Children-Basel最新文献

Background: Childhood-onset systemic lupus erythematosus (cSLE) is a rare, serious autoimmune disease characterized by multiorgan involvement and long-term morbidity. Although several studies have examined this condition in Japan, a comprehensive summary of recent findings remains lacking. Methods: PubMed was searched for Japanese publications on cSLE published between 2015 and 2025, including clinical studies, case reports, translational research, basic science studies, systematic reviews, clinical practice guidance, and transition care guidance. Results: Sixty publications met the inclusion criteria: 20 clinical studies, 30 case reports, 6 translational studies, 1 basic science study, 1 systematic review, 1 clinical practice guidance, and 1 transition care guidance. Most clinical studies were retrospective, although multicenter and registry-based designs have increased in recent years. Lupus nephritis remained the primary research focus, with accumulating evidence supporting mycophenolate mofetil, tacrolimus, and early belimumab as glucocorticoid (GC)-sparing approaches. Case reports illustrated the broad clinical spectrum of cSLE, with hematological and vascular complications being the most frequently reported. Translational studies highlighted the pathogenic role of type I interferon signaling and cytokine dysregulation, particularly in macrophage activation syndrome. Despite these advances, prospective studies and standardized assessment methods for pediatric-specific practice remain limited. Conclusions: Over the past decade, cSLE research in Japan has contributed to a deeper understanding of its clinical and immunological characteristics. However, treatment-related complications and long-term organ damage remain important challenges. Continued multicenter collaboration and domestic data accumulation may strengthen the evidence base, facilitate optimization of GC-sparing approaches, improve clinical management, and provide background information to support future discussions on clinical practice guidelines.

Background: Children's development is dependent on a range of factors influencing their life course outcomes. Protective and challenging social and cultural determinants impact how Indigenous families support their children's developmental foundations. However, there is a lack of international evidence investigating Indigenous child development interventions. To gain a perspective across nations with comparable settler-colonial histories, this scoping review summarised studies on family and community-centred approaches among Indigenous populations in Australia, Canada, New Zealand, and the United States, focusing on outcomes and evidence gaps. Methods: A scoping review followed PRISMA-ScR guidelines. Medline, CINAHL, and PsycINFO (Ovid) were searched from their inception to October 2025, including grey literature sources from Aboriginal HealthInfoNet, the Lowitja Institute and the Secretariat of National Aboriginal and Islander Child Care. Empirical studies, including quantitative, mixed-methods, evaluation studies, and descriptive or case-study designs, were included provided they reported empirical data on intervention outcomes. Due to study heterogeneity, data were synthesised narratively. Results: Following screening of 2355 records, eight from 2013 to 2020 met the inclusion criteria. These were mostly small-scale, non-randomising designs evaluating different interventions, with the behavioural and emotional domain being the most frequently assessed outcome, alongside developmental vulnerability and academic/educational areas. There was limited consideration of protective cultural determinants of health in the study design and implementation. Six studies reported positive associations between interventions or programmes and early childhood development outcomes. Conclusions: While the number and rigour of identified interventions were limited, several demonstrated potential benefits for Indigenous children's early childhood development. However, strengthening the evidence base requires culturally grounded, adequately powered evaluations using rigorous study designs that include culturally co-designed adaptations conducted with Indigenous families and communities. Support is recommended for capacity building and funding.

Background/objectives: Shared decision-making is a central principle in pediatric practice, yet its implementation becomes challenging in the context of alternative family configurations. Stepparents have substantial caregiving roles, but Belgian legislation does not include them in medical information or decision-making authority, creating a gap between legal frameworks and clinical realities. The objective of this study was to explore pediatricians' perspectives on the involvement of stepparents in medical information sharing and decision-making for minors, and to identify factors influencing whether and how stepparents are included.

Methods: A qualitative study was conducted using six semi-structured focus group interviews with 30 pediatricians from six hospitals across Flanders, Belgium. Participants were purposively sampled based on clinical experience. The interviews explored experiences with consent, confidentiality, and stepparent involvement in pediatric care. Data were audio-recorded, transcribed verbatim, and analyzed using constant comparative analysis to identify overarching themes.

Results: Three overarching themes emerged. First, the medical context strongly shaped decisions: medical information and minor decision-making were frequently shared, while major decision-making often involved consultation with the legal guardian. Second, relational dynamics, including the quality of the stepparent-child relationship, co-parenting conflict, and physicians' intuitive assessments, influenced the extent to which stepparents were involved. Third, vulnerability was a recurring theme across all actors: physicians felt legally exposed, children risked fragmented care, legal guardians feared loss of control, and stepparents lacked recognition despite significant caregiving roles.

Conclusions: This study shows the importance of a better alignment between clinical practice and legal reality. Aligning legal frameworks with contemporary family patterns may support more consistent, child-centered decision-making in pediatric practice.

Background: Regenerative endodontic treatment (RET) has emerged as a biologically based alternative to traditional apexification for managing immature permanent teeth with pulp necrosis. By promoting tissue ingrowth and continued root development, RET aims not only to eliminate infection but also to reinforce structurally compromised roots. Although its clinical use has expanded, evidence regarding the long-term predictability and durability of RET remains limited, as most published studies provide only short- or mid-term follow-up.

Case presentation: This report describes two pediatric cases involving regenerative procedures performed on three immature permanent maxillary incisors, each followed for more than six years. The first case involved a 7-year-old girl who developed pulp necrosis in a maxillary lateral incisor after acute dental trauma. Management followed a regenerative protocol using triple antibiotic paste (ciprofloxacin, metronidazole, and minocycline) as intracanal medication and mineral trioxide aggregate as the coronal barrier. The second case concerned an 8-year-old girl presenting with chronic infection and sinus tracts affecting both maxillary central incisors. These teeth were treated using a regenerative approach with calcium hydroxide as the intracanal medicament and Biodentine as the sealing material. Clinical, radiographic, and cone beam computed tomography evaluations demonstrated complete symptom resolution and periapical healing but incomplete progressive apical closure. All treated teeth developed a calcified apical barrier, and outcomes remained stable throughout the extended follow-up period.

Conclusions: While inherently limited by the nature of case reports, these findings support RET as a reliable and durable therapeutic option for necrotic immature permanent teeth, including cases in which conventional apexification has not been successful.

Background/Objectives: Admission of a newborn to a neonatal intensive care unit (NICU) places mothers under considerable psychological strain, yet there is limited research from resource-limited settings regarding this aspect. This study represents the Sri Lankan arm of the multicentre study titled "Empowering Parents in the NICU". It aimed to determine the prevalence and associated factors of stress, depression, and anxiety among mothers of neonates admitted to neonatal units and to assess mothers' participation in neonatal care. Methods: A cross-sectional study was conducted in two tertiary neonatal units in Sri Lanka. Maternal stress, depression, and anxiety were measured using the Parental Stressor Scale: NICU, Edinburgh Postnatal Depression Scale, and State-Trait Anxiety Inventory. Maternal involvement in care was assessed using the Index of Parental Participation (IPP-NICU). Univariate and multivariate logistic regression analyses were performed. Results: A total of 300 mothers were enrolled. The prevalence of stress, depression, and state anxiety was 73%, 87%, and 77.7%, respectively. Overall, 94.3% experienced at least one psychological condition, while 59% experienced all three. Only 13% achieved an IPP-NICU score ≥ 20 (maximum: 30). Mothers of infants admitted to NICUs, compared with those in semi-intensive care, showed significantly higher rates of depression. Tamil and Muslim mothers demonstrated lower rates of state anxiety compared to Sinhalese mothers. An IPP-NICU score ≥ 20 was associated with reduced stress and anxiety. Conclusions: High psychological morbidity is observed among mothers of neonates managed in neonatal units, emphasising the need for routine maternal mental health screening and promoting maternal participation in neonatal care.

Background/Objectives: Worldwide, Type 1 diabetes mellitus (T1DM) in youth represents a growing public health concern, and Italy is among the countries with the highest incidence in the pediatric population. The transition from pediatric to adult care is a vulnerable period associated with increased risks of acute complications and long-term morbidity. This scoping review aimed to map the available Italian evidence on healthcare transition in adolescents and young adults (AYAs) with T1DM, addressing five key areas: characteristics of the transition process and involved populations, emotional and psychological experiences, the role of technology, existing transitional care models and related outcomes, and assessment criteria and tools for transition readiness. Methods: This review followed the JBI methodology and included studies focused on Italian AYAs (aged 10-24 years) with T1DM. Study selection was documented using the PRISMA flow chart. Results: Twenty studies were included. The evidence revealed a heterogeneous and inconsistently implemented transition landscape. Several structured transition projects were identified, differing in multidisciplinary team composition, organization, and outcome evaluation. Emotional distress, fear of separation from pediatric providers, and variable satisfaction with transition experiences were commonly reported. Adoption of technologies increased over time and was associated with improved clinical outcomes, although overall uptake remained suboptimal. Importantly, no Italian-validated tools for assessing transition readiness were identified. Conclusions: Transitional care for Italian AYAs with T1DM is increasingly recognized but remains insufficiently standardized and evaluated. Future research should prioritize multicenter studies, stratified analyses, and the development of culturally validated readiness assessment tools to support effective and individualized transitions.

Background: Pediatric cataracts are a main cause of irreversible vision loss and a significant public health challenge. This study aimed to identify pharmacovigilance signals by analyzing large-scale data from the U.S. Food and Drug Administration Adverse Event Reporting System (FAERS). Methods: Using real-world data from FAERS (Q1 2004 to Q3 2025), we investigated associations between medications and pediatric cataracts. Following data standardization, signal detection was performed using multiple disproportionality analyses, including the Reporting Odds Ratio (ROR), Proportional Reporting Ratio (PRR), Bayesian Confidence Propagation Neural Network (BCPNN), and Multi-item Gamma Poisson Shrinker (MGPS). The time to onset was also evaluated. Results: Among 690,374 reports for individuals aged 0-17 years, 671 reports involving 232 drugs were reported with cataracts. Disproportionality analysis identified 24 drugs with significant signals, predominantly glucocorticoids (11/24), followed by immunosuppressants, monoclonal antibodies, cystic fibrosis transmembrane conductance regulator (CFTR) modulators, antineoplastic agents, an antiepileptic drug, and a colony-stimulating factor. Difluprednate showed the highest pharmacovigilance signal (ROR: 963.67; 95% CI: 316.27-2936.31; n = 4). Notably, CFTR modulators exhibited striking signals: ivacaftor (ROR: 30.75; 95% CI: 18.06-52.37; n = 14), elexacaftor-ivacaftor-tezacaftor (ROR: 15.58; 95% CI: 9.86-24.63; n = 19), and ivacaftor-lumacaftor (ROR: 13.2; 95% CI: 7.9-22.07; n = 15). Conclusions: This study provides a comprehensive large-scale pharmacovigilance profile of drug-induced pediatric cataracts, identifying agents with high-risk pharmacovigilance signals and underscoring the need for proactive ocular monitoring. These findings can inform clinical decision making and prevention strategies and guide future mechanistic research.

Background/objectives: The prevalence of autistic spectrum disorder has been increasing rapidly in the world population and the cause of this increase is unknown. Autistic spectrum disorder is an important cause of social, communication and specific learning difficulties in children. Assortative mating may increase the genetic burden leading to manifestation of polygenic diseases affecting mental health in the offspring. Correlation of scores in the social responsiveness scale (SRS), which is used to quantify autistic spectrum disorder features, between spouses, has been used as indicator of phenotypic assortative mating. We investigated whether assortative mating is involved in increased severity of autism spectrum disorder in the offspring. Methods: All studies reporting on investigation of assortative mating in relationship to autistic spectrum disorder were included. Information sources were PubMed, EMBASE and the Cochrane Library. Results were synthesized by entering correlation analyses of results of the SRS conducted in spouses in a meta-analysis. A sub-group analysis was performed comparing spouses with offspring with diagnosed autistic spectrum disorder to spouses without. Prevalence of autistic spectrum disorders in children in countries with and without predominant assortative mating was compared. Results: A total of 14 investigations of assortative mating including 9914 spouse pairs were included. In total, 8 studies (4641 spouse pairs) reported intra-class correlation (ICC) or Spearman's correlation coefficients between spouses' SRS scores. There was a significant correlation of SRS scores in studies using ICC or Spearman's correlation with a pooled coefficient = 0.37. Spouse pairs (n = 401) with offspring diagnosed with autistic spectrum disorder had a pooled ICC coefficient which was 0.278 (95% CI 0.08 to 0.46), significantly lower than spouse pairs without (n = 1525): 0.40 (95% CI 0.35 to 0.46). Higher scores in SRS of both spouses were associated with higher scores and more autism diagnoses in offspring. Pooled prevalence of autistic spectrum disorder in children in countries where assortative mating is most common was 63.1 per 10,000 of population and in countries without it was significantly lower with 14.1 per 10,000 of population. Conclusions: There is evidence of assortative mating according to social responsiveness scale score which correlates significantly in spouse pairs with and without children with autistic spectrum disorder. In countries where assortative mating is predominant, a higher prevalence of autism spectrum disorder in children is found compared to countries without.

Objectives: To evaluate the effects of age, body mass index, and treatment duration on treatment response in children with primary monosymptomatic nocturnal enuresis and to determine the contribution of these variables to clinical outcomes.

Methods: Data from 560 pediatric patients treated with desmopressin due to primary monosymptomatic nocturnal enuresis were retrospectively analyzed. Patient demographics, body mass index classifications, early treatment response, and dryness rates at 3 and 6 months were evaluated. Categorical variables were analyzed using the chi-square or Fisher's exact test, and continuous variables were evaluated using Spearman correlation analysis. Significant factors predicting treatment success were investigated using multivariable analyses.

Results: Treatment success was significantly higher in younger age groups, with increased early response rates and shorter treatment duration (p < 0.001). Higher body mass index was associated with a delay in early treatment response and the need for longer treatment (p < 0.0001). While the likelihood of treatment success decreased with increasing age, higher body mass index demonstrated a modest positive association with treatment response after multivariable adjustment.

Conclusions: The findings indicate that age and body mass index are important determinants of treatment response in pediatric monosymptomatic nocturnal enuresis. These results suggest that treatment strategies should be individualized by considering both age and body mass index.

Background: The aims of the study were to describe facial morphology and analyze facial growth in adolescents with Robin sequence (RS) or Stickler syndrome.

Methods: The facial morphology, mandibular size, and facial growth of 69 adolescents (ages 12-18) with RS were analyzed using existing cephalometric radiographs (n = 37) and photographs (n = 69). All participants were followed in our institution since birth. None underwent growth-modifying treatment for micrognathia during infancy, but all had conservative orthodontic treatment during adolescence.

Results: Cross-sectional cephalometric analysis according to Tweed revealed differences in RS adolescents as compared with reference values, such as a proportionate retrusion of both jaws, as indicated by decreased SNA and SNB angles (p < 0.05). This finding was mostly associated with skeletal Class I (62.2%) and a vertical facial pattern as indicated by increased FMA and CoGoMe angles (p < 0.05). In Delaire's analysis, patients showed decreased maxillary, maxillary alveolar (p < 0.05), and mandibular body territories (p > 0.05) but increased ramus (p > 0.05) and nasopremaxillary territories (p < 0.05). According to Ricketts' analysis, mandibular width was decreased in half of our patients (p > 0.05). The mandibles were harmoniously downsized before and after the growth spurt (p < 0.05); however, they exhibited greater growth velocities than controls. A long-term study during puberty revealed an increase in SNB angles and a decrease in ANB angles (both p < 0.05), which improved the maxillomandibular relationship. Additionally, the vertical facial pattern attenuated (FMA, SNGoGn, and CoGoMe angles decreased; p > 0.05). On cross-sectional photographic analysis, 33.3% of patients had an orthofrontal (straight), 59.4% a cisfrontal (convex), and 7.3% a transfrontal (concave) profile. Their vertical facial proportions were normal. In the subjective profile analysis, most patients (approximately 84%) had good or acceptable profiles, with no major deficit of chin projection. The initial degree of neonatal retrognathia and type of cleft palate surgery did not affect major skeletal parameters (p > 0.05). However, the degree of neonatal functional impairment affected the vertical parameters (SNGoGn, FMA angle; p < 0.05).

Conclusions: Overall, RS patients presented a bi-retrognathic profile, a normal jaw relationship, and a tendency toward a vertical growth pattern. Partial mandibular catch-up growth occurred during the pubertal growth spurt. The degree of neonatal retrognathia does not predict further mandibular growth.