Frontiers in Pediatrics最新文献

{"title":"A clinical prediction model for distant metastases of pediatric neuroblastoma: an analysis based on the SEER database.","authors":"Zhiwei Yan, Yumeng Wu, Yuehua Chen, Jian Xu, Xiubing Zhang, Qiyou Yin","doi":"10.3389/fped.2024.1417818","DOIUrl":"10.3389/fped.2024.1417818","url":null,"abstract":"<p><strong>Background: </strong>Patients with distant metastases from neuroblastoma (NB) usually have a poorer prognosis, and early diagnosis is essential to prevent distant metastases. The aim was to develop a machine-learning model for predicting the risk of distant metastasis in patients with neuroblastoma to aid clinical diagnosis and treatment decisions.</p><p><strong>Methods: </strong>We built a predictive model using data from the Surveillance, Epidemiology, and End Results (SEER) database from 2010 to 2018 on 1,542 patients with neuroblastoma. Seven machine-learning methods were employed to forecast the likelihood of neuroblastoma distant metastases. Univariate and multivariate logistic regression analyses were used to identify independent risk factors for building machine learning models. Secondly, the subject operating characteristic area under the curve (AUC), Precision-Recall (PR) curves, decision curve analysis (DCA), and calibration curves were used to assess model performance. To further explain the optimal model, the Shapley summation interpretation method (SHAP) was applied. Ultimately, the best model was used to create an online calculator that estimates the likelihood of neuroblastoma distant metastases.</p><p><strong>Results: </strong>The study included 1,542 patients with neuroblastoma, multifactorial logistic regression analysis showed that age, histology, tumor size, tumor grade, primary site, surgery, chemotherapy, and radiotherapy were independent risk factors for distant metastasis of neuroblastoma (<i>P </i>< 0.05). Logistic regression (LR) was found to be the optimal algorithm among the seven constructed, with the highest AUC values of 0.835 and 0.850 in the training and validation sets, respectively. Finally, we used the logistic regression model to build a network calculator for distant metastasis of neuroblastoma.</p><p><strong>Conclusion: </strong>The study developed and validated a machine learning model based on clinical and pathological information for predicting the risk of distant metastasis in patients with neuroblastoma, which may help physicians make clinical decisions.</p>","PeriodicalId":12637,"journal":{"name":"Frontiers in Pediatrics","volume":null,"pages":null},"PeriodicalIF":2.1,"publicationDate":"2024-09-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11447546/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142371636","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":"Comparative study of robotic-assisted single-incision-plus-one port and single-incision laparoscopic choledochal cyst excision.","authors":"Ling Zhang, Shan Chen, Yang Lin, Jianbin Wang, Xinyi Qiu, Lizhi Li","doi":"10.3389/fped.2024.1403358","DOIUrl":"10.3389/fped.2024.1403358","url":null,"abstract":"<p><strong>Objective: </strong>To compare the efficacy of robotic-assisted single-incision-plus-one-port laparoscopic choledochal cyst excision (R-SILC + 1) and single-incision laparoscopic choledochal cyst (SILC) in treating pediatric choledochal cyst (CDC).</p><p><strong>Methods: </strong>We retrospectively analyzed the clinical data of patients diagnosed with CDC in our hospital from June 2021 to October 2023. Among them, patients underwent either R-SILC + 1 or SILC procedures. Demographic parameters, operative details, and postoperative outcomes were studied.</p><p><strong>Results: </strong>A total of forty-nine patients were included, with 23 children undergoing R-SILC + 1 and 26 children undergoing SILC. There were no statistically significant differences in demographic data, postoperative pain scores, and postoperative complication rates between the two groups (all <i>p</i> > 0.05). Compared with the SILC group, the R-SILC + 1 group demonstrated less intraoperative bleeding volume (10.4 ± 3.6 vs. 15.0 ± 3.6 ml, <i>p</i> < 0.05), a shorter indwelling time of the abdominal drainage tube [5(5,6) vs. 7(5.8,8.3) d, <i>p</i> < 0.05], a shorter postoperative fasting time [4(3,4) vs. 6(5,7) d, <i>p</i> < 0.05], and a shorter postoperative discharge time [6(6,7) vs. 8(6,11) d, <i>p</i> < 0.05]. However, the R-SILC + 1 group had a longer operation time [388(295,415) vs. 341(255.8,375.2) min, <i>p</i> < 0.05] and higher hospitalization cost (7.9 ± 0.4 vs. 3.2 ± 0.3 ten thousand, <i>p</i> < 0.05).</p><p><strong>Conclusion: </strong>Compared with the SILC group, the R-SILC + 1 group demonstrated clear advantages in treating pediatric CDC, but it is associated with a prolonged learning curve and operation time, and high costs. With improvements in physician experience and technological advancements, its potential will be further unleashed.</p>","PeriodicalId":12637,"journal":{"name":"Frontiers in Pediatrics","volume":null,"pages":null},"PeriodicalIF":2.1,"publicationDate":"2024-09-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11447616/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142371638","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":"Lateral column lengthening versus subtalar arthroereisis for pes planovalgus in patients with cerebral palsy: a systematic review and meta-analysis","authors":"Chang-Hao Lin, Chun-Ho Chen, Shu-Hsin Yao","doi":"10.3389/fped.2024.1443447","DOIUrl":"https://doi.org/10.3389/fped.2024.1443447","url":null,"abstract":"IntroductionAlthough pes planus, a common deformity in children with cerebral palsy (CP), is predominantly treated through lateral column lengthening (LCL), subtalar arthroereisis (SA) has also gained popularity for this purpose. This systematic review was conducted to compare surgical outcomes between LCL and SA for pes planovalgus in children with CP.MethodsPubMed, EMBASE, Cochrane Library, and Google Scholar were comprehensively searched for relevant articles reporting the outcomes of LCL and SA in the target population. Surgical outcomes were evaluated in terms of radiographic parameters and postoperative complications.ResultsThis review included 22 studies involving patients undergoing LCL (LCL group) and 9 studies involving those undergoing SA (SA group). LCL outperformed SA in terms of corrections in the talonavicular coverage angle (8.1°–42.1° vs. 8.0°–30.7°), anteroposterior talo–first metatarsal angle (12.3°–33.7° vs. 9.8°–21.4°), and calcaneal pitch angle (2.5°–29.7° vs. 3.5°–8.0°). Furthermore, the risk of postoperative complications, such as recurrence, pain, undercorrection, and overcorrection, was higher in the LCL group than in the SA group. However, the risks of reoperation and implant-related problems were higher in the SA group than in the LCL group. A meta-analysis of two randomized studies revealed that improvement in calcaneal pitch angle was significantly greater in the LCL group than in the SA group (mean difference: 2.09°; <jats:italic>P</jats:italic> = 0.0488).ConclusionLCL outperforms SA in correcting pes planus–related radiographic parameters in patients with CP. However, postoperative complications appear to be more common after LCL than after SA.Systematic Review Registration<jats:uri>https://inplasy.com/inplasy-2024-5-0126</jats:uri>, Identifier 202450126.","PeriodicalId":12637,"journal":{"name":"Frontiers in Pediatrics","volume":null,"pages":null},"PeriodicalIF":2.6,"publicationDate":"2024-09-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142248405","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Physical space of thirty pediatric intensive care units in the United States of America: a national survey.","authors":"Oliver Karam, Aziez Ahmed, Matthew Bizzarro, Clifford Bogue, John S Giuliano","doi":"10.3389/fped.2024.1473805","DOIUrl":"10.3389/fped.2024.1473805","url":null,"abstract":"<p><strong>Introduction: </strong>The design of Pediatric Intensive Care Unit (PICU) rooms significantly impacts patient care and satisfaction. The aims were first, to describe the current physical space across PICUs in the USA, and second, to identify what proportion of PICUs are compliant with current guidelines.</p><p><strong>Methods: </strong>A descriptive cross-sectional survey was conducted, targeting division chiefs and medical directors of PICUs nationwide. The survey collected data on unit type, construction and renovation dates, room sizes, and available amenities. According to the Guidelines for Design and Construction of Hospitals, PICU rooms are recommended to be single rooms, at least 200 sq ft, have a window and a private bathroom. Data were anonymized and reported as median and interquartile ranges or frequencies and percentages.</p><p><strong>Results: </strong>Thirty units responded. Among the respondents, 26 had general PICUs, 9 had cardiac ICUs, and 3 had intermediate care units, with some units containing multiple types of ICUs. The median annual admissions were 1,125, with a median occupancy rate of 78%. Twenty-three percent of units had at least one double room, and 3% had triple or quadruple rooms. The median room size was 265 sq ft (IQR 230; 304), the smallest room size was 220 sq ft (IQR 179; 275), and the largest single room size was 312 sq ft (IQR 273; 330). Thirty-seven percent of units had bathrooms in every room, while 80% had windows in every room. Additionally, 46% of units had dialysis capabilities in every room, and 7% had negative pressure capabilities in every room. The median building year was 2008 (IQR 2001;2014), with 36% of units having undergone at least one renovation. Larger rooms were associated with more recent build dates (<i>p</i> = 0.01). Only 30% of the PICUs met the guidelines for physical space. These compliant units were built at a median of 4 years ago (IQR 1; 8).</p><p><strong>Conclusion: </strong>This study highlights the variability in PICU room design and amenities across healthcare facilities. Many units still fall short of meeting the guidelines for room size, windows, and private bathrooms. Future research should investigate the relationship between room characteristics and patient outcomes to inform better design practices, with a goal of improving patient experiences and clinical outcomes.</p>","PeriodicalId":12637,"journal":{"name":"Frontiers in Pediatrics","volume":null,"pages":null},"PeriodicalIF":2.1,"publicationDate":"2024-09-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11445063/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142365026","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":"Influence of catheter thickness on respiratory physiology during less invasive surfactant administration in extremely preterm infants","authors":"Chamindu C. Gunatilaka, Qiwei Xiao, Alister J. Bates, Axel R. Franz, Christian F. Poets, Christian A. Maiwald","doi":"10.3389/fped.2024.1352784","DOIUrl":"https://doi.org/10.3389/fped.2024.1352784","url":null,"abstract":"IntroductionDelivering surfactant via thin catheters (minimal-invasive surfactant therapy (MIST); less invasive surfactant administration (LISA)) has become a common procedure. However, the effect of tracheal obstruction caused by catheters of different sizes on tracheal resistance in extremely low gestational age newborns (ELGANs) is unknown.MethodsTo investigate the effect of catheters size 3.5, 5 and 6 French on airway resistance in ELGANs of 23–28 weeks gestational age during LISA, we performed calculations based on Hagen-Poiseuille's law and compared these with a clinically and physically more accurate method: computational fluid dynamics (CFD) simulations of respiratory airflow, performed in 3D virtual airway models derived from MRI.ResultsThe presence of the above catheters decreased the cross-sectional area of the infants' tracheal entrance (the cricoid ring) by 13–53%. Hagen-Poiseuille's law predicted an increase in resistance by 1.5–4.5 times and 1.3–2.6 times in ELGANs born at 23 and 28 weeks, respectively. However, CFD simulations demonstrated an even higher increase in resistance of 3.4–85.1 and 1.1–3.5 times, respectively. The higher calculated resistances were due to the extremely narrow remaining lumen at the glottis and cricoid with the catheter inserted, resulting in a stronger glottal jet and turbulent airflow, which was not predicted by Hagen-Poiseuille.ConclusionCatheter thickness can greatly increase tracheal resistance during LISA-procedures in ELGANs. Based on these models, it is recommended to use the thinnest catheter possible during LISA in ELGANs to avoid unnecessary increases in airway resistance in infants already experiencing dyspnea due to respiratory distress syndrome.","PeriodicalId":12637,"journal":{"name":"Frontiers in Pediatrics","volume":null,"pages":null},"PeriodicalIF":2.6,"publicationDate":"2024-09-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142248406","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Novel loss-of-function variants in WDR26 cause Skraban-Deardorff syndrome in two Chinese patients","authors":"Qi Yang, Xunzhao Zhou, Sheng Yi, XiaoLing Li, Qiang Zhang, Shujie Zhang, Li Lin, Shang Yi, Biyan Chen, Zailong Qin, Jingsi Luo","doi":"10.3389/fped.2024.1429586","DOIUrl":"https://doi.org/10.3389/fped.2024.1429586","url":null,"abstract":"IntroductionMutations in the protein WD repeat structural domain 26 (<jats:italic>WDR26</jats:italic>, MIM 617424) have been identified as the cause of autosomal dominant Skraban-Deardorff syndrome, a rare genetic disorder characterized by intellectual disability (ID), developmental delay (DD), hypotonia, epilepsy, infant feeding difficulties, gait abnormalities and distinctive facial features. The objective of this study is to investigate the genetic factors that may contribute to the development of Skraban-Deardorff syndrome in affected individuals.MethodsIn this study, we used whole-exome sequencing (WES) to analyze pathogenic and likely pathogenic variants in two unrelated Chinese patients with DD and ID. We confirmed the origin of the variants by conducting Sanger sequencing and classified them according to ACMG/AMP guidelines.ResultsHere, two novel <jats:italic>de novo</jats:italic> variants (c.1797delC(p.His599fs*11) and c.1414C&amp;gt;T(p.Gln472*)) in the <jats:italic>WDR26</jats:italic> gene have been identified in two Chinese patients with Skraban-Deardorff syndrome. These patients exhibit a range of symptoms, including varying degrees of ID, DD, speech delay, an abnormal wide-foot and/or stiff-legged gait, facial dysmorphism, behavioural abnormalities, with or without seizures.ConclusionsIn this study, We report two unrelated Chinese patients with Skraban-Deardorff syndrome caused by novel <jats:italic>de novo</jats:italic> pathogenic variants of the <jats:italic>WDR26</jats:italic> gene. These patients showed a clinical phenotype similar to that of patients with the <jats:italic>WDR26</jats:italic> variant. Compared to reported cases with <jats:italic>WDR26</jats:italic> pathogenic variants, patient 2 presented a novel complication of severe behavioural problems, including hyperactivity, social anxiety, self-mutilation, impulsivity and violent behaviour. This research broadens the range of genetic and clinical features of Skraban-Deardorff syndrome. In addition, the symptoms may become more pronounced as the patient ages. Furthermore, our report highlights the clinical diversity of Skraban-Deardorff syndrome. The findings may assist healthcare professionals in providing more accurate genetic testing and counselling to affected families and improving the overall management of the condition.","PeriodicalId":12637,"journal":{"name":"Frontiers in Pediatrics","volume":null,"pages":null},"PeriodicalIF":2.6,"publicationDate":"2024-09-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142248571","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Determinants of measles second dose vaccination dropout among children aged 18–24 months in Ejere woreda, central Ethiopia; unmatched case-control study","authors":"Kitessa Nurgi, Seifadin Ahmed, Gemechu Ganfure, Gemechu Gelan Bekele","doi":"10.3389/fped.2024.1432762","DOIUrl":"https://doi.org/10.3389/fped.2024.1432762","url":null,"abstract":"BackgroundMeasles continues to pose a significant public health challenge, especially in low- and middle-income countries. Despite the implementation of national vaccination programs, measles outbreaks persist in some parts of Ethiopia, and the determinants of dropout from the second measles vaccine dose are not well understood. Hence, this study aimed to assess determinants of measles second dose vaccination dropout among children aged 18–24 months in Ejere woreda, central Ethiopia.MethodsA community-based unmatched case-control design was conducted in the Ejere Woreda of the Oromia regional state in Ethiopia between February 14 and April 6, 2023. Data were collected using a pre-tested structured questionnaire. The collected data were coded and entered into Epi-data version 3.1 and then transported to SPSS version 27 for statistical analysis. Descriptive analysis like frequency, mean, and percentage was calculated. Binary and multivariable logistic regression analysis was done. Finally, variables with a <jats:italic>p</jats:italic>-value &amp;lt;0.05 were considered statistically significant.ResultA total of 446 mothers/caregivers, comprising 110 cases and 336 controls, participated in this study, making the response rate 97.8%. Lack of a reminder for the measles vaccine during postnatal care (PNC) (AOR = 5.19; 95% CI: 2.34, 7.83), having ≤2 antenatal care (ANC) contacts (AOR = 4.95; 95% CI: 2.86, 9.24), long waiting times during previous vaccination (AOR = 2.78; 95% CI: 1.19, 4.38), children of mothers/caregivers without formal education (AOR = 6.46; 95% CI: 2.81, 11.71), mothers/caregivers of children who were unaware of the importance of the second dose of measles (AOR = 8.37; 95% CI: 4.22, 15.08), and mothers/caregivers whose children did not receive at least two doses of vitamin A (AOR = 4.05; 95% CI: 2.15, 8.11) were significant determinants of measles second dose vaccination dropout.ConclusionImplementing targeted interventions during antenatal care and when mothers visit health facilities for other vaccines can significantly improve the uptake of the second dose of the measles vaccine. These strategies not only enhance overall vaccination coverage but also mitigate the risk of measles outbreaks in the community.","PeriodicalId":12637,"journal":{"name":"Frontiers in Pediatrics","volume":null,"pages":null},"PeriodicalIF":2.6,"publicationDate":"2024-09-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142248452","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Quantitative and functional changes in platelets and fibrinogen following cardiopulmonary by-pass in children","authors":"Margherita Plebani, David Longchamp, Pauline Lauwers, Stefano Di Bernardo, Maria-Helena Perez","doi":"10.3389/fped.2024.1453182","DOIUrl":"https://doi.org/10.3389/fped.2024.1453182","url":null,"abstract":"IntroductionCardiopulmonary bypass (CPB) causes coagulopathy, increasing the risk of postoperative bleeding and mortality. The underlying causes of post-CPB coagulopathy and the factors associated with its occurrence are not yet fully understood. This study assesses platelet and fibrinogen concentration and function following CPB in children with congenital heart diseases (CHD).MethodsWe analyzed prospective data from 104 patients aged 0–16 years who underwent CPB surgery for CHD. Blood samples were collected before surgery and within 30 min of CPB completion. In addition to usual coagulation tests, functional analyses were performed using point of care systems with thromboelastometry and impedance aggregometry.ResultsPlatelet count, fibrinogen concentration, and platelet and fibrinogen activities significantly decreased after CPB. The duration of CPB was directly associated with a reduction in platelet count and fibrinogen level (<jats:italic>r</jats:italic> = −0.38, <jats:italic>p </jats:italic>&amp;lt; 0.001; <jats:italic>r</jats:italic> = −0.21, <jats:italic>p </jats:italic>= 0.03, respectively), but not with their measured activity. Postoperative percentages of baseline values for platelet count (58.36% [43.34–74.44] vs. 37.44% [29.81–54.17], <jats:italic>p </jats:italic>&amp;lt; 0.001) and fibrinogen concentration (73.68% [66.67–82.35] vs. 65.22% [57.89–70.83], <jats:italic>p </jats:italic>&amp;lt; 0.001) were significantly higher in patients who did not experience hypothermia during surgery. Age was inversely associated with the decrease in platelet count (<jats:italic>r</jats:italic> = 0.63, <jats:italic>p </jats:italic>&amp;lt; 0.001), TRAPTEM AUC (<jats:italic>r</jats:italic> = 0.43, <jats:italic>p </jats:italic>&amp;lt; 0.001), fibrinogen concentration (<jats:italic>r</jats:italic> = 0.44, <jats:italic>p </jats:italic>&amp;lt; 0.001) and FIBTEM MCF (<jats:italic>r</jats:italic> = 0.57, <jats:italic>p </jats:italic>&amp;lt; 0.001).ConclusionPost-CPB coagulopathy is multifactorial and not solely attributed to hemodilution. It also involves functional changes in coagulation cascade components, which can be demonstrated by thromboelastometry and impedance aggregometry. Young children, patients requiring prolonged CPB surgery, or those experiencing hypothermia are particularly affected.","PeriodicalId":12637,"journal":{"name":"Frontiers in Pediatrics","volume":null,"pages":null},"PeriodicalIF":2.6,"publicationDate":"2024-09-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142248403","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Surgical management of pancreatic neoplasms in children: a single-institution experience over 15 years","authors":"In Geol Ho, Kyong Ihn, Sung Min Le, Soyong Shin, Seok Joo Han","doi":"10.3389/fped.2024.1468276","DOIUrl":"https://doi.org/10.3389/fped.2024.1468276","url":null,"abstract":"IntroductionPancreatic neoplasms are rare among children and very few studies have reported on surgical outcomes for pediatric pancreatic neoplasms. Therefore, we aimed to describe patient and tumor characteristics and report on the surgical outcomes of pediatric pancreatic neoplasm.MethodsIn this retrospective single-center study, we reviewed and analyzed the data of patients who underwent surgery for pediatric pancreatic neoplasms at Severance Children's Hospital between January 2007 and December 2022. Clinical data including demographics, surgical procedures, and postoperative and long-term outcomes were evaluated.ResultsA total of 28 patients underwent surgical treatment for pancreatic neoplasms with a median age of 11.7 years (range: 0.4–17.8). The most common histological diagnosis among benign tumors was solid pseudopapillary neoplasm (SPN), which occurred in 20 patients (71.4%). This was followed by a mucinous cyst, nesidioblastosis, pseudocyst, duplication cyst, and benign cyst, each occurring in one patient (3.5%). Regarding malignant tumors, pancreatoblastoma, solid pseudopapillary carcinoma, and malignant pheochromocytoma were noted in one patient each (3.5%). Tumor locations included the head in 4 patients (14.2%), the body in 7 (25%), and the tail in 16 (57.1%), and was diffuse in 1 (3.5%). The most common surgical resection range was distal pancreatectomy, found in 22 patients (78.5%), followed by pylorus-preserving pancreaticoduodenectomy, found in 2 (7.2%); duodenum-preserving pancreatic resection, central pancreatectomy, tumor enucleation, and near-total pancreatectomy were performed in one patient each (3.5%). Overall, 4 patients developed grade B or C postoperative pancreatic fistulas, and 1 experienced postoperative mortality due to uncontrollable bleeding. The mean follow-up period was 6.1 years (range: 1–15.6 years), during which no significant impact on growth after surgery was detected. Among the 20 patients with SPN, tumor rupture occurred in 4 (20%), among whom 2 experienced tumor recurrences.ConclusionsHistological diagnosis of benign tumors was predominant in this case series and various extents of surgical resection were performed. Surgical treatment for pediatric pancreatic neoplasms appears to be safe and effective. However, considering the long-term prognosis of these patients, it is essential to determine the appropriate extent of surgical resection based on the location of the tumor.","PeriodicalId":12637,"journal":{"name":"Frontiers in Pediatrics","volume":null,"pages":null},"PeriodicalIF":2.6,"publicationDate":"2024-09-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142248453","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A case report navigating CVID and sarcoidosis overlaps in pediatric nephritis","authors":"Amanda Salih, Amanda Brown, Amanda Grimes, Sana Hasan, Manuel Silva-Carmona, Leyat Tal, Joud Hajjar","doi":"10.3389/fped.2024.1417724","DOIUrl":"https://doi.org/10.3389/fped.2024.1417724","url":null,"abstract":"Common variable immunodeficiency (CVID) can be complicated by granulomatous disease, often granulomatous lymphocytic interstitial lung disease (GLILD). Granulomatous interstitial nephritis represents an atypical presentation in pediatrics. Our patient is a previously healthy 13-year-old white male with a recent diagnosis of CVID. He presented with a rash and laboratory findings included pancytopenia (white blood cells 2.6 cells × 10<jats:sup>3</jats:sup>/μl, hemoglobin 11.8 g/dl, platelets 60 × 10<jats:sup>3</jats:sup>/μl), hypercalcemia (14.9 mg/dl), elevated Vit D 1,25 OH level (&amp;gt;200 pg/ml), hyperuricemia (8.8 mg/dl), and acute kidney injury (AKI) (serum creatinine 1.1 mg/dl; baseline 0.64 mg/dl). A broad infectious workup was unremarkable. The rash improved with empiric doxycycline. Hypercalcemia and hyperuricemia were managed with fluid resuscitation, calcitonin, and zoledronic acid. Evaluation for malignancy including a positron emission tomography scan, revealed multiple mediastinal hypermetabolic lymph nodes and pulmonary ground glass opacities, later reported as small pulmonary nodules by computed tomography (CT). Splenomegaly was confirmed by ultrasound and CT. Peripheral smear, bone marrow biopsy, and genetic testing were non-revealing. His angiotensin-converting enzyme level was elevated (359 U/L), raising concerns for sarcoidosis. Given Stage 1 AKI, a renal biopsy was pursued and identified non-caseating granulomatous interstitial nephritis. Treatment with 60 mg of prednisone began for presumed sarcoidosis for 4 months, causing steroid-induced hypertension and mood changes. Zoledronic acid minimally reduced serum creatinine. <jats:italic>Pneumocystis jirovecii</jats:italic> pneumonia prophylaxis was initiated due to <jats:italic>T</jats:italic>-cell cytopenia. Chest CT findings showed a suboptimal response to steroids. A bronchoalveolar lavage demonstrated &amp;gt;50% lymphocytes (normal &amp;lt;10%) and the lung biopsy exhibited non-caseating granulomas, indicating GLILD. Rubella was identified by staining. Following a fever, he was found to have elevated liver enzymes and confirmed hepatitis with portal hypertension on CT. A liver biopsy revealed epithelioid non-caseating granuloma and HHV6 was detected by PCR. He was treated with four cycles of rituximab and granulocyte-colony stimulating factor for persistent neutropenia. Subsequent treatment with mycophenolate led to the resolution of the granulomatous lesions and cytopenias. The rare complication of granulomatous interstitial nephritis in CVID illustrates the intricate nature of diagnosis. This case underscores the necessity for a holistic view of the patient’s clinical and immune phenotype, including distinctive radiological presentations, for precise diagnoses and tailored management of CVID.","PeriodicalId":12637,"journal":{"name":"Frontiers in Pediatrics","volume":null,"pages":null},"PeriodicalIF":2.6,"publicationDate":"2024-09-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142248404","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}