Journal of pediatric surgery最新文献

Introduction: Neonatal postoperative outcomes may be negatively affected by perioperative red blood cell transfusion (RBCT). This study compared 30-day postoperative outcomes between transfused and non-transfused neonates.

Methods: The National Surgical Quality Improvement Program (NSQIP) Pediatric dataset (2021-2022) was used to analyze the association between RBCT and 30-day morbidity and mortality after neonatal surgery. RBCT was defined as transfusion during or within 72 h after surgery. Propensity score matching compared transfused and non-transfused neonates. Secondary analyses examined outcomes among matched neonates with relative anemia and mortality trends across deciles of preoperative hematocrit (Hct).

Results: Overall, 2687 neonates underwent surgery during the study period, and 14 % received PRBCT. In the matched cohort, 30-day mortality was higher in transfused neonates (26.2 % vs. 13.8 %, p < 0.0001). Transfused neonates also had increased rates of wound dehiscence (2.2 % vs. 0.9 %; p < 0.005), mechanical ventilation >48 h (60.3 % vs. 43.7 %; p < 0.0001), cardiac arrest (3.8 % vs. 2.3 %; p = 0.022), and septic shock (3.8 % vs. 1.1 %; p < 0.0001). Matched neonates with similar rates of PRBCT had comparable morbidity and mortality, regardless of preoperative Hct (<35 % vs. >40 %). Mortality diverged significantly above Hct 33 % for transfused neonates, increasing steadily with higher Hct.

Conclusions: Perioperative RBCT is associated with worse postoperative morbidity and mortality, particularly at higher preoperative Hct levels. Relative preoperative anemia alone is not a significant predictor of outcomes, supporting restrictive transfusion practices in perioperative neonatal care.

Type of study: Retrospective Comparative Study.

Level of evidence: III.

Introduction: Thyroid nodules are infrequent findings in children, though malignancy rates are higher in this population. The Bethesda System for Reporting Thyroid Cytopathology (TBSRTC) standardizes the reporting of thyroid fine needle aspiration (FNA) specimens and has become a global reference for assessing the risk of malignancy (ROM) of thyroid nodules. The 2023 update includes pediatric-specific risk predictions and management recommendations. Our study aimed to evaluate the ROM for each Bethesda (BT) category in our pediatric population and compare them with the 2023 TBSRTC update.

Methods: This retrospective cohort study studied pediatric patients who underwent FNA from 2008 to 2023 at our tertiary care center. ROM was assessed by comparing each Bethesda category with histology after thyroid surgery or with long-term follow-up data for non-surgical cases. Comparison among our observed ROM and the 2023 TBSRTC was done by assessing whether the mean ROM from the 2023 TBSRTC fell within the 95 % Confidence Intervals (CIs) of our cohort's ROM for each Bethesda category.

Results: 165 patients with thyroid nodules underwent FNA and Bethesda system classification. 55 patients were excluded due to incomplete follow-up. Thyroid surgery was required in 58 patients. All Bethesda I nodules were benign, while malignancy rates (ROM) were 10.5 % for Bethesda II, 42.8 % for Bethesda IV, 87.5 % for Bethesda V, and 100 % for Bethesda VI. The mean follow-up was 58,2 months (±41,4 SD, range 6-170 months). The comparison of the ROM in our cohort with the 2023 Bethesda pediatric population reveals notable consistency across all Bethesda categories.

Discussion: The ROM among patients with Bethesda II, IV, V, and VI was higher than reported in TBSRTC for adults and similar to those published in the 2023 TBSRTC for children. The development of updated pediatric-specific guidelines could have a significant impact on follow-up strategies and therapeutic algorithms.

Type of study: Prognosis Study.

Level of evidence: II (retrospective).

Background: Pediatric Surgical Critical Care (PSCC) is a unique specialty incorporating fundamental principles of surgical, neonatal, and pediatric critical care. This study aims to characterize the current landscape of PSCC training to identify opportunities for educational standardization and improvement.

Methods: An anonymous electronic survey-based assessment was distributed to the program directors (PDs) of all current ACGME-accredited PSCC fellowships (n = 14). The survey investigated two main program domains: administrative (program size, accreditation, recruitment strategies) and educational (curricula components, learning resources, rotation schedule). Graduate outcomes (estimated board passage rates) were also assessed. Descriptive statistics were performed.

Results: The survey response rate was 100 %. The majority of primary administrative ACGME accreditation responsibilities are managed either by the pediatric surgery section/department (79 %) or the adult surgery department (21 %). Only 29 % of PDs use a pediatric specific structured curriculum that details specific benchmarks for medical and procedural knowledge. Formalized reading lists and standardized resources are utilized by 64 %. All programs offer recurrent educational lectures to fellows by a variety of faculty. There is marked heterogeneity related to time spent in various core and elective rotations. Average duration spent on a PSCC service was 5.1 months (0-10months). However, only 14 % of programs reported these months to consist of primary patient management responsibilities.

Conclusion: This evaluation of PSCC fellowships demonstrated variability in curriculum, content, and resources. These results support future multidisciplinary efforts to more clearly standardize the fellowship experience in order to ensure practice readiness of these uniquely qualified surgeons.

Levels of evidence: Level V.

Background: Patients with diffuse anaplastic Wilms tumor (DAWT) experience relatively poor oncologic outcomes. Previous work has described mechanisms of telomerase upregulation in DAWT, posing a potential therapeutic target.

Methods: We assessed in vitro sensitivity to vincristine, irinotecan, and telomerase-targeting drug 6-thio-2'-deoxyguanosine (6 dG) in DAWT cell lines WiT49 and PDM115 and in spheroids derived from cell lines and four DAWT patient-derived xenografts (PDX). We also tested in vivo response to vincristine/irinotecan (VI), 6 dG, or combination in WTPDX.

Results: Sensitivity to vincristine varied with EC₅₀ between 0.13 and 44.92 nM in spheroids, with EC₅₀ for SN-38 (irinotecan active metabolite) from 3.06 to 70.96 nM. All were resistant to 6 dG monotherapy with EC₅₀ from 3.06 to 50+ μM. In KT-51, 10 μM 6 dG significantly slowed spheroid growth. 6 dG treatment increased DNA damage response markers pChk1 S345, p53 and γH2AX levels in KT-51, KT-53 and KT-60 spheroids. In WiT49 2D culture, treatment of sub-toxic doses of 6 dG did not induce apoptosis or cell cycle arrest and exhibited minimal synergistic capacity with VI; TERT overexpression did not increase 6 dG sensitivity. In vivo treatment of KT-51, KT-53, and KT-60 with VI exhibited variable responses from progressive disease to complete clinical responses, but 6 dG monotherapy resulted in no tumor responses and 6 dG addition to VI conferred no increased tumor suppression.

Conclusions: DAWT models are variably sensitive to VI but are resistant to 6 dG monotherapy or combination with VI. Future research will address limitations of preclinical WT model systems and assess additional targeted therapies for high-risk WT subtypes.

Purpose: Previous research on pediatric motor vehicle collisions (MVC) and fatalities has primarily focused on patient demographics and crash specific information. This study evaluates whether various measures of local infrastructure, including the National Walk Index (NWI), population density, and public school density, or macroeconomic forces, encapsulated in Social Vulnerability Index (SVI) and food area deprivation (PFA) can predict which counties are most at risk for pediatric traffic fatalities.

Methods: Counties with more than 100,000 children in the most recent US census and ≥1 pediatric traffic fatality as identified in the Fatality Analysis Reporting System (FARS) between 2017 and 2021 were included in the study. Poisson regression modeling was used to identify county level infrastructure and macroeconomic forces that predicted increasing MVC related average annual mortality rate per 100,000 children.

Results: There were 158 counties that met inclusion criteria. Univariate Poisson regression demonstrated that NWI, SVI, PFA, population density, and school density each individually correlated with MVC related mortality rate (p < 0.001 for all predictors). When controlling for SVI and population density, multivariable Poisson regression demonstrated that each decile increase in walkability was associated with a 7 % decrease in MVC related mortality rate (IRR 0.93, 95 % CI 0.91-0.96).

Conclusion: Areas with poor walkability predict the likelihood of pediatric traffic fatality. These findings highlight tangible local and state policy changes that could be implemented to decrease the likelihood of traffic-related child fatality rates in specific counties.

Level of evidence: Not applicable.

Background: We sought to determine whether transamniotic stem cell therapy (TRASCET) could be a viable alternative for the fetal administration of genetically modified hematopoietic stem cells (HSCs) carrying a human hemoglobin subunit beta gene (hHBB) in a healthy syngeneic rat model.

Methods: Time-dated pregnant Lewis dams underwent volume-matched intra-amniotic injections in all their fetuses (n = 61) of a suspension of donor HSCs genetically modified with either both a hHBB gene and a firefly luciferase reporter gene (n = 42) or the firefly luciferase reporter gene alone to control for HBB-derived protein interspecies homology (n = 19) on gestational day 17 (E17; term = E21). Donor HSCs consisted of syngeneic cells phenotyped by flow cytometry with successful hHBB transduction confirmed by ELISA prior to administration in vivo. At term, fetal samples from five anatomical sites relevant to hematopoiesis were screened for the presence of human hemoglobin subunit beta by ELISA and by digital droplet PCR (ddPCR).

Results: When controlled by HSCs without hHBB injections, human hemoglobin subunit beta production was documented at term in the fetal bone marrow and spleen (p < 0.001 and p = 0.028 respectively). Positive hHBB expression by ddPCR was detected in the spleen (54 %), bone marrow (46 %), blood (46 %), liver (23 %), and thymus (15 %).

Conclusions: Genetically modified hematopoietic stem cells carrying a human hemoglobin subunit beta gene can reach fetal hematopoietic sites after simple intra-amniotic injection in a healthy syngeneic rat model. Transamniotic hematopoietic stem cell-based gene therapy could become a novel strategy for the perinatal management of select hemoglobinopathies.

Level of evidence: N/A (animal and laboratory study).

Type of study: animal and laboratory study.

Introduction: Timing of repair for infants with congenital diaphragmatic hernia (CDH) requiring extracorporeal life support (ECLS) remains controversial. Approaches include early repair on ECLS, late repair on ECLS, or repair after ECLS decannulation; all have potential risks and benefits. To mitigate risk and maximize benefit, our group developed an individualized hybrid model in 2016 in which approach is based on prenatal risk stratification. Here we report the outcomes of this model.

Methods: This is a single-institution retrospective review (2002-2023) of infants diagnosed with CDH requiring ECLS, grouping patients according to temporal protocols: Delayed Repair (DR; 2002-2009), Early Repair (ER; 2010-2016), and Hybrid Model (HM; 2017-2023). Demographics, disease characteristics, and outcomes were analyzed. Chi-squared/Fisher's exact/Kruskal-Wallis tests were used, with significance of p < 0.05.

Results: 103 infants were included- 35 (34 %) with DR, 32 (31 %) with ER, and 36 (35 %) using HM. Patient demographics and markers of prenatal severity were similar among groups. Median ECLS duration was significantly less in patients treated with HM (10 days; IQR: 5,17) compared to DR (12 days, IQR: 6,17) and ER (17 days, IQR: 11,22) [p = 0.019]. Survival to discharge was highest using the HM (78 %) compared to DR (69 %) and ER (34 %) groups [p < 0.001]. Subgroup analysis of the HM cohort demonstrated prenatal predictors differed significantly based on timing selected for repair.

Conclusion: We demonstrate a physiologic spectrum across infants with CDH requiring ECLS, suggesting utility of a hybrid model. This individualized approach may be beneficial in discerning patients of moderate severity who could be decannulated prior to repair to mitigate bleeding complications.

Level of evidence: III.