Clinical and Experimental Pediatrics最新文献

{"title":"Impact of hematopoietic stem cell transplantation on growth outcomes in mucopolysaccharidosis: a systematic review.","authors":"Farzaneh Abbasi, Asal Khalili Dehkordi, Reihaneh Mohsenipour","doi":"10.3345/cep.2024.01725","DOIUrl":"https://doi.org/10.3345/cep.2024.01725","url":null,"abstract":"<p><p>Mucopolysaccharidosis (MPS) is a group of genetic disorders characterized by defective lysosomal enzyme activity that can result in growth abnormalities and other complications. Hematopoietic stem cell transplantation (HSCT), especially bone marrow transplantation (BMT), aims to restore enzyme function and improve growth parameters in patients with MPS. This systematic review evaluates the impact of HSCT on growth outcomes, including height, weight, body mass index (BMI), head circumference, and pubertal development, in pediatric patients with MPS. Using the Preferred Reporting Items for Systematic reviews and Meta-Analyses guidelines, we systematically searched the PubMed, Embase, Scopus, and Web of Science databases. The retrieved studies focused on the growth outcomes of patients with MPS treated with HSCT emphasizing the role of BMT. Study quality was assessed using the Newcastle-Ottawa Scale and Joanna Briggs Institute checklist. The study protocol was registered in PROSPERO (registration no. CRD42024571488). These findings indicate that HSCT improves height, weight, and BMI, and better outcomes were observed in patients who underwent early transplantation. However, many patients still experience declining height Z-scores, resulting in short stature in adulthood, an elevated BMI, disproportionate head growth, and, in some cases, precocious puberty or pubertal arrest. Therefore, ongoing monitoring and personalized care are necessary to address these long-term growth challenges.</p>","PeriodicalId":36018,"journal":{"name":"Clinical and Experimental Pediatrics","volume":" ","pages":""},"PeriodicalIF":3.2,"publicationDate":"2025-03-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143626403","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Hematopoietic stem cell transplantation in pediatric patients with type VI mucopolysaccharidosis.","authors":"Vedat Uygun, Koray Yalçın, Hayriye Daloğlu, Seda Öztürkmen, Suna Çelen, Suleimen Zhumatayev, Gülsün Karasu, Akif Yeşilipek","doi":"10.3345/cep.2024.02033","DOIUrl":"https://doi.org/10.3345/cep.2024.02033","url":null,"abstract":"<p><strong>Background: </strong>It is uncertain whether hematopoietic stem cell transplantation (HSCT), versus standard enzyme replacement therapy (ERT), is effective for type VI mucopolysaccharidosis (MPS VI).</p><p><strong>Purpose: </strong>New related advances in HSCT prompted an examination of the transplant procedures performed in a recent cohort.</p><p><strong>Methods: </strong>This single-center retrospective study reviewed the medical records of 17 pediatric patients with MPS VI who underwent allogeneic HSCT in 2021-2023. All conditioning regimens were myeloablative. Engraftment days, complications, and survival data were recorded. As follow-up was short, we recorded only 6-min walk test distance before versus after HSCT.</p><p><strong>Results: </strong>The patients underwent transplantation at a median of 6 years post-diagnosis. All were engrafted and had a full or mixed chimerism. Enzyme levels were within normal ranges. Walking tests of all evaluable patients improved at a median 9-month follow-up.</p><p><strong>Conclusion: </strong>HSCT aims to improve the disease and provides a permanent solution at the enzyme level, eliminating enzyme replacement therapy. Our study showed that HSCT, a less expensive and permanent treatment option, should be offered to patients with MPS VI.</p>","PeriodicalId":36018,"journal":{"name":"Clinical and Experimental Pediatrics","volume":" ","pages":""},"PeriodicalIF":3.2,"publicationDate":"2025-03-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143626401","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Efficacy and safety of carbon dioxide versus room-air insufflation in pediatric colonoscopy: a randomized controlled trial.","authors":"Ajay Aravind, Ujjal Poddar, Anshu Srivastava, Moinak Sen Sarma","doi":"10.3345/cep.2024.02012","DOIUrl":"https://doi.org/10.3345/cep.2024.02012","url":null,"abstract":"<p><strong>Background: </strong>Adequately powered studies in children are scarce and there are reports on the risk of carbon dioxide (CO2) retention after colonoscopy.</p><p><strong>Purpose: </strong>This study investigated the efficacy and safety of CO2 insufflation in children undergoing colonoscopy.</p><p><strong>Methods: </strong>This prospective randomized clinical trial was conducted at a tertiary care hospital between March 2023 and July 2024. We recruited 200 consecutive children (age, 5-18 years; n=100 in each arm) who underwent colonoscopy under conscious sedation. Patients were randomized to receive CO2 or room air using a random number table. The primary outcome measure was postprocedural pain assessed by using a visual analog scale. Secondary outcome measures included time to reach the cecum, total procedure duration, abdominal distension, and end-tidal CO2 level. Complications were recorded.</p><p><strong>Results: </strong>Pain scores at 2 and 4 h post-procedure were significantly lower in the CO2 versus room-air group (1.12 vs. 1.66, p=0.001 at 2 h and 0.37 vs. 0.61, p=0.002 at 4 h). The time to reach the cecum was significantly higher in the CO2 group (39.6 vs. 26.6 min, p=0.01). A greater proportion of children in the room-air group (29% vs. 19%, p=0.04) reported significant pain (visual analog scale score, ≥3). The subgroup analysis revealed a significantly longer time to reach the cecum and total procedure duration in the CO2 group among first-year trainees. End-tidal CO2 levels were significantly higher in the CO2 group (36 [interquartile range, 35-37] mmHg vs. 34 [interquartile range, 32-35] mmHg, p=0.001), but none developed any signs of CO2 retention. No significant intergroup differences were noted in abdominal girth, bloating sensation, analgesic requirements, or procedure-related complications.</p><p><strong>Conclusion: </strong>s: CO2 insufflation is safer and makes the procedure less painful but slower than room-air insufflation, especially in first-year trainees, without an increased risk of retention.</p>","PeriodicalId":36018,"journal":{"name":"Clinical and Experimental Pediatrics","volume":" ","pages":""},"PeriodicalIF":3.2,"publicationDate":"2025-03-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143626379","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Nonlinear association between neutrophil-to-lymphocyte ratio and asthma in children and adolescents in the USA: a cross-sectional study.","authors":"Chuhan Cheng, Liyan Zhang","doi":"10.3345/cep.2024.01844","DOIUrl":"https://doi.org/10.3345/cep.2024.01844","url":null,"abstract":"<p><strong>Background: </strong>The neutrophil-to-lymphocyte ratio (NLR) is a marker of systemic inflammation associated with various diseases including respiratory conditions. However, the relationship between NLR and asthma in the pediatric population remains underexplored.</p><p><strong>Purpose: </strong>This study aimed to explore the association between NLR and asthma in children and adolescents and assess its potential role as a predictive biomarker for pediatric asthma.</p><p><strong>Methods: </strong>We retrospectively analyzed the medical records of 12,974 children and adolescents from the National Health and Nutrition Examination Survey in 2011-2020. NLR was defined as the ratio of neutrophil to lymphocyte counts. Asthma was diagnosed using a structured questionnaire. Multivariate logistic regression models were used to evaluate the association between NLR and asthma. A restricted cubic spline was used to explore non-linear relationships, and a threshold analysis was conducted to identify potential cutoff values for the NLR.</p><p><strong>Results: </strong>A total of 12,974 children and adolescents were included (male: 6,686 [51.5%]; mean age: 10 years [IQR, 5.0-14.0]). After the adjustment for confounders, participants with the highest versus lowest NLR exhibited a significantly elevated risk of asthma (odds ratio [OR]=1.39; 95% confidence interval [CI], 1.13-1.71). Additionally, a multivariate restricted cubic spline analysis revealed a non-linear relationship between NLR and asthma (p=0.023). A threshold analysis revealed that an NLR<2.23 was significantly associated with an increased risk of asthma (OR=1.23; 95% CI, 1.05-1.45), while an NLR≥2.23 showed no significant association. A subgroup analysis revealed no interactive role of NLR and asthma.</p><p><strong>Conclusion: </strong>Our findings indicate a non-linear saturation-effect relationship between NLR and asthma in children and adolescents.</p>","PeriodicalId":36018,"journal":{"name":"Clinical and Experimental Pediatrics","volume":" ","pages":""},"PeriodicalIF":3.2,"publicationDate":"2025-03-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143626405","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Somatic symptom severity during acute illnesses among children with functional gastrointestinal disorders.","authors":"Rattanachart Sirinil, Anundorn Wongteerasut","doi":"10.3345/cep.2024.01795","DOIUrl":"https://doi.org/10.3345/cep.2024.01795","url":null,"abstract":"<p><strong>Background: </strong>Functional gastrointestinal disorders (FGIDs) are associated with various gastrointestinal (GI) and non-GI symptoms, risk factors for which commonly include psychosocial and physical stresses.</p><p><strong>Purpose: </strong>This study aimed to compare somatic symptom severity between children with FGIDs and healthy controls during acute illnesses.</p><p><strong>Methods: </strong>This was a prospective descriptive cross-sectional study whose inclusion criterion was age 4-18 years. Children were classified into FGID and control groups using the Rome IV diagnostic criteria. Somatic symptom severity was estimated using a visual analog scale (VAS) and the Children's Somatic Symptoms Inventory-24 (CSSI-24) questionnaire and compared between groups.</p><p><strong>Results: </strong>Ninety-three children, including 40 with FGIDs (43%), were enrolled. The FGID group had statistically significantly higher VAS scores for abdominal pain than controls (2.93 ± 3.68 vs. 0.72 ± 2.08, p<0.001). However, no significant intergroup differences were noted in VAS scores for nausea (p=0.493) or headache (p=0.311). For somatization symptoms, the CSSI-24 total (20.58 ± 18.32 vs. 7.06 ± 10.49, p<0.001), GI symptom (9.60 ± 7.48 vs. 2.43 ± 3.39, p≤0.001) and non-GI symptom (10.98 ± 11.67 vs. 4.62 ± 7.88, p<0.001) scores were significantly higher for the FGID versus control groups, respectively.</p><p><strong>Conclusion: </strong>Children with FGIDs exhibited more significant somatic symptoms than controls during acute illnesses. GI and non-GI manifestations were significantly more common in children with FGIDs.</p>","PeriodicalId":36018,"journal":{"name":"Clinical and Experimental Pediatrics","volume":" ","pages":""},"PeriodicalIF":3.2,"publicationDate":"2025-03-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143626407","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Debate around and impact of digital screen time and media parenting on children's development.","authors":"Gowda Prashanth","doi":"10.3345/cep.2025.00073","DOIUrl":"https://doi.org/10.3345/cep.2025.00073","url":null,"abstract":"","PeriodicalId":36018,"journal":{"name":"Clinical and Experimental Pediatrics","volume":" ","pages":""},"PeriodicalIF":3.2,"publicationDate":"2025-03-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143626367","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Aplasia cutis congenita with unique vascular malformation and cranial hypoplasia: a case in a preterm infant.","authors":"Yasufumi Sakata, Natsumi Fujii, Sadahiro Nomura, Yoshihiro Azuma, Hiroki Hamano, Hidenobu Kaneyasu, Seigo Okada, Kazumasa Takahashi, Shunji Hasegawa","doi":"10.3345/cep.2024.01998","DOIUrl":"https://doi.org/10.3345/cep.2024.01998","url":null,"abstract":"","PeriodicalId":36018,"journal":{"name":"Clinical and Experimental Pediatrics","volume":" ","pages":""},"PeriodicalIF":3.2,"publicationDate":"2025-03-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143626364","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Knowledge, attitude, and practice regarding dengue vaccine: a baseline study of community members and health providers in Indonesia.","authors":"Abdul Wahab, Ida Safitri Laksanawati, Retna Siwi Padmawati, Asal Wahyuni Erlin Mulyadi, Wahyu Triadmajani, Jarir At Thobari","doi":"10.3345/cep.2024.00962","DOIUrl":"10.3345/cep.2024.00962","url":null,"abstract":"<p><strong>Background: </strong>Despite recent behavioral interventions aimed at reducing the burden of dengue, persistent unfavorable related behaviors in the Indonesian community suggest that vaccination could be effective. However, before this is implemented nationally, public perception and acceptance of the vaccine should be assessed in addition to health providers' points of view.</p><p><strong>Purpose: </strong>This study aimed to assess the knowledge, attitudes, and practices (KAP) of community members and health providers in Indonesia.</p><p><strong>Methods: </strong>A cross-sectional survey was conducted in 6 districts of 3 Indonesian provinces. Respondents included both community members and health providers living within the territory of the selected primary health care centers. Pretested questionnaires were used to gather information on the sociodemographic characteristics and KAPs regarding dengue vaccines. An independent t test was performed to compare the mean KAP scores between community members and health providers, while a logistic regression analysis was used to identify the predictors in each domain.</p><p><strong>Results: </strong>Only 18.5% of health providers possessed high knowledge of the dengue vaccine versus only 12.5% of community members. Approximately one-third of the health providers versus one-fourth of community members displayed a high to moderate score for attitudes about dengue vaccines. Dengue vaccination practices demonstrated a more favorable score for both health providers and community members at 59.1% and 41.1%, respectively. No individual sociodemographic characteristics were associated with KAPs; rather, only education was correlated with better dengue vaccination practices. We also found that good knowledge of dengue was the only factor associated with all domains.</p><p><strong>Conclusion: </strong>Our findings highlight the urgent need to deliver educational interventions to health providers and community members in Indonesia to improve their awareness of dengue vaccines.</p>","PeriodicalId":36018,"journal":{"name":"Clinical and Experimental Pediatrics","volume":" ","pages":"228-237"},"PeriodicalIF":3.2,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11884950/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142629688","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Zinc as a treatment modality for acute infectious diarrhea in children.","authors":"Ji Sook Park","doi":"10.3345/cep.2024.00381","DOIUrl":"10.3345/cep.2024.00381","url":null,"abstract":"","PeriodicalId":36018,"journal":{"name":"Clinical and Experimental Pediatrics","volume":" ","pages":"223-224"},"PeriodicalIF":3.2,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11884947/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142559029","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Congenital antral web: rare cause of gastric outlet obstruction successfully managed with endoscopic balloon dilatation.","authors":"Upasana Ghosh, Ujjal Poddar, Srinivas Srinidhi Vadlapudi, Moinak Sen Sarma, Anshu Srivastava","doi":"10.3345/cep.2024.01242","DOIUrl":"10.3345/cep.2024.01242","url":null,"abstract":"","PeriodicalId":36018,"journal":{"name":"Clinical and Experimental Pediatrics","volume":" ","pages":"266-268"},"PeriodicalIF":3.2,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11884953/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142984906","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}