Frontiers in Pediatrics最新文献

{"title":"Ambroxol hydrochloride and clenbuterol hydrochloride oral solution for wheezing disorders in children in China: evidence mapping and meta-analysis.","authors":"Yongsheng Guo, Run Guo, Yingxue Zou, Bing Huang, Jiao Li, Mei Yu, Jia Zhai, Shiyin Mu, Yingying An, Weiwei Gao","doi":"10.3389/fped.2026.1588948","DOIUrl":"https://doi.org/10.3389/fped.2026.1588948","url":null,"abstract":"<p><strong>Objective: </strong>To evaluate the efficacy and safety of ambroxol hydrochloride and clenbuterol hydrochloride oral solution (AHCHOS) in pediatric patients with wheezing disorders and provide a comprehensive evidence map of the current clinical status.</p><p><strong>Methods: </strong>A literature search was conducted in PubMed, Embase, Cochrane Library, CNKI, Wanfang, and CBM to identify randomized controlled trials (RCTs) evaluating the efficacy and safety of AHCHOS in children with wheezing disorders. The clinical status was descriptively summarized. Meta-analyses for efficacy and safety outcomes were performed using a random-effects model in Review Manager 5.4. Outcomes were treatment effectiveness, times to symptom and clinical sign resolution, length of hospital stay, and adverse events (AEs). All time-related outcomes were reported in days.</p><p><strong>Results: </strong>A total of 227 RCTs were included in the evidence mapping, 14 of which met the criteria for meta-analysis. Evidence mapping revealed a general increase in the number of studies from 2005 to a peak in 2012, followed by a decline. Most studies were conducted in eastern China, particularly in Henan Province. Meta-analysis results demonstrated that combination therapy with AHCHOS significantly improved the overall response rate [risk ratio (RR) 1.26, 95% confidence interval (CI) 1.10-1.44] and significant effective rate (RR 1.55, 95% CI 1.36-1.76). It also significantly reduced the duration (days) of coughing [mean difference (MD) -1.40, 95% CI -1.75 to -1.06], wheezing (MD -1.88, 95% CI -2.50 to -1.26), cough phlegm (MD -2.00, 95% CI -2.76 to -1.24), wet lung sounds (MD -1.82, 95% CI -2.33 to -1.30), pulmonary rales (MD -2.27, 95% CI -2.90 to -1.64), and hospital stay (MD -1.13, 95% CI -1.45 to -0.82). Furthermore, AHCHOS did not increase the risk of AEs compared with conventional treatment alone.</p><p><strong>Conclusion: </strong>Combination therapy with AHCHOS was associated with modest but statistically significant improvements in symptom resolution and length of hospital stay compared with conventional treatment alone. However, all included studies were conducted in China, which may limit the generalizability of the findings. In addition, no definitive conclusions regarding safety could be drawn due to limited and inconsistent adverse event reporting. Further safety assessments in future trials are warranted.</p><p><strong>Systematic review registration: </strong>The protocol has been registered on INPLASY (INPLASY202480121).</p>","PeriodicalId":12637,"journal":{"name":"Frontiers in Pediatrics","volume":"14 ","pages":"1588948"},"PeriodicalIF":2.0,"publicationDate":"2026-04-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13143944/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147836863","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Emergency call training in primary school children: effects of instruction format and dispatcher influence.","authors":"Anthea Peters, Soyhan Bagci, Nicole Müller","doi":"10.3389/fped.2026.1782304","DOIUrl":"https://doi.org/10.3389/fped.2026.1782304","url":null,"abstract":"<p><strong>Background: </strong>Early activation of emergency medical services is the first link in the chain of survival, yet training in emergency call competence has received limited attention compared to cardiopulmonary resuscitation (CPR). Teaching children to place an emergency call may provide a crucial and age-appropriate entry point for first aid education.</p><p><strong>Methods: </strong>In this prospective randomized, controlled experimental study, 71 pupils (7-9 years old) from second and third grades of a German elementary school were randomized to frontal instruction or simulation-based training; a control group completed a test call prior to training. Test calls were conducted using mobile phones, assessed by trained dispatchers, and scored with a validated evaluation sheet (maximum 17 points; ≥11 defined as sufficient).</p><p><strong>Results: </strong>Overall, 84.5% of children achieved a sufficient emergency call. After training, 100% recalled the correct emergency number, compared with 78% in the control group (<i>p</i> = 0.042). No significant differences were found between frontal and simulation groups. Grade level was a strong predictor: 91% of third graders delivered sufficient calls, compared to 64% of second graders. Notably, 9.9% of all children were unable to speak after the dispatcher's greeting, and 12.5% could not provide a location, the majority being second graders. Dispatcher behavior strongly influenced outcomes, with significant differences in 11 of 13 assessed items.</p><p><strong>Conclusion: </strong>Primary school children are capable of placing sufficient emergency calls after a brief training. Instruction format (frontal vs. simulation) had little impact, suggesting that emergency call teaching can be delivered efficiently in classroom settings, preserving simulation time for psychomotor CPR skills. Future trainings should emphasize reducing fear of speaking, ensuring knowledge of addresses, and incorporating dispatcher training to optimize communication with child callers.</p>","PeriodicalId":12637,"journal":{"name":"Frontiers in Pediatrics","volume":"14 ","pages":"1782304"},"PeriodicalIF":2.0,"publicationDate":"2026-04-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13144035/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147837043","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Case Report and literature review: X-linked severe combined immunodeficiency complicated by <i>Talaromyces marneffei</i> infection caused by a novel pathogenic <i>IL2RG</i> mutation.","authors":"Di Qing, Lin Lin, Fang Chen, Junzheng Peng","doi":"10.3389/fped.2026.1769503","DOIUrl":"https://doi.org/10.3389/fped.2026.1769503","url":null,"abstract":"<p><strong>Background: </strong>X-linked severe combined immunodeficiency (X-SCID), caused by mutations in the gamma-chain gene of the interleukin-2 receptor (<i>IL2RG</i>), is a prevalent form of SCID characterized by recurrent and fatal opportunistic infections that occur early in life. <i>Talaromyces marneffei</i> (<i>T. marneffei</i>) infection rarely occurs in children and has a high mortality rate.</p><p><strong>Case presentation: </strong>The patient was a 7-month-old male infant who presented with recurrent cough, fever, and hepatosplenomegaly. Lymphocyte subset analysis confirmed the presence of T-B + natural killer immunodeficiency, and blood culture was positive for <i>T. marneffei.</i> Whole-exome sequencing revealed a novel microdeletion insertion mutation (c.818_819delins A (p. Ile273Lys fsTer21) in <i>IL2RG</i>, resulting in a rare shift in the amino acid sequence of the coding protein. The child was diagnosed with X-SCID due to a novel <i>IL2RG</i> mutation, which was further complicated by <i>T. marneffei</i> infection. Despite receiving systemic anti-infection treatment, the patient died 3 days after discharge. To the best of our knowledge, this novel <i>IL2RG</i> mutation has not been reported previously.</p><p><strong>Conclusions: </strong>For early-onset <i>T. marneffei</i> infection, clinicians must maintain a high index of suspicion for underlying inborn errors of immunity, and definitive diagnosis hinges on genetic testing.</p>","PeriodicalId":12637,"journal":{"name":"Frontiers in Pediatrics","volume":"14 ","pages":"1769503"},"PeriodicalIF":2.0,"publicationDate":"2026-04-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13143903/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147836870","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Effectiveness of the Global Integration Method (Método de Integração Global - MIG) for improving motor and functional outcomes in children with autism spectrum disorder: a randomised controlled trial protocol.","authors":"Thalita Karla Flores Cruz, Reinaldo da Costa Paulino Netto, Fabiana Rachel Martins Costa, Elisa Braz Cota, Amanda Aparecida Alves Cunha Nascimento, Simone Rosa Barreto, Deisiane Oliveira Souto","doi":"10.3389/fped.2026.1804826","DOIUrl":"https://doi.org/10.3389/fped.2026.1804826","url":null,"abstract":"<p><strong>Introduction: </strong>Motor impairments are highly prevalent in children with Autism Spectrum Disorder (ASD) and have been associated with reduced functional independence, participation, and sociocommunicative development. Despite the growing body of evidence supporting the relevance of motor functioning in ASD, motor-based interventions remain underrepresented in high-quality randomized clinical trials. The Global Integration Method (Método de Integração Global - MIG) is an intensive, interdisciplinary intervention grounded in the theories of predictive coding and embodied cognition, with emphasis on motor organization, proprioceptive stimulation, and generalization of functional skills in real-life contexts.</p><p><strong>Objective: </strong>The aim of this study is to evaluate the effectiveness of the MIG program in improving fundamental motor skills and achieving functional goals, compared with conventional physiotherapy and psychological interventions, in children with ASD. Secondary objectives are to investigate the effects of MIG on balance, sociocommunicative skills, and motor performance.</p><p><strong>Methods and analysis: </strong>This is a three-arm randomized controlled trial with concealed allocation and blinded outcome assessors. Sixty-six children with ASD, aged 6 to 12 years, classified as requiring level 1 or 2 support, will be randomized into one of three groups: (I) MIG program, (II) conventional psychological intervention, and (III) conventional motor physiotherapy. Interventions will last five weeks. Assessments will be conducted at baseline, immediately post-intervention, and three months after completion. Primary outcomes include fundamental motor skills and functional goal attainment. Secondary outcomes include measures of balance, sociocommunicative skills, and motor performance. Data will be analyzed using linear mixed-effects models, following the intention-to-treat principle.</p><p><strong>Ethics and dissemination: </strong>The protocol was approved by the Research Ethics Committee of the Faculty of Medical Sciences of Minas Gerais, Brazil (Approval No. 7,456,658). Written informed consent will be obtained from parents or legal guardians, and assent will be sought from participating children when developmentally appropriate. Study findings will be disseminated through peer-reviewed publications, conference presentations, and reports to participating families and institutions.</p><p><strong>Clinical trial registration: </strong>This protocol was prospectively registered in the Brazilian Registry of Clinical Trials https://ensaiosclinicos.gov.br/rg/RBR-7r6n8zd, identifier U1111-1326-2272.</p>","PeriodicalId":12637,"journal":{"name":"Frontiers in Pediatrics","volume":"14 ","pages":"1804826"},"PeriodicalIF":2.0,"publicationDate":"2026-04-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13144026/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147836919","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Management of hypogammaglobulinemia in pediatric patients with refractory lupus nephritis: a focus on belimumab.","authors":"Yanan Han, Yanjun Yang, Peitong Han, Xiaoying Yuan, Chunzhen Li, Jieyuan Cui","doi":"10.3389/fped.2026.1753010","DOIUrl":"https://doi.org/10.3389/fped.2026.1753010","url":null,"abstract":"&lt;p&gt;&lt;strong&gt;Background: &lt;/strong&gt;Although the use of belimumab in children with lupus nephritis (LN) has increased over the past few years, there are limited data on the safety of belimumab in such patients with hypogammaglobulinemia. There are scarce reports of an association between hypogammaglobulinemia and infection in patients with LN who are receiving belimumab treatment.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Methods: &lt;/strong&gt;We reviewed the cases of 27 patients with lupus nephritis and nephrotic-range proteinuria admitted to Hebei Children's Hospital from January 2019 to October 2022. Among the 27 patients, 12 received intravenous (IV) belimumab (at a dose of 10 mg per kilogram of body weight) plus the standard systemic lupus erythematosus therapy (SoC) (belimumab group) and the other 15 received the SoC (glucocorticoids plus cyclophosphamide or mycophenolate mofetil) (control group). Estimated Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) score; total amount of protein in urine in 24 h; the serum levels of IgG, IgM, IgA, and C3; total B lymphocyte count (BLC); total white lymphocyte count (WBC); erythrocyte sedimentation rate (ESR); and C-reactive protein level were measured five times (at weeks 0, 4, 12, 24, and 52, respectively) in the two groups.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Results: &lt;/strong&gt;Hypogammaglobulinemia was observed in 22/27 (81.5%) participants with LN prior to initiating treatment. Participants developed hypogammaglobulinemia by week 4. There were no significant differences between the two groups in the total amount of protein in urine in 24 h, serum IgG level, and total B cell count at 0 weeks. Furthermore, no IgG replacement therapy was used in either group until week 15 of observation. However, five patients in the belimumab group and one patient in the control group, whose serum IgG level was below 4 g/L, received 1-2 intravenous immunoglobulin (IVIG) treatments in weeks 16-26 due to severe or recurrent infections. The incidence of infection in the belimumab group was significantly higher than that in the control group, and the IgG serum level in the belimumab group was significantly lower than that in the control group. We also found that the ESR in the belimumab group was significantly lower than that in the control group at weeks 12 and 24 (&lt;i&gt;P&lt;/i&gt; &lt; 0.05). At weeks 24 and 52, the C3 level in the belimumab group was significantly higher than that in the control group, and the SLEDAI score in the belimumab group was significantly lower than that in the control group (&lt;i&gt;P&lt;/i&gt; &lt; 0.05). At week 52, the WBC in the belimumab group was significantly higher than that in the control group (&lt;i&gt;P&lt;/i&gt; &lt; 0.05). However, there was no significant difference in the total amount of protein in urine in 24 h between the two groups at the five time points.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Conclusions: &lt;/strong&gt;Hypogammaglobulinemia is common in refractory LN. Belimumab treatment may increase the possibility of IgG reduction and the risk of infection. Pediatric patients with L","PeriodicalId":12637,"journal":{"name":"Frontiers in Pediatrics","volume":"14 ","pages":"1753010"},"PeriodicalIF":2.0,"publicationDate":"2026-04-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13144007/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147836986","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Association of image-defined risk factors with clinical features in thoracic neuroblastoma and the development of a prognostic prediction model.","authors":"Chong Shen, Zihao Bai, Kaiqian Zhou, Yujun Ma, Ming Yang, Jirong Qi, Bo Qian, Kaihong Wu","doi":"10.3389/fped.2026.1793866","DOIUrl":"https://doi.org/10.3389/fped.2026.1793866","url":null,"abstract":"<p><strong>Background: </strong>Image-defined risk factors (IDRFs) were critical in managing and predicting outcomes for neuroblastoma. This study systematically evaluated baseline clinical features, IDRFs prevalence, and prognosis in thoracic neuroblastoma, explored the association between IDRFs and these features, and developed a nomogram to predict event-free survival (EFS).</p><p><strong>Methods: </strong>Clinical and prognostic data were collected retrospectively from pediatric patients diagnosed with thoracic neuroblastoma at Children's Hospital of Nanjing Medical University. Logistic regression was used to identify factors associated with IDRFs presence. Kaplan-Meier analysis assessed the effect of IDRFs on survival. Cox regression identified independent predictors of EFS, with model selection based on the Akaike Information Criterion (AIC). A nomogram was developed from these predictors and evaluated using calibration curves, time-dependent AUC curves, and decision curve analysis (DCA).</p><p><strong>Results: </strong>Total of 105 patients were included, 56 males, with a median diagnosis age of 55 (29, 77) months. The most common diagnosis was ganglioneuroblastoma intermixed (GNBi) (<i>n</i> = 49). Preoperative imaging found 93 IDRFs in 64 patients (60.95%), with infiltrative IDRFs being the most prevalent. 65.63% of patients had only one IDRF. Logistic regression showed that total protein (TP) ≥ 69.90 g/L and maximum tumor diameter (MTD) ≥ 5.50 cm independently predicted IDRFs presence. Among 85 patients with non-ganglioneuroma diagnoses, IDRFs did not significantly affect overall survival (OS) (<i>p</i> = 0.289), but were linked to worse EFS (<i>p</i> < 0.05). Cox regression identified infiltrative IDRFs, vascular IDRFs, LDH ≥273.00 U/L, and TP ≥ 70.00 g/L as independent risk factors for poor EFS. Nomogram based on these variables showed favorable discrimination with C-index = 0.77, good calibration, and clinical utility.</p><p><strong>Conclusions: </strong>Thoracic neuroblastoma had unique demographic and clinical features. TP levels and tumor size were associated with the presence of IDRFs, which significantly affected EFS. The nomogram accurately predicted EFS and held potential for clinical utility.</p>","PeriodicalId":12637,"journal":{"name":"Frontiers in Pediatrics","volume":"14 ","pages":"1793866"},"PeriodicalIF":2.0,"publicationDate":"2026-04-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13144076/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147836933","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Determinants of perceived stress among adolescents during wartime in Ukraine.","authors":"Tetyana Hariyan, Tymur Hariyan, Oksana Boyarchuk","doi":"10.3389/fped.2026.1838159","DOIUrl":"https://doi.org/10.3389/fped.2026.1838159","url":null,"abstract":"<p><strong>Introduction: </strong>Adolescents living in war-affected environments are exposed to prolonged and multifactorial stressors that may adversely impact their mental health. In Ukraine, the ongoing war has created conditions of chronic stress even among those not directly exposed to combat. Understanding the determinants of perceived stress in this population is essential for developing targeted preventive strategies. The aim of this study was to assess the level of perceived stress among adolescents and to examine its associations with demographic characteristics, sleep patterns, and physical activity.</p><p><strong>Methods: </strong>A cross-sectional study was conducted among adolescents to evaluate levels of perceived stress and its associated factors. Perceived stress was assessed using the 10-item Perceived Stress Scale (PSS-10). Data on sleep duration, sleep quality, physical activity, and selected health-related factors were collected using self-reported questionnaires. Correlation and comparative analyses were performed to evaluate associations between variables.</p><p><strong>Results: </strong>The study included 198 participants. A high prevalence of perceived stress was observed, with 52.0% of participants reporting moderate and 37.4% high levels of perceived stress, while only 10.6% demonstrated low stress levels. A significantly higher proportion of females reported high stress levels compared to males (21.5% vs. 5.1%, <i>p</i> < 0.0005). Poor sleep quality and difficulties initiating sleep were also associated with higher stress levels (<i>p</i> < 0.05). Adolescents sleeping more than 8 h per night were significantly more likely to report low stress levels compared to those sleeping less than 8 h (33.3% vs. 11.8%, <i>p</i> = 0.0006). Higher levels of physical activity were associated with lower stress (<i>p</i> < 0.05). Participants engaging in physical activity ≥5 days per week more frequently reported low stress compared to less active peers (32.7% vs. 9.4%, <i>p</i> < 0.001). No significant association was found between perceived stress and the presence of chronic diseases.</p><p><strong>Conclusion: </strong>Adolescents in a war-affected setting demonstrate a markedly elevated level of perceived stress. Sleep and physical activity are significantly but modestly associated with perceived stress. These findings support the integration of lifestyle-focused and psychosocial interventions into adolescent health strategies to help mitigate the long-term impact of chronic stress.</p>","PeriodicalId":12637,"journal":{"name":"Frontiers in Pediatrics","volume":"14 ","pages":"1838159"},"PeriodicalIF":2.0,"publicationDate":"2026-04-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13144074/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147836922","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Morphological and functional echocardiographic findings in pediatric patients diagnosed with hypermobile Ehlers-Danlos syndrome.","authors":"Walter Vignaroli, Gioia Mastromoro, Carolina Putotto, Filippo Camerota, Claudia Celletti, Mauro Celli, Luca Celli, Anna Zambrano, Francesco Carlomagno, Emanuele Monda, Annapaola Cirillo, Bruno Marino, Paolo Versacci","doi":"10.3389/fped.2026.1768520","DOIUrl":"https://doi.org/10.3389/fped.2026.1768520","url":null,"abstract":"<p><strong>Introduction: </strong>Hypermobile Ehlers-Danlos syndrome (hEDS) is a connective tissue disorder that may involve the cardiovascular system. While mitral valve prolapse (MVP) and aortic root dilatation (ARD) have been documented in adults with hEDS, their prevalence in children remains unclear.</p><p><strong>Methods: </strong>This study evaluated 28 pediatric patients with hEDS and 28 age- and body surface area-matched healthy controls using two-dimensional echocardiography, Doppler, Tissue Doppler Imaging (TDI), and Speckle Tracking Echocardiography (STE), including global longitudinal strain (GLS) analysis.</p><p><strong>Results: </strong>Nine hEDS patients (32%) exhibited MVP. Although aortic root and ascending aorta Z-scores remained within the normal range, they were significantly higher in hEDS patients compared to controls. TDI revealed reduced late diastolic septal myocardial velocities (<i>p</i> = 0.004). While left ventricular ejection fraction (EF) was preserved, hEDS patients showed significantly reduced GLS values (<i>p</i> < 0.001), indicating subclinical myocardial dysfunction. hEDS diagnosis correlated independently with reduced GLS (<i>p</i> = 0.01), but not with EF.</p><p><strong>Discussion: </strong>These findings suggest that cardiac alterations such as MVP can manifest in childhood and that subtle myocardial impairment can occur despite normal EF. STE and GLS analyses are valuable tools for the early detection of functional abnormalities. Routine cardiac follow-up in pediatric hEDS patients may help identify and monitor these early cardiovascular changes, potentially improving long-term outcomes.</p>","PeriodicalId":12637,"journal":{"name":"Frontiers in Pediatrics","volume":"14 ","pages":"1768520"},"PeriodicalIF":2.0,"publicationDate":"2026-04-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13144140/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147836975","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Etiology and clinical characteristics of infantile cholestasis: a single-center retrospective study of 326 cases.","authors":"Yun-Ping Tang, Xu-Xia Wei, Ning Xue, Hai-Ying Yang, Hua Li","doi":"10.3389/fped.2026.1843435","DOIUrl":"https://doi.org/10.3389/fped.2026.1843435","url":null,"abstract":"<p><strong>Objective: </strong>To retrospectively investigate the clinical characteristics and etiological spectrum of infantile cholestasis, with an emphasis on evolving diagnostic approaches.</p><p><strong>Methods: </strong>Clinical data of 326 infants diagnosed with infantile cholestasis at the Children's Hospital Affiliated to Shandong University from January 2020 to December 2025 were retrospectively analyzed. Etiological distribution was systematically examined. Serum bile acid profiling was performed for suspected bile acid synthesis defects, and genetic sequencing for unexplained or suspected genetic cholestasis.</p><p><strong>Results: </strong>Among 326 infants with infantile cholestasis, 56.7% presented with light- or clay-colored stools, 62.9% had hepatomegaly, and 8.3% had comorbidities. The etiological spectrum included biliary tract anomalies [50.6%, including 161 biliary atresia (BA)], genetic metabolic liver diseases (9.8%, <i>n</i> = 32), infectious causes (7.4%), drug-related causes (3.4%), idiopathic cholestasis (6.7%), other rare causes (0.9%), and undetermined etiology (21.2%). No significant differences in sex or age were observed between the genetic metabolic group (<i>n</i> = 32) and BA group (<i>n</i> = 161) (both <i>P</i> > 0.05). After excluding 165 surgical cases, genetic testing was performed in 55 of 161 remaining infants (34.2%), with pathogenic or likely pathogenic variants identified in 33 (60.0% detection rate) across 14 genes (e.g., <i>JAG1</i>, <i>SLC25A13</i>, <i>ABCC2</i>). In an exploratory subgroup analysis (genetic metabolic, <i>n</i> = 16; BA, <i>n</i> = 20), the BA subgroup showed significantly higher levels of matrix metalloproteinase-7 (MMP-7), direct bilirubin, and GGT (<i>P</i> = 0.002 for GGT), with no other significant differences between the two subgroups.</p><p><strong>Conclusion: </strong>The etiology of infantile cholestasis is complex and highly heterogeneous. Genetic testing improves the diagnostic yield of inherited metabolic liver diseases. Serum bile acid profiling provides metabolomic signatures for etiological differentiation. Conventional liver function tests combined with serum MMP-7 represent a simple, reliable, noninvasive approach for early differentiation of biliary atresia.</p>","PeriodicalId":12637,"journal":{"name":"Frontiers in Pediatrics","volume":"14 ","pages":"1843435"},"PeriodicalIF":2.0,"publicationDate":"2026-04-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13144150/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147836948","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Enteral nutrition versus immunomodulators for induction and maintenance of remission in pediatric Crohn's disease: a systematic review and network meta-analysis.","authors":"Jiajia Chen, Keying Yang, Qiongyue Zhang, Lijing Xiong","doi":"10.3389/fped.2026.1769493","DOIUrl":"https://doi.org/10.3389/fped.2026.1769493","url":null,"abstract":"<p><strong>Background: </strong>Enteral nutrition (EN) and immunomodulators are established therapies for pediatric Crohn's disease (CD), yet comparative effectiveness data remain limited.</p><p><strong>Objective: </strong>We conducted a network meta-analysis (NMA) to compare the efficacy and safety of EN therapies vs. immunomodulators and corticosteroids for remission induction and maintenance in pediatric CD.</p><p><strong>Methods: </strong>We systematically searched PubMed, Embase, Cochrane CENTRAL, and Web of Science from inception through October 2024. Randomized controlled trials (RCTs) and comparative observational studies evaluating exclusive enteral nutrition (EEN), partial enteral nutrition (PEN), Crohn's Disease Exclusion Diet plus PEN (CDED + PEN), supplemental enteral nutrition (SEN), corticosteroids (CS), azathioprine/6-mercaptopurine (AZA/6-MP), or methotrexate (MTX) were included. Primary outcomes were clinical remission and mucosal healing. Frequentist NMA was performed using random-effects models. Surface under the cumulative ranking curve (SUCRA) values determined treatment rankings.</p><p><strong>Results: </strong>Twenty studies (7 RCTs, 13 observational) comprising 1,182 patients were included. For clinical remission induction, EEN was significantly superior to CS [odds ratio [OR] 1.72; 95% confidence interval [CI] 1.18-2.52; 7 studies; <i>I</i> ² = 0%]. EEN demonstrated marked superiority for mucosal healing vs. CS (OR 7.55; 95% CI 3.59-15.88). SUCRA rankings for remission induction were: CDED + PEN (0.80), EEN (0.78), MTX (0.55), AZA/6-MP (0.47), CS (0.31), and PEN (0.08). For maintenance, AZA/6-MP was superior to placebo (OR 12.50; 95% CI 2.47-63.14). EN therapies exhibited favorable safety profiles with serious adverse event rates of 0%-3.1% compared with 15.1% for CS and 11.8% for AZA/6-MP.</p><p><strong>Conclusions: </strong>EEN and CDED + PEN are the most effective treatments for inducing clinical and endoscopic remission in pediatric CD, with superior safety profiles compared to pharmacological therapies. Immunomodulators remain essential for maintenance therapy. These findings support EN as first-line induction therapy in pediatric CD.</p><p><strong>Systematic review registration: </strong>https://www.crd.york.ac.uk/PROSPERO/view/CRD420261345561, PROSPERO CRD420261345561.</p>","PeriodicalId":12637,"journal":{"name":"Frontiers in Pediatrics","volume":"14 ","pages":"1769493"},"PeriodicalIF":2.0,"publicationDate":"2026-04-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13144049/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147836960","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}