Pediatrics International最新文献

{"title":"Safety of transporting ventilated children by skilled personnel: A multicenter prospective study.","authors":"Masahiro Nozawa, Noriyuki Kaku, Naoki Shimizu, Yosuke Nakabayashi, Mamoru Ayusawa, Hiroyuki Matsuura, Ryugo Hiramoto, Takashi Hamazaki, Yasuhiro Takeshima","doi":"10.1111/ped.70187","DOIUrl":"https://doi.org/10.1111/ped.70187","url":null,"abstract":"<p><strong>Background: </strong>A reliable interfacility transport system to safely transfer patients from the initial care setting to the pediatric intensive care unit (PICU) is essential to improve outcomes. This study aimed to evaluate whether using skilled versus unskilled personnel reduces the incidence of transport-related adverse events in the transportation of pediatric patients on ventilators in Japan.</p><p><strong>Methods: </strong>This multicenter, prospective, observational cohort study enrolled patients aged <16 years transported on ventilators to a Japanese PICU between August 2020 and July 2021. Transport personnel were classified as skilled (those working in PICUs or pediatric transport teams or with ≥1 year of experience in those facilities) or unskilled. Adverse airway events were investigated, and the equipment and medications used during transportation and neurological outcomes between the two groups were compared.</p><p><strong>Results: </strong>The study included 294 patients (171 with skilled, 123 with unskilled personnel). The skilled personnel had a lower incidence of adverse airway events than the unskilled group (9.9% vs. 26.0%, p < 0.001). The skilled personnel used end-tidal CO₂ monitors, heat and moisture exchangers, analgesics, sedatives, and muscle relaxants more frequently than the unskilled personnel. No significant differences were observed between the two groups in the changes in Pediatric Cerebral Performance Category or Functional Status Scale scores from PICU admission to discharge.</p><p><strong>Conclusions: </strong>The results of this study highlight the importance of experience and specialized training in reducing the adverse events. Establishing a transport system with skilled personnel is important when considering measures to consolidate critically ill pediatric patients in PICUs.</p>","PeriodicalId":20039,"journal":{"name":"Pediatrics International","volume":"67 1","pages":"e70187"},"PeriodicalIF":0.9,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144964927","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Severe hepatic steatosis contributes to liver dysfunction in non-obese patients with insulin-resistant diabetes mellitus after hematopoietic stem cell transplantation.","authors":"Haruki Yamano, Reiko Kagawa, Yoko Saito, Ryosei Iemura, Eriko Adachi, Gau Maki, Takeru Yamauchi, Ryuichi Nakagawa, Akito Sutani, Shigeru Takishima, Fukiko Kitahata, Miyako Murakawa, Takeshi Isoda, Takahiro Kamiya, Satoshi Okada, Tomohiro Morio, Kei Takasawa, Kenichi Kashimada","doi":"10.1111/ped.70192","DOIUrl":"10.1111/ped.70192","url":null,"abstract":"<p><strong>Background: </strong>Non-obese survivors of childhood hematopoietic stem cell transplantation (HSCT) often exhibit severe insulin resistance and mild hepatic dysfunction. Although the exact pathophysiology remains unclear, one possible explanation is the increasingly recognized condition known as HSCT-associated partial lipodystrophy. In lipodystrophy, hepatic dysfunction is characterized by fibrosis and severe steatosis, resembling nonalcoholic steatohepatitis. Accordingly, detailed hepatic assessment would improve our overall understanding of the condition. This study aimed to evaluate hepatic dysfunction using a non-invasive method (FibroScan) in patients who developed glucose intolerance post-HSCT.</p><p><strong>Methods: </strong>Fourteen non-obese childhood HSCT survivors from two institutions underwent FibroScan. They were classified according to the presence or absence of diabetes mellitus (DM) with insulin resistance (HOMA-IR >2.5 or insulin therapy >1.5 U/kg/day). FibroScan provided controlled attenuation parameter (CAP) for hepatic steatosis and liver stiffness measurements (LSM) for fibrosis. Serum hepatic fibrosis markers (collagen type IV and hyaluronic acid) were also measured.</p><p><strong>Results: </strong>Seven patients developed insulin-resistant DM while seven had normal glucose/lipid metabolism. All DM patients showed \"Dunnigan\"-type subcutaneous fat distribution. CAP values were higher in the DM group [307 (261.0-343.5) vs. 237 (216.5-271.0), p = 0.041], as were LSM scores [6.7 (5.9-9.9) vs. 4.1 (3.5-4.7), p = 0.007], indicating more severe steatosis and increased liver stiffness. However, serum hepatic fibrosis markers did not differ significantly.</p><p><strong>Conslusions: </strong>Non-obese HSCT survivors with insulin-resistant DM exhibited severe hepatic fat accumulation and progression of liver fibrosis attributable to lipodystrophy. FibroScan may have potential utility for monitoring liver health in this population.</p>","PeriodicalId":20039,"journal":{"name":"Pediatrics International","volume":"67 1","pages":"e70192"},"PeriodicalIF":0.9,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145114009","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Evaluation of clinical and genetic characteristics of cystic fibrosis patients in the Southeastern Anatolia of Turkey.","authors":"Elif Arik, Ozlem Keskin, Murat Korkmaz, Velat Şen, Melih Hangül, Mahmut Cesur, Ercan Kucukosmanoglu, Sibel Oğuzkan Balcı, Hadice Selimoğlu Şen, Suat Savaş, İlkay Doğan","doi":"10.1111/ped.70197","DOIUrl":"10.1111/ped.70197","url":null,"abstract":"<p><strong>Background: </strong>Due to the amazing developments in modulatory treatments, genetic analysis of cystic fibrosis (CF) patients has become even more important. More than 2000 disease-causing variants of the cystic fibrosis transmembrane conductance regulator (CFTR) gene have been found, and their ethnic and geographical distributions vary. We aimed to present the first genetic data from the Southeastern Anatolia region of Turkey and evaluate patients' clinical and genetic characteristics and identify modulatory drugs covering a wider range of mutations by detecting and reporting new mutations.</p><p><strong>Methods: </strong>Our study included 337 CF patients from three CF reference centers in the Southeastern Anatolia region.</p><p><strong>Results: </strong>Ninety-one distinct mutations and four significant deletions were identified by analyzing CFTR mutations. The most prevalent mutation in our research was F508del (8.92%); the second most prevalent mutation was 2183AA->G, and the third most prevalent mutation was R347P. Additionally, a novel mutation (V1160X) was identified in two siblings. Only 33.5% of our patients qualified for CFTR modulator medication therapy.</p><p><strong>Conclusion: </strong>This study elucidates the diverse nature of CFTR mutations in the Turkish population. The heterogenous genetic pool of the Southeastern Anatolia region is more similar to Mesopotamia than to other regions of our country, and receives immigration from the East. Detection and reporting of novel mutations and CFTR mutations that occur at very low frequencies from different populations living in various geographical areas are essential for identifying modulatory medicines that cover a broader range of mutations and also help genetic diagnosis of CF in newborn screening.</p>","PeriodicalId":20039,"journal":{"name":"Pediatrics International","volume":"67 1","pages":"e70197"},"PeriodicalIF":0.9,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145070103","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The role of urinary chloride concentrations in the determination of kidney injury in children.","authors":"Emre Leventoğlu, Furkan Çağrı Kavas, Bahar Büyükkaragöz","doi":"10.1111/ped.70130","DOIUrl":"10.1111/ped.70130","url":null,"abstract":"<p><strong>Background: </strong>Early stages of chronic kidney disease (CKD) are usually asymptomatic, but kidney damage that starts in childhood may persist until adulthood and can lead to serious morbidity and mortality in the following years. Therefore, preventive practices that can slow the progression of kidney damage are strongly needed. In this study, we evaluated the relationship of urinary chloride concentrations and fractional excretion of chloride (FeCl) with proteinuria and estimated glomerular filtration rate (eGFR).</p><p><strong>Methods: </strong>This study is a retrospective descriptive study. Patients were divided into subgroups based on eGFR values and proteinuria status. It was investigated whether there was a difference between the groups in terms of urine chloride and FeCI levels.</p><p><strong>Results: </strong>A total of 176 patients with a mean age of 11.5 ± 5.0 (2-18) years were included in the study. The most common primary diagnosis was CAKUT (n = 56, 31.8%), followed by glomerular diseases (n = 45, 25.5%). The number of patients with eGFR <90 mL/min/1.73 m² was 20 (11.4%). Urinary chloride levels were significantly lower in patients with proteinuria compared to those without (95.5 ± 74.8 mEq/L vs. 146.7 ± 85.7 mEq/L, p < 0.001). Regression analysis showed that higher chloride and FeCI levels were associated with less proteinuria. Urinary chloride was lower in stage 3 CKD compared to stage 2 (34.20 ± 20.2 mEq/L vs. 70.9 ± 60.5 mEq/L, p = 0.087), while FeCI was higher in patients with decreased eGFR (1.73 ± 0.91%, p < 0.001).</p><p><strong>Conclusion: </strong>Strategies towards increasing urinary chloride excretion may help to slow the progression of CKD by providing a renoprotective effect via tubuloglomerular feedback (TGF), which inhibits the increase in glomerular pressure.</p>","PeriodicalId":20039,"journal":{"name":"Pediatrics International","volume":"67 1","pages":"e70130"},"PeriodicalIF":0.9,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145070178","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Challenges and support needs for flood evacuation of children with medical complexity in Japan: A qualitative study.","authors":"Asumi Yamamoto, Naomi Komori, Yasuhiro Kotera, Hirotomo Miyatake, Makoto Kosaka, Akihiko Ozaki, Masaharu Tsubokura, Hiroyuki Beniya","doi":"10.1111/ped.70165","DOIUrl":"https://doi.org/10.1111/ped.70165","url":null,"abstract":"<p><strong>Background: </strong>The increasing frequency and scale of flood disasters pose significant challenges for evacuating children with medical complexity. This study aimed to clarify the challenges and necessary support for safely evacuating these children in Japan during floods.</p><p><strong>Methods: </strong>A qualitative interview-based study was conducted with 10 caregivers of children with medical complexity. Interview data were analyzed using inductive thematic analysis following Braun and Clarke's six-phase approach.</p><p><strong>Results: </strong>Four main themes related to challenges were identified: (1) Shortage of support personnel, (2) Inadequate response from the administration, (3) Specificity of medical needs, and (4) Limitations in coordination and communication. Four themes related to support needs were also identified: (1) Assistance, (2) Support by the administration, (3) Medical care, and (4) Information. These findings highlight the multifaceted nature of evacuation challenges for children with medical complexity, emphasizing the need for tailored support systems that address both social and medical aspects of disaster preparedness.</p><p><strong>Conclusion: </strong>This study highlights the complex challenges in evacuating children with medical complexity during floods in Japan, addressing the increasing concerns raised by more frequent and severe flood disasters. These findings emphasize the urgent need for comprehensive community-based disaster preparedness involving various stakeholders, improved coordination, and tailored support systems for vulnerable children.</p>","PeriodicalId":20039,"journal":{"name":"Pediatrics International","volume":"67 1","pages":"e70165"},"PeriodicalIF":0.9,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145075851","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Evaluation of the effects of antiepileptic monotherapy on pubertal hormones in adolescent girls with epilepsy.","authors":"Ali Nazım Güzelbağ, Alkım Öden Akman, Pınar Kocaay, Altan Güneş, Murat Kızılgün, Berivan Güzelbağ, Zeynep Selen Aslan","doi":"10.1111/ped.70209","DOIUrl":"https://doi.org/10.1111/ped.70209","url":null,"abstract":"<p><strong>Background: </strong>Epilepsy often begins in childhood or adolescence, a period marked by significant hormonal changes. Antiepileptic drugs (AEDs) like valproic acid (VPA) and levetiracetam (LEV) are commonly used, but VPA is associated with notable endocrine side effects, particularly in adolescent girls. This raises concerns about drug choice during puberty.</p><p><strong>Objective: </strong>To compare the effects of VPA and LEV monotherapy on pubertal hormone profiles, menstrual characteristics, physical development, and gynecologic ultrasound findings in adolescent girls with primary epilepsy.</p><p><strong>Methods: </strong>This prospective, cross-sectional study included 61 adolescent girls (16 on VPA, 15 on LEV, 30 controls) at a tertiary hospital from January 2018 to January 2019. Participants underwent anthropometric measurements, hormonal evaluations (FSH, LH, estradiol, testosterone, SHBG, TSH, 17-OH progesterone, DHEASO₄), menstrual assessments, and pelvic ultrasound. Statistical analysis was performed with a significance level of p < 0.05.</p><p><strong>Results: </strong>VPA users had significantly higher TSH levels than LEV users and controls (p = 0.036), suggesting possible subclinical thyroid dysfunction. They also had lower 17-OH progesterone and DHEASO₄ levels (p = 0.008 and p = 0.022, respectively). No significant differences were observed between groups in weight, height, BMI, pubertal hormone levels, menstrual cycle characteristics, or ultrasound findings. Treatment duration did not significantly affect hormone levels.</p><p><strong>Conclusion: </strong>VPA appears to negatively impact thyroid and adrenal hormones in adolescent girls, whereas LEV has a safer endocrine profile. Given the hormonal sensitivity during puberty, LEV may be a safer alternative for adolescent girls with epilepsy to reduce potential endocrine disturbances.</p>","PeriodicalId":20039,"journal":{"name":"Pediatrics International","volume":"67 1","pages":"e70209"},"PeriodicalIF":0.9,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145225659","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Temporal trends in pediatric intestinal intussusception following the COVID-19 outbreak in Seoul, Korea.","authors":"Jung Hee Hong, Kiook Baek","doi":"10.1111/ped.70224","DOIUrl":"10.1111/ped.70224","url":null,"abstract":"<p><strong>Background: </strong>The COVID-19 pandemic led to significant epidemiological shifts in various diseases due to reduced interpersonal contact. This study examined changes in pediatric intestinal intussusception incidence, a disease with unclear etiology, during the pandemic.</p><p><strong>Methods: </strong>Weekly intussusception cases in children ≤10 years old in Seoul (2009-2022) were collected from the Health Insurance Review and Assessment Service (HIRA). Trends were analyzed using spline functions, and breakpoints were identified via student methods. Bayesian Structural Time Series (BSTS) models assessed incidence changes relative to predicted values. Cases were stratified by surgical intervention, and the complicated-to-uncomplicated case ratio was evaluated.</p><p><strong>Results: </strong>A sharp decline in intussusception incidence was observed around 2020, with breakpoints aligning with the first reported COVID-19 case. Post-breakpoint, total cases decreased by 58.8% (95% CrI: -79.0%, 7.8%), with reductions of 62.4% (95% CrI: -78.9%, -20.2%) for uncomplicated cases and 51.1% (95% CrI: -62.6%, -33.8%) for complicated cases. The complicated-to-uncomplicated ratio increased by 43.3% (95% CrI: 13.0%, 87.1%). When analyzing only the post-COVID-19 period, total and uncomplicated cases increased by 23.1% (95% CrI: 5.7%, 45.5%) from May 2022, but the change in ratio was not statistically significant.</p><p><strong>Conclusion: </strong>Pediatric intussusception incidence significantly declined following COVID-19 onset, supporting a link between disease occurrence and contact or infectious exposure.</p>","PeriodicalId":20039,"journal":{"name":"Pediatrics International","volume":"67 1","pages":"e70224"},"PeriodicalIF":0.9,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12495373/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145225703","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Neuroendocrine cell hyperplasia of infancy: A noteworthy Japanese case report.","authors":"Keiko Funata, Naotaka Tamai, Chizuko Haga, Takako Yoshioka, Goro Koinuma","doi":"10.1111/ped.15895","DOIUrl":"https://doi.org/10.1111/ped.15895","url":null,"abstract":"","PeriodicalId":20039,"journal":{"name":"Pediatrics International","volume":"67 1","pages":"e15895"},"PeriodicalIF":1.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143987406","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Immaturity of gut functions and induction of tolerance during early infancy.","authors":"Yoshikazu Ohtsuka, Toshiaki Shimizu","doi":"10.1111/ped.15855","DOIUrl":"10.1111/ped.15855","url":null,"abstract":"<p><p>Tolerance to foreign molecules is primarily induced through three pathways: anergy, active suppression, and clonal deletion. The immaturity of gut functions, including digestion and barrier protection against foreign molecules during early infancy, is closely linked to the induction of tolerance. A significant number of undigested peptides can pass through leaky gut walls during this period, making it an opportune time to introduce active suppression and clonal deletion in the intestine. Increased production and expression of TGF-β and CXCL13 with lymphoid hyperplasia in early infancy can be a convenient way to introduce tolerance and immunoglobulin A (IgA) synthesis against food and other antigens to protect hosts from allergic diseases. In this article we will discuss the immaturity of gut functions and mucosal immunity in infancy and its relationship to the introduction of tolerance.</p>","PeriodicalId":20039,"journal":{"name":"Pediatrics International","volume":"67 1","pages":"e15855"},"PeriodicalIF":1.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142910133","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Acrodermatitis-like rash induced by infliximab in an immunoglobulin-resistant case of Kawasaki disease.","authors":"Yusuke Morita, Daisuke Matsubara, Mitsuru Seki, Takaomi Minami, Atsuko Sato, Mayumi Komine, Toshihiro Tajima","doi":"10.1111/ped.70062","DOIUrl":"https://doi.org/10.1111/ped.70062","url":null,"abstract":"","PeriodicalId":20039,"journal":{"name":"Pediatrics International","volume":"67 1","pages":"e70062"},"PeriodicalIF":1.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143987405","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}