and the prognostic impact of histology and genetics retrospectively evaluated. METHODS: All YCMB (aged≤3 years) treated with upfront HDCT in the period 1998-2019 were included, reclassified according to the WHO2021 classification of CNS tumours. Mutational status ofPTCH1, SUFU, and TP53 was analysed in selected cases. Histology and genetics were correlated with survival, secondary tumours(STs), and cancer predisposition syndromes(CPSs). RESULTS: Fifty-three patients were enrolled (62.3% male), median age 2.2 years. 21 had classic(CMB), 15 desmoplastic/nodular(DMB), 11 MBEN and 6 large-cell/anaplastic(AMB/LCMB) medulloblastoma. Metastases were present in 18. Genomic pattern showed SHH-TP53wt in 29 cases, non-WNT/non-SHH in 22; 2 were SHH-TP53mut. Induction chemotherapy (VCR/HDMTX, HDVP16, VCR/HDCTX and HDCARBO) was followed by 2-3 HDCT courses; irradiation reserved to cases with metastatic disease and/or residual tumours. 22 patients never received irradiation. SHH-TP53wt cases had significantly less metastasis (p=0.002), while non-WNT/non-SHH received more often irradiation (p<0.0001). OS at 5, 10, and 20 yrs was 0.73, 0.70 and 0.57 respectively in the entire cohort; stable at 0.85 (at 5, 10, and 20 yrs) in SHH-TP53wt patients while 0.58, 0.51 and 0.17 in the non-WNT/non-SHH. PFS at 5, 10, 20 yrs was stable at 0.89 in SHH-TP53wt and remained 0.35 in non-WNT/non-SHH. 13/53 patients presented Gorlin Syndrome; 1 had familial MB. 16 STs were reported in 14 cases; life-threatening, irradiation-related STs mainly in non-WNT/non-SHH cases. In SHH-TP53wt benign tumours or related to CPS were reported. CONCLUSIONS: This is one of the first series of YCMB treated with HDCT without stratification for stage and histology. The long follow-up highlights the frequency/types of associated CPS and STs; the latter, in non-WNT/non-SHH, were treatment-related and life-threatening.
Background We evaluated the impact of the COVID-19 pandemic on adolescents born preterm versus at term. Methods This prospective cohort study included participants aged 13-21 years in the Environmental influences on Child Health Outcomes (ECHO) Cohort. Participants completed questionnaires from April 2020 to June 2022 about their behaviors during the pandemic and the impact of the pandemic on their social connectedness and overall well-being. Analyses compared adolescents born extremely preterm (before 28 weeks of gestation; n = 419) and those born moderate or late preterm (≥ 28 weeks and < 37 week; n = 118) with those born at term (n = 1,598). Associations were expressed as adjusted prevalence ratios (PRadj) and 95% confidence intervals (CIs). Results Adolescents in both preterm groups were less likely than those born at term to participate in unhealthy behaviors, such as drinking alcohol and using marijuana (moderate preterm: PRadj 0.78 [95% CI 0.61, 0.99]; extremely preterm: PRadj 0.74 [95% CI 0.58, 0.94]). Girls born extremely preterm were more likely to report health-promoting/prosocial behaviors (PRadj 1.15 [95% CI 1.06, 1.25]). Participants born moderate or late preterm reported feeling more socially connected during the pandemic compared with pre-pandemic (PRadj 1.29 [95% CI 1.09, 1.52]); nonetheless, this group was more likely to report a negative overall impact of the pandemic on their lives (PRadj 0.84 [95% CI 0.76, 0.92]). Conclusion During the COVID-19 pandemic, adolescent behaviors and self-reported impact differed for participants born preterm compared with those born at term. These findings can inform counseling for parents and adolescents during future national crises.
Abstract Many pediatric oncology patients report medical marijuana (MMJ) and hemp-based CBD use. Eleven states and Washington, DC have legalized marijuana for recreational use for adults greater than 21. Medical marijuana is legalized in 33 states. Additionally, due to the bipartisan Farm Bill passed in December of 2018, hemp is federally legal. Marijuana has medical legalization in 23 countries worldwide. Clinical trials in adults have examined MMJ for cancer-related symptoms. New research is emerging on MMJ in anticancer therapy, MMJ receptors on tumor cells, and the potential role for MMJ as an immunomodulator. Few pediatric oncology studies have evaluated MMJ. We describe the initial findings of a prospective observational study of MMJ on the quality of life (QOL) in pediatric brain tumor patients. Specific aims included (1) MMJ’s association with symptoms (nausea, anxiety, pain, fatigue, and cognitive problems) and (2) MMJ’s impact on family dynamics. The legality of hemp plus the increasing use of MMJ raises concerns with pharmacological interactions with CBD and the medications routinely administered to children with cancer. Nurses are the frontline for discussions with patients about MMJ and must be aware of the emerging field of MMJ in pediatric cancer. Additionally, nurses can influence patient care protocols and processes for alternative therapy administration enabling an open dialogue between providers, parents, and patients regarding treatments, symptoms, adverse effects, and drug interactions. Education about how to have conversations about important facets to cover and consider is crucial to patient safety and increased quality of life.
Abstract BACKGROUND Atypical teratoid/rhabdoid tumors (AT/RT) are highly aggressive brain tumors affecting early childhood and are characterized by bi-allelic inactivation of the SMARCB1 gene. Though patients benefit from multimodal therapy, there is no improvement in overall survival necessitating exploration of alternative approaches including innate-based immune therapy and epigenetic therapy, which have shown promise in treating adult brain tumors and other cancers. Though reconstitution of SMARCB1 in SMARCB1-deficient cells leads to activation of interferon-stimulated genes, the role of innate immune signaling has not been investigated in AT/RTs. METHODS Our data from a panel of AT/RT cell lines indicates loss of expression of key innate signaling components, like RIG-I, MDA-5, cGAS and STING that are required for sensing extracellular dsRNA and dsDNA. These cell lines also do not respond to dsDNA-based or dsRNA-based innate agonists. However, co-treatment of the BT-16 cell line with two epigenetic drugs, panobinostat and 5-azacytidine leads to re-expression of STING and RIG-I. Panobinostat/5-azacytidine co-treatment followed by either genomic DNA (dsDNA agonist) or poly(I:C) (dsRNA agonist) treatment results in induction of innate responses, measured by STAT1 phosphorylation and production of ISG-15 and IFIT-1. CONCLUSION Our data suggests that AT/RT cell lines are unresponsive to innate agonists possibly due to the loss of expression of key innate immune components. However, these pathways can be reactivated by epigenetic drugs and further potentiated by dsDNA/dsRNA-based innate agonists. Combined epigenetic reprogramming and innate pathway stimulation may serve as a potential therapy option for treating AT/RT.
Background The proposal to involve patients and the public (Patient and Public Involvement; PPI) in research is increasingly recognised as important in research concerning speech, language, and hearing and communication difficulties. Whilst there is extensive guidance about PPI involvement by national agencies such as the National Institute of Health Research in the UK, little information is currently available concerning whether these expectations are met in any area of healthcare provision. Aims This systematic review aimed to identify, describe and evaluate how PPI has been implemented and reported in research concerning childhood speech, language, hearing and communication difficulties. Methods Six electronic databases were systematically searched for eligible studies reporting PPI in these fields. Records were screened against predefined eligibility criteria. Data were extracted on study characteristics, PPI contributors, stages and methods of involvement, reported impacts, and quality of PPI reporting. The methodological and PPI quality were assessed using MMAT and GRIPP2 Short Form, and findings were synthesised narratively. Results Twenty-one studies were included. PPI was reported variously across speech, language, hearing and communication topics, the implementations varied considerably. Parents, carers and professionals were more commonly involved than children and young people themselves. Most studies used consultation-based approaches, often during early stages (e.g., study design or material development), while fewer reported involvement in later stages (e.g., dissemination, implementation or evaluation). Reporting was inconsistent, particularly regarding contributor characteristics, accessibility adaptations, decision-making influence, and the impact of PPI. Conclusions The systematic review showed that PPI in childhood speech, language, and hearing difficulties has advanced significantly, with the growing interest and number of publications, particularly in the past five years. The review also highlighted challenges such as confusion and heterogeneity of PPI concepts, representativeness issues, tokenistic practices, lack of support given to lay contributors, and limited impact evaluation. Future research should distinguish PPI from research participation, involve children and young people more directly where appropriate, report contributors' roles and influence more transparently, and consider accessibility and communication support throughout the involvement process. What this paper adds What is already known on the subject Health service providers and research funders now require attention to Patients and Public Involvement (PPI) in healthcare research. PPI highlights the need to work jointly with stakeholders at all steps involved in research to allow them to take governance over their health conditions. Whilst emphasis on PPI applies to health issues in general, there is no specific review of PPI considerations in research on health factors that affect hearing and communication difficulties. This omission is particularly noteworthy when considering issues that arise in early development. What this study adds to existing knowledge A systematic review was conducted to assess current progress with respect to PPI for hearing and communication research in childhood and adolescence. The review identified research involving PPI that met quality requirements. Challenges related to PPI were highlighted in further analyses that revealed confusion and heterogeneity in PPI concepts, representativeness issues, tokenistic practices, a lack of support given to lay contributors, and a limited evaluation of the impacts of PPI. What are the clinical implications of this work? PPI involvement in clinical research for communication and hearing difficulties should have a positive impact on the well-being of children and adolescents. Four challenges were highlighted when considering the current state of research on PPI in this domain: shallow involvement of the public and patients, conflicting expectations between groups participating in research, lack of clarity of research outcomes as they relate to PPI and variable standards of reporting PPI findings across studies. Tentative recommendations concerning how each of these matters could be addressed are offered.
Abstract Background: Ten percent of children born small for gestational age with a birth weight and/or length of below -2 SD for their gestational age fail to catch-up and remain short during childhood (SGA-SS). The etiology of SGA-SS is heterogeneous: some children have specific phenotypic features that allow targeted genetic testing; in others, elucidating genetic or environmental background is more challenging. Aim: To decipher genetic etiologies among a large single-center cohort of SGA-SS children and to stratify them according to molecular mechanisms leading to pre- and postnatal growth failure. Patients/Methods: In our center 447 children (223 females) fulfilled the criteria of SGA-SS. Of these 182 families agreed to take part and offered the child’s and both parents’ DNA for genetic testing by a panel of 399 growth-related genes, or by Whole Exome Sequencing (WES). The results were processed by a bioinformatic pipeline and detected variants were filtered using variant analysis software. Pathogenic or likely pathogenic variants (according to ACMG standards and guidelines) were confirmed by Sanger sequencing. Results: The genetic etiology was elucidated in 73/182 (40%) children so far. We confirmed (likely) pathogenic gene variants affecting pituitary development and/or the GH-IGF-1 axis in 10/73 (14%) patients (PTCH1, HGMA2 [in two], OTX2, LHX4, GHSR, STAT3, IGFALS, IGF1R [in two]), abnormal components of cartilaginous matrix in 17/73 (23%) (ACAN [in two], FLNB [in three], FBLN5, COL11A1[in four], COL1A2, COL2A1[in five], MATN3), impaired paracrine regulation of chondrocytes in 4/73 (6%) (NPR2 [in three], FGFR3), SHOX gene defects in 12/73 (16%), gene variants affecting other components of intracellular regulation and signaling in 9/73 (12%) (CDC42, KMT2A, KMT2D, NSD1, SRCAP, PRG4, PTPN11, SON, LMNA), Silver-Russell syndrome (11p15 [in seven] or UPD7) in 11/73 (15%), and miscellaneous single-gene or chromosomal conditions (TRPS1, TRHR, RAI1, chromosomal microdeletions and/or translocations) in an additional ten (14%) children. Conclusions: In our study we showed that by using current genetic techniques we were able to elucidate the genetic cause in a significant number of patients born SGA-SS. The genetic etiology spectrum of SGA-SS reflects the complex system of growth regulation, with a significant role of growth plate genes that are causative in 33/73 (45%) cases clarified thus far. Acknowledgements: The study was co-funded by grants AZV NV18-07-00283 and GAUK 408120.
Abstract Background Multi-system inflammatory syndrome in children (MIS-C) is a rare consequence of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). MIS-C shares features with common infectious and inflammatory syndromes and differentiation early in the course is difficult. Identification of early features specific to MIS-C may lead to faster diagnosis and treatment. We aimed to determine clinical, laboratory, and cardiac features distinguishing MIS-C patients within the first 24 hours of admission to the hospital from those who present with similar features but ultimately diagnosed with an alternative etiology. Methods We performed retrospective chart reviews of children (0-20 years) who were admitted to Vanderbilt Children’s Hospital and evaluated under our institutional MIS-C algorithm between June 10, 2020-April 8, 2021. Subjects were identified by review of infectious disease (ID) consults during the study period as all children with possible MIS-C require an ID consult per our institutional algorithm. Clinical, lab, and cardiac characteristics were compared between children with and without MIS-C. The diagnosis of MIS-C was determined by the treating team and available consultants. P-values were calculated using two-sample t-tests allowing unequal variances for continuous and Pearson’s chi-squared test for categorical variables, alpha set at < 0.05. Results There were 128 children admitted with concern for MIS-C. Of these, 45 (35.2%) were diagnosed with MIS-C and 83 (64.8%) were not. Patients with MIS-C had significantly higher rates of SARS-CoV-2 exposure, hypotension, conjunctival injection, abdominal pain, and abnormal cardiac exam (Table 1). Laboratory evaluation showed that patients with MIS-C had lower platelet count, lymphocyte count and sodium level, with higher c-reactive protein, fibrinogen, B-type natriuretic peptide, and neutrophil percentage (Table 2). Patients with MIS-C also had lower ejection fraction and were more likely to have abnormal electrocardiogram. Conclusion We identified early features that differed between patients with MIS-C from those without. Development of a diagnostic prediction model based on these early distinguishing features is currently in progress. Disclosures Natasha B. Halasa, MD, MPH, Genentech (Other Financial or Material Support, I receive an honorarium for lectures - it’s a education grant, supported by genetech)Quidel (Grant/Research Support, Other Financial or Material Support, Donation of supplies/kits)Sanofi (Grant/Research Support, Other Financial or Material Support, HAI/NAI testing) Natasha B. Halasa, MD, MPH, Genentech (Individual(s) Involved: Self): I receive an honorarium for lectures - it’s a education grant, supported by genetech, Other Financial or Material Support, Other Financial or Material Support; Sanofi (Individual(s) Involved: Self): Grant/Research Support, Research Grant or Support James A. Connelly, MD, Horizon Therapeutics (Advisor or Review Panel member)X4 Pharmaceuticals (Advisor or Review Panel member)
Abstract This study examines whether employment security influences fertility decisions across countries by introducing a multidimensional Employment Security Index that captures broader labor market conditions underlying the growing one-and-done family phenomenon. The study employs an unbalanced panel of 176 countries covering the period 2000–2024. The Employment Security Index is constructed from unemployment, youth unemployment, vulnerable employment, and female labor force participation using World Bank data. The relationship is estimated using a two-way fixed-effects model with Driscoll–Kraay standard errors, complemented by robustness tests and heterogeneity analyses across income groups and geographical regions. Stronger employment security is positively and significantly associated with higher fertility after controlling for macroeconomic and demographic characteristics. The findings remain robust across lagged specifications, alternative samples, exclusion of the COVID-19 period, and alternative employment security measures. The positive association is particularly evident in upper-middle- and high-income countries and varies across geographical regions, highlighting substantial institutional heterogeneity. This study extends population economics by conceptualizing employment security as a multidimensional construct rather than relying on individual labor market indicators. It also provides one of the largest global longitudinal assessments of the employment–fertility relationship, offering new evidence that labor market stability constitutes an important economic foundation of contemporary fertility decline and the increasing prevalence of one-child family formation. JEL Classification: J13; J21; J64
