Clinical and Experimental Pediatrics最新文献

{"title":"Prognostic role of mid-regional pro-adrenomedullin in predicting infection in pediatric cancer with febrile neutropenia.","authors":"Seham M Ragab, Sara Mahmoud El-Deeb, Ahmed Saeed, Asmaa A Mahmoud","doi":"10.3345/cep.2024.01620","DOIUrl":"10.3345/cep.2024.01620","url":null,"abstract":"<p><strong>Background: </strong>Febrile neutropenia (FN) remains an important complication of cytotoxic chemotherapy for which an urgent and appropriate evaluation is imperative.</p><p><strong>Purpose: </strong>To assess the diagnostic and prognostic roles of mid-regional pro-adrenomedullin (MR-ProADM) levels in predicting infection in patients with FN.</p><p><strong>Methods: </strong>This comparative cross-sectional study included 137 patients with chemotherapy-induced FN. Complete blood count, C-reactive protein (CRP), procalcitonin (PCT), and MR-ProADM were evaluated on the 1st day of FN. Chest computed tomography was performed on the 5th day.</p><p><strong>Results: </strong>MR-ProADM levels were significantly higher in patients with FN than in controls. CRP and MR-ProADM levels were significantly higher and absolute neutrophil count (ANC) was significantly lower in patients with versus without bacterial infections. CRP, PCT, and MR-ProADM levels were significantly negatively correlated with ANC. CRP, PCT, and MR-ProADM levels were significantly and positively correlated with FN degree, FN duration, and hospital stay length. A multivariate regression analysis showed that a longer FN duration and hospital stay length, along with elevated CRP, PCT, and MR-ProADM levels, were significant risk factors for mortality.</p><p><strong>Conclusion: </strong>MR-ProADM is a reliable prognostic and diagnostic tool for predicting infection in patients with FN.</p>","PeriodicalId":36018,"journal":{"name":"Clinical and Experimental Pediatrics","volume":" ","pages":"445-453"},"PeriodicalIF":3.2,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12146680/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142984915","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":"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":"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 PRISMA (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":"417-427"},"PeriodicalIF":3.2,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12146678/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143626403","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":"COVID-19 vaccine hesitancy among parents of children with systemic lupus erythematosus.","authors":"Karnchanit Sausukpaiboon, Nuanpan Penboon, Pornpimol Rianthavorn","doi":"10.3345/cep.2024.01340","DOIUrl":"10.3345/cep.2024.01340","url":null,"abstract":"<p><strong>Background: </strong>Coronavirus disease 2019 (COVID-19) vaccination remains an essential strategy for reducing disease burden. Specific guidelines for vaccinating children with systemic lupus erythematosus (SLE) are currently unavailable, highlighting the gap in tailored recommendations for this population.</p><p><strong>Purpose: </strong>This study aimed to estimate parental intention to vaccinate children with SLE against COVID-19 and identify factors associated with this intention. It also explored parents' attitudes toward the vaccine.</p><p><strong>Methods: </strong>Seventy-four parents of patients aged 5-21 years who were diagnosed with SLE before 18 years of age were surveyed regarding their willingness to further vaccinate their children with SLE against COVID-19. The parents were categorized into vaccine acceptance (VA) and vaccine hesitancy (VH) groups and completed a validated 6-item questionnaire designed to gauge their attitudes toward the vaccine. Vaccine hesitancy scale (VHS) scores were calculated with higher scores indicating increased VH. The adjusted odds ratios (aOR) (95% confidence interval [CI]) for VA-associated factors were determined using multivariate analysis.</p><p><strong>Results: </strong>Twenty-five parents (33.8%) were diagnosed with VH. Compared with the VH group, the VA group showed a higher frequency of previous COVID-19 vaccine uptake, completed immunization in children, and parental willingness to be vaccinated themselves. Children were older in the VA versus VH group. The mean total VHS score was significantly higher in the VH versus VA group. In a multivariate model of factors differing significantly between the VA and VH groups, parental willingness to vaccinate themselves (aOR, 5.0; 95% CI, 1.2-20.4), patient age (aOR, 1.4; 95% CI, 1.1-1.9), and VHS score on vaccine efficacy belief (aOR, 0.1 [0.0-0.5]) were significantly associated with VA.</p><p><strong>Conclusion: </strong>A significant proportion of parents were hesitant to vaccinate their children with SLE against COVID-19. These insights underscore the importance of developing targeted educational interventions to address specific parental concerns and improve vaccine uptake in children with SLE.</p>","PeriodicalId":36018,"journal":{"name":"Clinical and Experimental Pediatrics","volume":" ","pages":"454-462"},"PeriodicalIF":3.2,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12146599/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143123717","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":"Protocolized sedation may reduce ventilation and sedation requirements in the pediatric intensive care unit: a systematic review and meta-analysis.","authors":"Ambrus Szemere, Alíz Fazekas, Anna Réka Sebestyén, Rani Ezzeddine, Veronika Upor, Marie Anne Engh, Péter Hegyi, Zsolt Molnár, Klára Horváth","doi":"10.3345/cep.2024.01711","DOIUrl":"10.3345/cep.2024.01711","url":null,"abstract":"<p><p>This study aimed to evaluate the effectiveness and safety of protocolized sedation in mechanically ventilated pediatric intensive care unit (PICU) patients. A comprehensive search was conducted in MEDLINE, CENTRAL, Embase, Web of Science, and Scopus from inception to October 18, 2023. Randomized controlled trials (RCTs) and observational studies that compared protocol-directed sedation management with conventional sedation regimens in pediatric patients who required invasive mechanical ventilation (IMV) for >24 hours were included. Twenty-six studies (15,214 participants) were included. We found a statistically significant reduction in IMV duration (median difference [MD]=-13.88 hours; 95% confidence interval [CI], -25.46 to -2.29; P=0.022), PICU length of stay (MD=-0.64 days; 95% CI, -1.26 to -0.02; P=0.045). We found significant reductions in the duration (MD=-1.28 days; 95% CI, -2.26 to -0.31; P=0.016) and peak dose (MD=-0.05 mg/kg/hr 95% CI, -0.11 to 0.002; P=0.044) of benzodiazepines. A significant increase was found in the odds of unplanned extubation (odds ratio, 1.13; 95% CI, 1.02 to 1.26; P=0.029). We found no significant results regarding the other outcomes. Our results suggest that protocolized sedation may reduce ventilation requirements and PICU length of stay; however, these findings were not confirmed by RCTs. Moreover, we observed a trend toward a reduction in sedative exposure and an increased odds of unplanned extubation.</p>","PeriodicalId":36018,"journal":{"name":"Clinical and Experimental Pediatrics","volume":" ","pages":"406-416"},"PeriodicalIF":3.2,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12146681/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143459933","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":"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":"10.3345/cep.2024.01998","url":null,"abstract":"","PeriodicalId":36018,"journal":{"name":"Clinical and Experimental Pediatrics","volume":" ","pages":"472-474"},"PeriodicalIF":3.2,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12146677/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143626364","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":"Impact of Xmn1 polymorphism on hydroxyurea therapy in children with HbE-β non-transfusion dependent thalassemia: a cohort study.","authors":"Saheli Roy, Paramita Bhattacharya, Atanu Kumar Dutta, Mrinal Kanti Das","doi":"10.3345/cep.2024.01284","DOIUrl":"10.3345/cep.2024.01284","url":null,"abstract":"<p><strong>Background: </strong>Fetal hemoglobin (HbF) inducers, among which hydroxyurea is the most extensively used, have shifted the paradigm toward the treatment of non-transfusion-dependent thalassemia (NTDT). Xmn1 polymorphism (rs7482144) is characterized by substitution (C>T) at -158 position of the γ-globin gene, which leads to CC, CT, or TT genotype. Recently, the role of the Xmn1 polymorphism as a modifier of hydroxyurea therapy has attracted immense research interest.</p><p><strong>Purpose: </strong>This study aimed to estimate the prevalence of the Xmn1 polymorphism and determine its impact on the efficacy of hydroxyurea therapy in children with NTDT in Eastern India.</p><p><strong>Methods: </strong>This observational ambispective cohort study involved the assessment of 50 patients with NTDT, of whom 28 qualified, who had been receiving hydroxyurea for less than a month. Relevant molecular analyses were performed, and data on the annual transfusion requirement (ATR), height, and HbF level before starting hydroxyurea treatment were derived from medical records. The same parameters were reassessed after 6 months of hydroxyurea therapy. Furthermore, patients were monitored for drug toxicity.</p><p><strong>Results: </strong>All patients included in this study exhibited HbE-β-thalassemia, thus implying it to be one of the commonest NTDT genotypes in Eastern India. The prevalence rates of CC and CT were 43% and 57%, respectively, and none of the patients harbored the TT genotype. Toxicity developed in 22% of patients; however, it was not significantly associated with the Xmn1 polymorphism. Significant decrease in ATR and increase in height were observed following hydroxyurea therapy in both groups. Nevertheless, the change was more marked in CT genotype (median ATR drop: 33%, increase in median height: 3.7%, pCT=0.001) than in CC genotype (median ATR drop: 28%, increase in median height: 2.8%, pCC= 0.003).</p><p><strong>Conclusion: </strong>The T allele of the Xmn1 polymorphism had a favorable effect on the efficacy of hydroxyurea in patients with HbE-β-NTDT.</p>","PeriodicalId":36018,"journal":{"name":"Clinical and Experimental Pediatrics","volume":" ","pages":"437-444"},"PeriodicalIF":3.2,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12146598/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143123800","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":"Effect of postoperative enteral protein supplementation on nitrogen balance in critically ill children.","authors":"Irene Yuniar, Kadek Apik Lestari, Antonius Hocky Pudjiadi, Fatima Safira Alatas, Yoga Devaera","doi":"10.3345/cep.2025.00227","DOIUrl":"https://doi.org/10.3345/cep.2025.00227","url":null,"abstract":"<p><strong>Background: </strong>Critically ill children are at risk of postoperative malnutrition. Thus, optimal nutritional therapy is essential for preventing morbidity development and reducing mortality rates among this population. An adequate protein intake increases anabolism. However, data on the effect of enteral protein supplementation on nitrogen balance and intestinal fatty acid-binding protein (I-FABP) levels in postoperative critically ill children remain limited.</p><p><strong>Purpose: </strong>This study aimed to analyze whether an increased protein intake via enteral nutrition improves nitrogen balance and reduces serum I-FABP levels among postoperative critically ill children.</p><p><strong>Methods: </strong>This double-blind randomized controlled trial examined critically ill children aged 1-5 years who received early postoperative enteral nutrition. A total of 76 subjects were randomized into a standard-protein group (3.0 g/100 mL) or a high-protein group (4.35 g/100 mL). Nitrogen balance was assessed on days 1 and 3, while I-FABP levels were measured before and after 72 h of enteral feeding.</p><p><strong>Results: </strong>The high-protein group showed a significantly greater increase in average nitrogen balance (283.4 [standard deviation, 82.5] mg/kg/day) compared to the standard-protein group (114.7 [standard deviation, 53] mg/kg/day) (p < 0.0001). However, no significant decrease in I-FABP levels was noted in either group despite the above- and below-average nitrogen balance improvements.</p><p><strong>Conclusion: </strong>High-protein enteral supplementation improves nitrogen balance in postoperative critically ill children without causing adverse side effects.</p>","PeriodicalId":36018,"journal":{"name":"Clinical and Experimental Pediatrics","volume":" ","pages":""},"PeriodicalIF":3.2,"publicationDate":"2025-05-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144226988","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":"Adenosine deaminase and interleukin-1 receptor antagonist genetic polymorphisms among obese children with versus without metabolic dysfunction-associated fatty liver disease.","authors":"Hala M Sakhr, Mohammed H Hassan, Azza Mohamed Taha, Ali Helmi Bakri","doi":"10.3345/cep.2025.00731","DOIUrl":"https://doi.org/10.3345/cep.2025.00731","url":null,"abstract":"<p><strong>Background: </strong>Metabolic disorder-associated fatty liver disease (MAFLD) in children is an emerging global health concern, particularly in terms of obesity and metabolic disturbances. Inflammation plays a crucial role in the pathogenesis of MAFLD, with adenosine deaminase (ADA) and interleukin-1 receptor antagonist (IL-1Ra) being potential contributors.</p><p><strong>Purpose: </strong>This study aimed to assess the association between ADA G22A and IL-1Ra single nucleotide polymorphisms (SNPs) and MAFLD among a cohort of Egyptian children. It also aimed to evaluate the validity of VLDL/HDL-C and triglyceride-to-HDL-C ratios for predicting MAFLD in obese children.</p><p><strong>Methods: </strong>One hundred obese children and 50 healthy controls were included. The obese group was further categorized into those with versus without MAFLD. IL-1Ra and ADA G22A SNPs were evaluated using conventional polymerase chain reaction (PCR) and restriction fragment length polymorphism (RFLP)-PCR, respectively. VLDL/HDL and triglyceride-to-HDL ratios were calculated from the lipid profiles of the included participants.</p><p><strong>Results: </strong>The obese children had significantly higher weight, weight Z-score, body mass index (BMI), BMI Z-score, and waist circumference than the healthy controls. These parameters were considerably higher in children with versus without MAFLD p˂0.05 all. The GG genotype and G allele of ADA G22A were significantly more frequent in the obese children versus controls (p˂0.05 for both); however, no significant difference was observed between obese children with versus without MAFLD. Regarding IL-1Ra polymorphisms, the *2/*2 genotype was more common in the controls and obese children without MFLD, whereas the *1/*2 genotype was prevalent in the obese children with MAFLD (p˂0.05 all). A VLDL/HDL-C cutoff ratio of >0.6308 showed 80% sensitivity, 58% specificity, a 65.6% positive predictive value (PPV), a 74.4% negative predictive value, and 69% accuracy at differentiating among MAFLD cases. The triglyceride-to-HDL-C ratio cutoff of >3.0685 demonstrated high specificity (88%) and a high PPV (84.2%) but moderate sensitivity (64%) and overall accuracy (76%).</p><p><strong>Conclusion: </strong>The current study's findings support the possible genetic role of ADA G22A in childhood obesity, with a significant role for the IL-1Ra SNP in the development of MAFLD in obese children. The triglyceride-to-HDL-C ratio was more useful than the VLDL/HDL-C ratio for predicting pediatric MAFLD.</p>","PeriodicalId":36018,"journal":{"name":"Clinical and Experimental Pediatrics","volume":" ","pages":""},"PeriodicalIF":3.2,"publicationDate":"2025-05-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144181393","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":"Navigating the complex behavioral landscape of children in foster care and adopted families.","authors":"Anisha Choi, Sandhya J Kadam","doi":"10.3345/cep.2025.00822","DOIUrl":"https://doi.org/10.3345/cep.2025.00822","url":null,"abstract":"","PeriodicalId":36018,"journal":{"name":"Clinical and Experimental Pediatrics","volume":" ","pages":""},"PeriodicalIF":3.2,"publicationDate":"2025-05-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144081118","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":"Expanding genotype-phenotype correlation of Kenny-Caffey syndrome type 1.","authors":"Manuela Lo Bianco, Federica Sipala, Xena Giada Pappalardo, Gaia Fusto, Roberta Rizzo, Federico Favata, Carla Cimino, Silvia Marino, Martino Ruggieri, Agnese Suppiej, Simone Ronsisvalle, Raffaele Falsaperla","doi":"10.3345/cep.2025.00360","DOIUrl":"https://doi.org/10.3345/cep.2025.00360","url":null,"abstract":"","PeriodicalId":36018,"journal":{"name":"Clinical and Experimental Pediatrics","volume":" ","pages":""},"PeriodicalIF":3.2,"publicationDate":"2025-05-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144081106","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}