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Objective: The purpose of this study was to describe management and outcomes from a contemporary cohort of children with Wilms tumor complicated by inferior vena caval thrombus. Background: The largest series of these patients was published almost 2 decades ago. Since then, neoadjuvant chemotherapy has been commonly used to manage these patients, and outcomes have not been reported. Methods: Retrospective review of 19 North American centers between 2009 and 2019. Patient and disease characteristics, management, and outcomes were investigated and analyzed. Results: Of 124 patients, 81% had favorable histology (FH), and 52% were stage IV. IVC thrombus level was infrahepatic in 53 (43%), intrahepatic in 32 (26%), suprahepatic in 14 (11%), and cardiac in 24 (19%). Neoadjuvant chemotherapy using a 3-drug regimen was administered in 82% and postresection radiation in 90%. Thrombus level regression was 45% overall, with suprahepatic level showing the best response (62%). Cardiopulmonary bypass (CPB) was potentially avoided in 67%. The perioperative complication rate was significantly lower after neoadjuvant chemotherapy [(25%) vs upfront surgery (55%); P=0.005]. CPB was not associated with higher complications [CPB (50%) vs no CPB (27%); P=0.08]. Two-year event-free survival was 93% and overall survival was 96%, higher in FH cases (FH 98% vs unfavorable histology/anaplastic 82%; P=0.73). Neither incomplete resection nor viable thrombus cells affected event-free survival or overall survival. Conclusions: Multimodal therapy resulted in excellent outcomes, even with advanced-stage disease and cardiac extension. Neoadjuvant chemotherapy decreased the need for CPB to facilitate resection. Complete thrombectomy may not always be necessary. [ABSTRACT FROM AUTHOR]

Objective: The aim of this study was to assess current clinical outcomes in children with prenatally diagnosed congenital lung malformations (CLMs) and to identify prenatal characteristics associated with adverse outcomes. Summary Background Data: Despite a wide spectrum of clinical disease, the identification of fetal CLM subgroups at increased risk for hydrops and respiratory compromise at delivery has not been well defined. Methods: A retrospective cohort study was conducted using an operative database of prenatally diagnosed CLMs managed at 11 children's hospitals from 2009 to 2016. Statistical analyses were performed using nonparametric bivariate or multivariable logistic regression. Results: Three hundred forty-four children were analyzed. Fifteen (5.5%) fetuses were managed with maternal steroids in the setting of hydrops, and prenatal surgical intervention was uncommon (1.7%). Seventy-five (21.8%) had respiratory symptoms at birth, and 34 (10.0%) required neonatal lung resection. Congenital pulmonary airway malformation volume ratio (CVR) measurements were recorded in 169 (49.1%) cases and were significantly associated with perinatal outcome, including hydrops, respiratory distress at birth, need for supplemental oxygen, neonatal ventilator use, and neonatal resection (P < 0.001). An initial CVR ≤1.4 was significantly correlated with a reduced risk for hydrops [area under the curve (AUC), 0.93; 95% confidence interval (CI), 0.87–1.00]. A maximum CVR <0.9 (AUC, 0.72; 95% CI, 0.67–0.85) was associated with a low risk for respiratory symptoms at birth. Conclusions: In this large, multi-institutional study, an initial CVR ≤ 1.4 identifies fetuses at very low risk for hydrops, and a maximum CVR < 0.9 is associated with asymptomatic disease at birth. These findings represent an opportunity for standardization and quality improvement for prenatal counseling and delivery planning. [ABSTRACT FROM AUTHOR]

Background: Continuous passive motion (CPM) has been used for decades, but we are not aware of any randomized controlled trials (RCTs) in which CPM has been compared with physical therapy (PT) for rehabilitation following release of elbow contracture.Methods: In this single-blinded, single-center RCT, we randomly assigned patients undergoing arthroscopic release of elbow contracture to a rehabilitation protocol involving either CPM or PT. The primary outcomes were the rate of recovery and the arc of elbow motion (range of motion) at 1 year. The rate of recovery was evaluated by measuring range of motion at 6 weeks and 3 months. The secondary outcomes included other range-of-motion-related outcomes, patient-reported outcome measures (PROMs), flexion strength and endurance, grip strength, and forearm circumference at multiple time points.Results: A total of 24 patients were assigned to receive CPM, and 27 were assigned to receive PT. At 1 year, CPM was superior to PT with regard to the range of motion, with an estimated treatment difference of 9° (95% confidence interval [CI], 3° to 16°; p = 0.007). Similarly, the use of CPM led to a greater range of motion at 6 weeks and 3 months than PT. The percentage of lost motion recovered at 1 year was higher in the CPM group (51%) than in the PT group (36%) (p = 0.01). The probability of restoring a functional range of motion at 1 year was 62% higher in the CPM group than in the PT group (risk ratio for functional range of motion, 1.62; 95% CI, 1.01 to 2.61; p = 0.04). PROM scores were similar in the 2 groups at all time points, except for a difference in the American Shoulder and Elbow Surgeons (ASES) elbow function subscale, in favor of CPM, at 6 weeks. The use of CPM decreased swelling and reduced the loss of flexion strength, flexion endurance, and grip strength on day 3, with no between-group differences thereafter.Conclusions: Among patients undergoing arthroscopic release of elbow contracture, those who received CPM obtained a faster recovery and a greater range of motion at 1 year, with a higher chance of restoration of functional elbow motion than those who underwent routine PT.Level Of Evidence: Therapeutic Level I. See Instructions for Authors for a complete description of levels of evidence. [ABSTRACT FROM AUTHOR]

Objective: To describe utilization and long-term outcomes of pneumonectomy in children and adolescents with cancer. Summary Background Data: Pneumonectomy in adults is associated with significant morbidity and mortality. Little is known about the indications and outcomes of pneumonectomy for pediatric tumors. Methods: The Pediatric Surgical Oncology Research Collaborative (PSORC) identified pediatric patients <21 years of age who underwent pneumonectomy from 1990 to 2017 for primary or metastatic tumors at 12 institutions. Clinical information was collected; outcomes included operative complications, long-term function, recurrence, and survival. Univariate log rank, and multivariable Cox analyses determined factors associated with survival. Results: Thirty-eight patients (mean 12 ± 6 yrs) were identified; median (IQR) follow-up was 19 (5–38) months. Twenty-six patients (68%) underwent pneumonectomy for primary tumors and 12 (32%) for metastases. The most frequent histologies were osteosarcoma (n = 6), inflammatory myofibroblastic tumors (IMT; n = 6), and pleuropulmonary blastoma (n = 5). Median postoperative ventilator days were 0 (0–1), intensive care 2 (1–3), and hospital 8 (5–16). Early postoperative complications occurred in 10 patients including 1 death. Of 25 (66%) patients alive at 1 year, 15 reported return to preoperative pulmonary status. All IMT patients survived while all osteosarcoma patients died during follow-up. On multivariable analysis, metastatic indications were associated with nonsurvival (HR = 3.37, P = 0.045) Conclusion: This is the largest review of children who underwent pneumonectomy for cancer. There is decreased procedure-related morbidity and mortality than reported for adults. Survival is worse with preoperative metastatic disease, especially osteosarcoma. [ABSTRACT FROM AUTHOR]

Objectives: Survival rates in children born with esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) have improved; however, morbidity associated with the disease remains high. This study aimed to assess the prevalence of gastroesophageal reflux disease (GERD), eosinophilic esophagitis (EoE), fungal esophagitis, esophageal strictures, and long-term outcomes in children with EA/TEF., Methods: We conducted a retrospective chart review on patients with EA/TEF who were seen at Children's Wisconsin from January 2003 to January 2023. Patients born with EA/TEF were included if they underwent at least one endoscopy after 1 year of age. GERD was diagnosed based on abnormal findings on endoscopy, pH-metry, and/or history of fundoplication. EoE and fungal esophagitis were diagnosed based on abnormal endoscopy. Esophageal stricture diagnosis was based on findings on endoscopy and/or esophagram, and clinical symptoms necessitating esophageal dilation., Results: Eighty-five patients (64.7% males, mean age 7.5 years) were included, the majority had type C EA/TEF (90.6%). GERD was diagnosed in 61.1% (n = 52), 49.4% (n = 42) by macro and/or microscopic endoscopic findings, 22.3% (n = 19) by abnormal pH-metry, and 21.1% (n = 18) by the need for fundoplication for refractory reflux and/or esophageal stricture. Risk of GERD increased with lower gestational age (p = 0.0030), lower birth weight (p = 0.023), and long-gap EA (p = 0.034). In children diagnosed with GERD, only 13.4% of patients (n = 7/52) were able to be weaned off proton pump inhibitor (PPI) without disease recurrence. However, overall, at the completion of the study, 44.7% (n = 38) of patients were successfully weaned off PPI without evidence of GERD. EoE was diagnosed in 20% of the patients (n = 17). All patients diagnosed with EoE required escalation of therapy from PPI alone to swallowed corticosteroids in 52.9% (n = 9), dupilumab in 23.5% (n = 4), elemental formula in 17.6% (n = 3), and elemental formula and swallowed steroids in 5.8% (n = 1). Fungal esophagitis was diagnosed in 15.3% of patients (n = 13). An esophageal stricture requiring dilation was diagnosed in 77.6% (n = 66) of patients at a mean age of 28.5 months, with over 60% diagnosed by 24 months of age., Conclusions: Children born with EA/TEF continue to be at high risk of developing GERD, EoE, fungal esophagitis, and esophageal stenosis. Diagnostic and therapeutic endoscopy remains a high-yield test to identify and treat these comorbidities., (© 2024 European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

Background: Awareness among the medical students and junior doctors about organ donation and transplantation (ODT) may play an important role in increasing organ donor pool. This study surveys the knowledge, perceptions, and attitudes of ODT among the U.K. junior doctors and attempts to identify their educational needs. To our knowledge, this is first such study in the United Kingdom., Methods: A cross-sectional online survey was conducted among 1,696 junior doctors (809 foundation and 887 core trainees). A 36-point questionnaire explored the junior doctor's knowledge, perceptions, and attitudes toward ODT., Results: There were 523 respondents (30.8%). Majority were foundation trainees (58.9%). Only 29.6% had previous exposure to transplantation, which reflected in their poor knowledge on the basics of ODT. Only 47.0% of the respondents were aware that consent from next of kin or family is sought for all deceased organ donation. Those registered as organ donor (69.8%) had better knowledge, perceptions, and attitudes in comparison to those not registered. Majority (84.1%) felt that they were inadequately exposed to ODT, and 96.8% stated that ODT should be part of undergraduate curriculum., Conclusion: Junior doctors in the United Kingdom have limited knowledge about ODT. Although subjected to investigators bias, the results demonstrate that junior doctors' perceptions and attitudes toward ODT were favorable. Majority felt that their ODT knowledge was inadequate and suggested the need for a change in undergraduate ODT curriculum. Increasing knowledge and awareness among junior doctors may help to improve the continuing organ shortage for transplantation.

Background: Controversy still exists regarding the best endovascular treatment strategy for patients with symptomatic disease of the superficial femoral artery. There are conflicting data regarding the benefits of superficial femoral artery stenting and the role of primary stenting compared with balloon angioplasty with provisional stent implantation., Methods and Results: A total of 206 patients from 24 centers in the United States and Europe with obstructive lesions of the superficial femoral artery and proximal popliteal artery and intermittent claudication were randomized to implantation of nitinol stents or percutaneous transluminal angioplasty. The mean total lesion length was 71 mm for the stent group and 64 mm for the angioplasty group. Acute lesion success (<30%residual stenosis) was superior for the stent group compared with the angioplasty group (95.8% versus 83.9%; P<0.01). Twenty-nine (40.3%) patients in the angioplasty group underwent bailout stenting because of a suboptimal angiographic result or flow-limiting dissection. Bailout stenting was treated as a target lesion revascularization and loss of primary patency in the final analysis. At 12 months, freedom from target lesion revascularization was 87.3% for the stent group compared with 45.1% for the angioplasty group (P<0.0001). Duplex ultrasound-derived primary patency at 12 months was better for the stent group (81.3% versus 36.7%; P<0.0001). Through 12 months, fractures occurred in 3.1% of stents implanted. No stent fractures resulted in loss of patency or target lesion revascularization., Conclusions: In this multicenter trial, primary implantation of a self-expanding nitinol stent for moderate-length lesions in the superficial femoral artery and proximal popliteal artery was associated with better acute angiographic results and improved patency compared with balloon angioplasty alone., Clinical Trial Registration: URL: http://www.clinicaltrials.gov. Unique identifier: NCT00673985.

Background: The inflammatory/antiinflammatory properties of HDL were compared with HDL cholesterol in 2 groups of patients and in age- and sex-matched control subjects., Methods and Results: Group 1 consisted of 26 patients not yet taking a statin who presented with coronary heart disease (CHD) or CHD equivalents by National Cholesterol Education Program Adult Treatment Panel III criteria studied before and 6 weeks after 40 mg/d of simvastatin. Group 2 consisted of 20 patients with documented CHD and HDL cholesterol > or =84 mg/dL. The inflammatory/antiinflammatory properties of HDL were determined by the ability of the subject's HDL to alter LDL-induced monocyte chemotactic activity (MCA) in a human artery wall coculture. Induction of MCA by a control LDL was determined in the absence or presence of the subject's HDL. Values in the absence of HDL were normalized to 1.0. Values >1.0 after the addition of HDL indicated proinflammatory HDL; values <1.0 indicated antiinflammatory HDL. Group 1 values before simvastatin were LDL cholesterol, 118+/-24 mg/dL; HDL cholesterol, 57+/-13 mg/dL; triglycerides, 125+/-64 mg/dL; and high-sensitivity C-reactive protein (hs-CRP), 1.7+/-1.9 mg/L; and MCA values were 1.38+/-0.91, compared with 0.38+/-0.14 for control subjects (P=1.5x10(-5)). After simvastatin, values were LDL cholesterol, 73+/-24 mg/dL; HDL cholesterol, 61+/-14 mg/dL; triglycerides, 99+/-52 mg/dL; and hs-CRP, 1.3+/-1.3 mg/L; and MCA values were 1.08+/-0.71. In group 2, values were LDL cholesterol, 108+/-34 mg/dL; HDL cholesterol, 95+/-14 mg/dL; triglycerides, 89+/-44 mg/dL; and hs-CRP, 0.8+/-0.7 mg/L; and MCA values were 1.28+/-0.29, compared with 0.35+/-0.11 for control subjects (P=1.7x10(-14)). Similar results were obtained with the cell-free assay., Conclusions: The inflammatory/antiinflammatory properties of HDL distinguished patients from control subjects better than HDL cholesterol and were improved with simvastatin.

Cholesterol oxidation products (ChOx) have been reported to cause acute vascular injury in vivo; however, the pharmacokinetics of ChOx after administration and the mechanisms by which they cause chronic vascular injury are not well understood. To further study the pharmacokinetics and atherogenic properties of ChOx, New Zealand White rabbits were injected intravenously (70 mg per injection, 20 injections per animal) with a ChOx mixture having a composition similar to that found in vivo during a 70-day period. Total ChOx concentrations in plasma peaked almost immediately after a single injection, declined rapidly, and returned to preinjection levels in 2 hours. After multiple injections, the ChOx concentrations rose gradually to levels 2- to 3-fold above baseline levels, increasing mostly in the cholesteryl ester fraction of LDL and VLDL. Rabbit serum and the isolated LDL/VLDL fraction containing elevated ChOx concentrations were cytotoxic to V79 fibroblasts and rabbit aortic endothelial cells. At the time of killing, cholesterol levels in the aortas from ChOx-injected rabbits were significantly elevated despite the fact that plasma cholesterol levels remained in the normal range. In addition, aortas from the ChOx-injected rabbits retained more 125I-labeled horseradish peroxidase, measured 20 minutes after intravenous injection. Transmural concentration profiles across the arterial wall also showed increased horseradish peroxidase accumulation in the inner half of the media from the thoracic aorta in ChOx-injected rabbits. In conclusion, ChOx injection resulted in accumulation of circulating ChOx and induced increased vascular permeability and accumulation of lipids and macromolecules. This study reveals that even under normocholesterolemic conditions, ChOx can cause endothelial dysfunction, increased macromolecular permeability, and increased cholesterol accumulation, parameters believed to be involved in the development of early atherosclerotic lesions.