BMC Pediatrics最新文献

{"title":"Long-term risk of allergic disorders following Kawasaki disease: a population-based cohort study.","authors":"Jae-Hee Seol, Lucy Youngmin Eun, Ji-Ho Lee","doi":"10.1186/s12887-025-05724-3","DOIUrl":"https://doi.org/10.1186/s12887-025-05724-3","url":null,"abstract":"<p><strong>Background: </strong>Kawasaki disease (KD) is an acute systemic vasculitis primarily affecting children under five years old. While its etiology remains unclear, immune dysregulation has been implicated, suggesting a potential link between KD and allergic diseases. Previous epidemiological studies have reported inconsistent findings regarding this association across different countries. This study aims to investigate the association between KD and allergic diseases in Korea.</p><p><strong>Methods: </strong>A nationwide population-based cohort study was conducted utilizing data from the Korean National Health Insurance database. KD cases were defined as admissions with a primary diagnosis of KD plus treatment records for immunoglobulin or aspirin. The control group had no KD diagnosis. Between 2008 and 2015, 41,806 KD cases were matched 1:4 with 163,548 controls using propensity score matching. The incidence and prevalence of asthma, rhinitis, atopic dermatitis, and urticaria from 2017 to 2021 were analyzed using hazard ratios (HRs) and odds ratios (ORs).</p><p><strong>Results: </strong>Mean age was 2.63 ± 1.84 years for KD patients and 2.64 ± 1.85 years for controls (P = 0.119). The proportion of females was 42.20% in the KD group and 42.60% in the control group (P = 0.145). KD patients showed significantly higher risks for developing rhinitis (HR 1.045, 95%CI 1.013-1.078) and urticaria (HR 1.139, 95%CI 1.085-1.197). However, no significant association was found in the incidence of asthma or atopic dermatitis. KD diagnosis at age 5 or older was associated with decreased risk of all allergic disorders. The prevalence of all allergic disorders studied was significantly higher in the KD group, with the strongest associations observed for rhinitis (OR 1.178, 95%CI 1.151-1.205) and urticaria (OR 1.192, 95%CI 1.155-1.230). Gender and urban living also influenced the prevalence of allergic disorders. A sensitivity analysis conducted to account for the COVID-19 pandemic showed consistent results, confirming the association between KD and increased risk and prevalence of allergic disorders.</p><p><strong>Conclusions: </strong>This study demonstrates a significant association between Kawasaki disease and increased risk of allergic disorders, particularly rhinitis and urticaria. These findings suggest potential shared pathogenesis between the two conditions and highlight the need for long-term monitoring of allergic conditions in KD patients.</p><p><strong>Clinical trial number: </strong>Not applicable.</p>","PeriodicalId":9144,"journal":{"name":"BMC Pediatrics","volume":"25 1","pages":"380"},"PeriodicalIF":2.0,"publicationDate":"2025-05-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12076958/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144076000","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":"Early detection of pulmonary vasculopathy in children with sickle cell disease by new echocardiography-based blood speckle technology.","authors":"Antoine Fakhry AbdelMassih, Fatima Nabil, Niveen Salama, Ilham Youssry","doi":"10.1186/s12887-025-05689-3","DOIUrl":"https://doi.org/10.1186/s12887-025-05689-3","url":null,"abstract":"<p><strong>Background: </strong>Right ventricle (RV) dysfunction because of pulmonary vasculopathy is the most common cause of death in sickle cell disease (SCD). This study aimed to explore the diagnostic accuracy of blood-speckle tracking and vortex detection in the early detection of pulmonary vasculopathy in patients with SCD.</p><p><strong>Methods: </strong>This study was conducted as a cross-sectional study including thirty patients and thirty controls. Patients with SCD were examined using 3D echocardiography to determine the presence of RV dysfunction, as a surrogate of pulmonary vasculopathy: in addition to blood speckle tracking echocardiography to determine the vortex timing in the RV and its presence or absence in the pulmonary artery. Patients' demographic and hematologic data were also retrieved from patients' files.</p><p><strong>Results: </strong>Pulmonary vortex formation was 100% sensitive in the detection of RV dysfunction. LDH (Lactate Dehydrogenase) was the only variable significantly different between cases with pulmonary vortex formation and those without (520 vs. 257, P < 0.001), LDH > 400 was 72% sensitive and 100% specific in the detection of pulmonary vortex formation.</p><p><strong>Conclusions: </strong>Pulmonary vortex formation was a sensitive indicator of RV dysfunction, thus suggesting its accuracy in the early detection of early pulmonary vascular changes in SCD. LDH as a marker of intravascular hemolysis, is a sensitive marker that can be used for risk stratification of SCD patients.</p>","PeriodicalId":9144,"journal":{"name":"BMC Pediatrics","volume":"25 1","pages":"382"},"PeriodicalIF":2.0,"publicationDate":"2025-05-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12076994/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144075997","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":"Promoting active behaviours in schoolchildren using the socio-ecological model in the physical education class: study protocol of the ACTIBESE project.","authors":"Fernando Rodríguez-Rodríguez, Carlos Cristi-Montero, Natalia Zurita-Corvalán, Caroline Brand, Jorge Mota, Palma Chillón, Anelise Reis Gaya, Javier Brazo-Sayavera, Nicolás Aguilar-Farias","doi":"10.1186/s12887-025-05719-0","DOIUrl":"https://doi.org/10.1186/s12887-025-05719-0","url":null,"abstract":"<p><strong>Background: </strong>The correlation between parental and child physical activity levels has been the subject of significant investigation. Existing research has illuminated this association, particularly emphasising its influence during childhood. However, as children transition into adolescence, this relationship appears to weaken. Despite the recognised importance of parental involvement in promoting physical activity among children, scant attention has been directed towards understanding effective strategies and interventions to bolster this relationship. Consequently, there exists a critical gap in the literature concerning actionable measures to enhance parent-child physical activity engagement during the pivotal stage of adolescence.</p><p><strong>Methods: </strong>The ACTIBESE project aims to assess and intervene in Chilean schoolchildren's active behaviours by examining the influence of personal, interpersonal, and school factors. This study includes a cross-sectional and intervention study involving schools in the Valparaíso and Araucanía Regions, representing urban and rural areas. Participants, divided into children (8-11 years old) and adolescents (12-16 years old), will undergo a comprehensive evaluation, including sociodemographic characteristics, physical activity (PA) levels, parental and peer support, teaching styles of the teachers, and school environment characteristics. In the intervention study, a 5-month continuous training program for teachers will be implemented, focusing on improving teaching styles and enhancing teaching competencies related to physical education and health classes. The research will use mixed models, ANCOVA, and logistic regressions for data analysis, network analysis, exploratory factors analysis, and structural equation modelling.</p><p><strong>Discussion: </strong>Through innovative research methods, interventions involving schoolteachers, and a diverse geographical scope, this project aims to make significant contributions to both the scientific understanding of promoting PA and public policy efforts aimed at improving the health and well-being of school-aged children. The ACTIBESE project's multifaceted approach, considering school, family, and peer influences, is well positioned to contribute significantly to promoting AB and addressing the alarming decline in PA levels among Chilean youth.</p>","PeriodicalId":9144,"journal":{"name":"BMC Pediatrics","volume":"25 1","pages":"381"},"PeriodicalIF":2.0,"publicationDate":"2025-05-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12077047/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144074988","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":"\"It's like going through life at a mediocre level\": a qualitative study of the meaning and impact of fatigue in children and young people with sickle cell disease.","authors":"Brenda Agyeiwaa Poku, Karl Michael Atkin, John David Grainger, Iyamide Thomas, Rachael Oshinbolu, Abubakar Mohammed, Edith Kyewalyanga, Susan Kirk","doi":"10.1186/s12887-025-05720-7","DOIUrl":"10.1186/s12887-025-05720-7","url":null,"abstract":"<p><strong>Background: </strong>Fatigue is increasingly recognised as a prevalent and debilitating symptom for children and young people (CYP) with long-term conditions (LTCs), significantly affecting their family, social and educational participation. In sickle cell disease (SCD), fatigue is the most frequently reported symptom, surpassing pain related to vaso-occlusion. However, understanding of fatigue's nature and impact on CYP with SCD remains limited. This qualitative study explores the meaning and consequences of fatigue for CYP with SCD to inform services, treatments and care.</p><p><strong>Methods: </strong>This exploratory research interviewed 12 CYP with SCD aged 12-23 years, five parents and ten healthcare professionals across England. Participants were recruited through convenience sampling from an NHS Trust, SCD-focused charities and social media. Data were generated using audio-recorded online semi-structured or art-elicitation interviews. Interviews were transcribed and analysed using coding, constant comparison and thematic categorisation to identify key themes.</p><p><strong>Results: </strong>Six thematic categories were constructed from the data: (1) constant state of reduced energy, (2) the daily struggle, (3) the invisibility of fatigue, (4) being socially isolated, (5) managing fatigue, and (6) the future while negotiating fatigue. SCD fatigue was seen as a persistent, inescapable daily struggle, with reduced energy for day-to-day activities. This was often unnoticed or misunderstood by others. It hindered YP's daily routines, caused frequent school absences, reduced social participation, and promoted social exclusion. To meet social expectations and avoid stigma, CYP described constantly pushing themselves to conceal their fatigue, exacerbating their difficulties with SCD. Fatigue was invisible in clinical settings, leading to a lack of standardised/formalised support and increasing uncertainties and precarity about the future. CYP and parents employed energy economisation and recharging strategies to cope with and control fatigue.</p><p><strong>Conclusions: </strong>Fatigue dominates CYP's experience of living with SCD, significantly impacting their physical, social and educational functioning and leading to isolation and stigma. Often overlooked in clinical settings, addressing fatigue should be integral to SCD care and research. This includes incorporating fatigue assessments, developing targeted self-management programmes, and furthering research on its management. The findings emphasise recognising fatigue as a primary symptom in CYP with LTCs, given its severe impact on social and educational development and future stability.</p><p><strong>Trial registration: </strong>Not Applicable.</p>","PeriodicalId":9144,"journal":{"name":"BMC Pediatrics","volume":"25 1","pages":"379"},"PeriodicalIF":2.0,"publicationDate":"2025-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12070639/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143958669","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":"The Chinese adverse prognosis of very preterm infants (CARE-Preterm) cohort: study design and baseline characteristics for a prospective multicenter cohort study.","authors":"Ranran Shi, Xiaoyu Dong, Li Wang, Wenwen Zhang, Simmy Reddy, Yonghui Yu","doi":"10.1186/s12887-025-05722-5","DOIUrl":"10.1186/s12887-025-05722-5","url":null,"abstract":"<p><strong>Background: </strong>As the increasing survival of preterm infants bearing lower gestational age (GA) and birth weight (BW), new challenges have emerged regarding their management and prognosis. In low- and middle-income countries, there is notable absence of prospective multicenter cohorts to accurately reflect their real treatment capacity for these preterm infants. This cohort study aims to fill this gap by investigating the impact of perinatal management on the prognosis of preterm infants in Northern China.</p><p><strong>Methods: </strong>The Chinese Adverse Prognosis of Very Preterm infants (CARE-Preterm) cohort study is a prospective, multicenter, longitudinal, and open cohort study based on the Sino-northern Neonatal Network (SNN) since 2018, covering 60 neonatal intensive care units from 8 densely-populated provinces and autonomous regions in Northern China. All very preterm infants (VPIs) born with GA < 32 weeks or very low birth weight infants (VLBWI) born with BW < 1500 g admitted alive to the participating units are continuously enrolled from January 1, 2018. Baseline clinical data and biological samples are longitudinally collected from the perinatal period to discharge.</p><p><strong>Results: </strong>From January 1, 2018, to December 31, 2022, approximately 10,447 infants were included and 9325 infants were discharged alive. Notably,1472 (14.09%) were born with GA < 28 weeks, and 1566 (14.99%) with BW < 1000 g. Among the 9325 survivors, the smallest GA and BW were 23^+ 3 weeks and 450 g, respectively. The main findings of this cohort study highlight substantial improvements in perinatal medicine treatment capabilities and current treatment bottlenecks.</p><p><strong>Conclusion: </strong>This cohort study provides crucial insight into updated real-world data from low- and middle-income countries, helping to identify treatment bottlenecks and improve both the survival rate and life quality for preterm infants. Furthermore, it is expected to serve as a reference for establishing population-based cohort studies in other low and middle-income countries.</p><p><strong>Trial registration: </strong>The current projects based on this cohort study have been registered in the Chinese Clinical Trial Registration Center (Registration number: ChiCTR1900025234, ChiCTR1900020861, ChiCTR2000037918, ChiCTR2000029162, ChiCTR2100053780 and ChiCTR2200066764).</p>","PeriodicalId":9144,"journal":{"name":"BMC Pediatrics","volume":"25 1","pages":"377"},"PeriodicalIF":2.0,"publicationDate":"2025-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12070726/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143960911","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":"The association between smartphone use and myopia progression in children: a prospective cohort study.","authors":"Jing Li","doi":"10.1186/s12887-025-05715-4","DOIUrl":"10.1186/s12887-025-05715-4","url":null,"abstract":"<p><strong>Background: </strong>The increasing myopia of children has sparked speculations on whether the use of smartphones can accelerate this rate. This study aims to identify possible predictors for myopic progression in children with smartphones over a period of two years.</p><p><strong>Methods: </strong>This prospective cohort study recruited 523 children aged 6 to 14 years. A comprehensive eye examination was performed at baseline and at 6, 12, and 24 months, which included spherical equivalent refractive error (with cycloplegia) and axial length. Smartphones usage patterns were traced using mobile usage monitoring app. Outdoor activities, sleep duration, and parental history of myopia were documented with structured questionnaires. Data on myopic progression associated with smartphone use are presented with multivariate regression analyses.</p><p><strong>Results: </strong>It demonstrated that daily usage of smartphones was positively associated with the progression of myopia (5.1 ± 1.2 vs. 3.4 ± 1.0 h, p < 0.001). Increased time of outdoor activity (1.2 ± 0.6 vs. 2.1 ± 0.8 h/day, p < 0.001) and longer distances of screens (25.8 ± 5.4 vs. 31.4 ± 6.2 cm, p < 0.001) were inversely related to myopic progression. Of importance is that children whose parents experienced myopia exhibited higher progression rates compared to those who did not (65.5% vs. 44.4%, p < 0.001).</p><p><strong>Conclusion: </strong>This study indicated that daily duration of smartphones use, time of outdoor activity, distance of screen, and parental myopia are predictors of childhood myopic progression.</p>","PeriodicalId":9144,"journal":{"name":"BMC Pediatrics","volume":"25 1","pages":"378"},"PeriodicalIF":2.0,"publicationDate":"2025-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12070634/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143974506","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":"Lipid metabolism dysregulation in umbilical cord plasma of newborns from mothers with preeclampsia is associated with neonatal physical parameters at birth.","authors":"Jing Ding, Yihan Xiao, Jie Fu, Qionghui Huang, Zijun Ma, Chao Zhang, Yan Wang, Guoli Liu, Hongkun Wu, Yifang Yuan","doi":"10.1186/s12887-025-05744-z","DOIUrl":"10.1186/s12887-025-05744-z","url":null,"abstract":"<p><strong>Background: </strong>Preeclampsia is linked to fetal growth restriction and may have long-term implications for the offspring. Despite its significance, the fundamental mechanisms remain inadequately elucidated. The objective of this investigation was to undertake an untargeted lipidomics analysis of umbilical cord plasma, with the intention of investigating lipidomic profile alterations in newborns of mothers with preeclampsia and evaluating the associations between lipidomic patterns and neonatal physical parameters at birth.</p><p><strong>Methods: </strong>25 newborns from mothers with preeclampsia (PE group) and 25 newborns from healthy mothers (control group) were involved in the present investigation. Untargeted lipidomics was performed using ultra-performance liquid chromatography-tandem mass spectrometry (UPLC-MS/MS) to contrast the lipid compositions present in umbilical cord plasma. Co-expression correlation analysis was performed to explore the relationships between lipidomic patterns and neonatal weight and length percentile at birth.</p><p><strong>Results: </strong>Marked discrepancies in lipid metabolism profiles were detected in the comparison of the PE and control groups. In total, 364 separate lipids were noted, with AcylGlcADG (20:3-22:6-22:6) and GM3(d39:1) exhibiting the most significant decreases. Conversely, Cer-NS (d20:1-24:0) and DGTS (2:0-19:0) displayed the most significant increases. The primary lipid metabolic pathways altered in newborns from mothers with preeclampsia were enriched in choline and glycerophospholipid metabolic processes. Additionally, 20 distinct lipids exhibited significant associations with neonatal birth weight percentile between the two groups, while 21 distinct lipids showed significant associations with neonatal birth length percentile.</p><p><strong>Conclusions: </strong>Lipid profile disorders were identified in the umbilical cord plasma of infants born to mothers with preeclampsia, and the metabolic disturbances identified in this group correlated with neonatal physical parameters at birth. These findings suggest that lipidomic disorders in newborns from preeclamptic mothers may correlate with intrauterine growth outcomes.</p>","PeriodicalId":9144,"journal":{"name":"BMC Pediatrics","volume":"25 1","pages":"376"},"PeriodicalIF":2.0,"publicationDate":"2025-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12070651/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143965174","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":"Epidemiology of respiratory infections during the different levels of non-pharmaceutical interventions.","authors":"Ting Shi, Linlin Huang, Jianmei Tian","doi":"10.1186/s12887-025-05723-4","DOIUrl":"https://doi.org/10.1186/s12887-025-05723-4","url":null,"abstract":"<p><strong>Objective: </strong>This study aimed to describe epidemiological changes of common respiratory pathogens in children with acute respiratory infections (ARIs) during the different levels of non-pharmaceutical interventions(NPIs).</p><p><strong>Methods: </strong>In this retrospective study, we analyzed the prevalence of 12 respiratory pathogens from September 2021 through February 2024 in all inpatients with ARIs admitted to Children's Hospital of Soochow University(SCH). We compared the detection rates of these pathogens at different levels of public NPI measures.</p><p><strong>Results: </strong>In total, 27,851 respiratory samples were analyzed, including 15,231 boys and 12,620 girls. Under strict NPIs, inpatients with ARIs were more commonly boy and infants (p < 0.01). However, after the discontinuation of NPIs, they were more commonly children over 6 years of age (p < 0.01). Under the strict NPIs, the most detected respiratory pathogen was HRV(23.6%), followed by HRSV(16.0%), HMPV(10.8%) and HPIV(10.4%). But after the abolition strict NPIs, the most common respiratory pathogen was MP(35.9%), followed by HRV(19.7%), HRSV(17.5%) and HPIV (6.6%). Moreover, the number and detection rate of MP and ADV had significantly increased and remained at a high level (P<0.05). In addition, the number of HRSV detection increased and the epidemic season has changed(transitioning from winter to summer), and detection rate of multiple pathogens was more high after the abolition NPIs (P < 0.01).</p><p><strong>Conclusions: </strong>Withdrawal of major measures, the disease burden of ARIs in children in Suzhou has sharply increased, mainly attributed to MP, and mixed infections were more common.</p>","PeriodicalId":9144,"journal":{"name":"BMC Pediatrics","volume":"25 1","pages":"375"},"PeriodicalIF":2.0,"publicationDate":"2025-05-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12066070/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143964223","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":"Application of fibrobronchoscopy-guided aortic suspension in pediatric patients with bronchial compression resulting from repair of coarctation of the aorta: a case report.","authors":"Huayi Tan, Xinke Zou, Ke Gong, Yifeng Yang, Zhongshi Wu, Jijia Liu, Sijie Wu","doi":"10.1186/s12887-025-05717-2","DOIUrl":"https://doi.org/10.1186/s12887-025-05717-2","url":null,"abstract":"<p><strong>Background: </strong>Coarctation of the aorta (CoA) is one of the more prevalent congenital heart diseases (CHD). The conventional treatment approach involves surgical correction of the coarctation. This procedure, however, can be associated with several complications, such as re-stenosis, aneurysm formation and pseudoaneurysm. One rare but significant complication is left bronchial compression, which may result from anterior displacement of the thoracic aorta or compression of the trachea by adherent tissue.</p><p><strong>Case presentation: </strong>We present a case of a 7-month-old postoperative CoA patient who developed stenosis due to compression of the left main bronchus by the thoracic aorta. After comprehensive evaluation, we utilized a fiberoptic bronchoscope to accurately expose the site of aortic compression and subsequently performed bronchial release and aortic suspension procedures to alleviate the symptoms. The child demonstrated a satisfactory recovery following the surgical intervention. This successful case provides valuable insights for managing similar cases in the future.</p><p><strong>Conclusions: </strong>Computing tomography (CT) airway reconstruction enables precise diagnosis of tracheal stenosis secondary to aortic arch pathology or coarctation repair. Intraoperative fiberoptic bronchoscopy provides real-time anatomical localization of stenotic segments, warranting standardization in high-risk aortic surgeries to ensure airway patency. Three-dimensional airway modeling should be incorporated into perioperative reassessment and longitudinal follow-up protocols.</p>","PeriodicalId":9144,"journal":{"name":"BMC Pediatrics","volume":"25 1","pages":"373"},"PeriodicalIF":2.0,"publicationDate":"2025-05-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12066068/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143971495","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":"Long-term prognosis of 47 pediatric patients with Blau syndrome in China.","authors":"Xinwei Shi, Jianghong Deng, Junmei Zhang, Xiaozhen Zhao, Yinan Zhao, Li Li, Fengqiao Gao, Weiying Kuang, Jiang Wang, Xiaohua Tan, Chao Li, Shipeng Li, Caifeng Li","doi":"10.1186/s12887-025-05584-x","DOIUrl":"https://doi.org/10.1186/s12887-025-05584-x","url":null,"abstract":"<p><strong>Objectives: </strong>Blau syndrome (BS) is a rare autoinflammatory disease characterized by a clinical triad of uveitis, dermatitis and arthritis. The aim of our study was to summarize organ involvement, predict disease prognosis and evaluate treatment response.</p><p><strong>Methods: </strong>Clinical data of 47 Chinese children who were diagnosed with Blau syndrome in Beijing Children's hospital, Capital Medical University was retrospectively analyzed. Direct sequencing of NOD2 gene was performed by sanger sequencing. Data were analyzed through SPSS 21.0. A Bayesian network was constructed to integrate prediction algorithms of genetic mutations and clinical manifestations, exploring the complex relationship between genotype and phenotype through R (Version 4.4.1, R Core Development Team). P value < 0.05 was significant.</p><p><strong>Results: </strong>The 47 patients included 26 males and 21 females. Median age of disease onset was 13.64 months, ranging from 1 to 51 months. At baseline, incidence of fever, arthritis, rash, dermatitis and uveitis were 34%, 93.6%, 72.3% and 31.9%. Nearly 30% patients (14 patients) presented with characteristic triad. Incidence of vasculitis and interstitial lung disease were 27.7% and 17.0%, respectively. Inflammatory indices (e.g., erythrocyte sedimentation rate and C reactive protein) were above normal range. Twelve different NOD2 mutations were identified. R334Q was associated with arthritis, rash, uveitis and fever, whereas R334W was associated with arthritis, rash and fever. Approximately 95.7% patients (45 patients) were treated with combination of prednisolone and methotrexate and 42.6% patients (20 patients) were treated with tumor necrosis factor inhibitors. At the most recent follow-up visit, 34 patients (72.3%) achieved disease control. Patients treated with TNF-α inhibitors had a higher remission rate.</p><p><strong>Conclusions: </strong>Clinical manifestations of Blau syndrome in this study were various. TNF-α inhibitors were effective in inducing remission rate of Blau syndrome.</p>","PeriodicalId":9144,"journal":{"name":"BMC Pediatrics","volume":"25 1","pages":"374"},"PeriodicalIF":2.0,"publicationDate":"2025-05-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12066035/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143980217","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}