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A cache consistency model and locking protocol customized for file systems that are used as scalable infrastructure for web server farms are studied. High performance web sites rely on web server farms with hundreds of computers serving the same content for scalability, reliability and low-latency access to internet content. Simulation results are validated against an implementation of this protocol. Publish consistency and producer-consumer locking are key technologies for scalable web hosting.

An architecture for network-authenticated disks that implements distributed file systems without file servers of encryption is presented. The system provides network clients with direct network access to remote storage.

Background: Data on median arcuate ligament syndrome (MALS) in children are scant. It is postulated that MALS can cause chronic abdominal pain. It is unclear what percentage of children with this condition are symptomatic and what comorbidities are associated with this syndrome., Methods: In this retrospective study, data on consecutive patients in a single center diagnosed coincidentally with MALS during routine echocardiogram were reviewed. Symptom burden, comorbidities, and the effect of anthropometric indices on MALS were investigated. Descriptive statistics and nonparametric tests were used to describe the findings and to compare variables with normal distribution., Results: Between 2013 and 2020, there were 82 children, 55 females (67%), mean age 13.9 ± 3.2 years, with MALS and complete record. Mean velocity across the stenotic area was 2.6 ± 0.4 m/s. Forty-six patients (57%) had abdominal pain. Age, gender, weight, body mass index (BMI), and Doppler velocity had no statistically significant influence on symptom occurrence. Conversely, patients with joint hypermobility and symptoms of orthostatic intolerance were more likely to have abdominal pain from MALS. Of 24 patients with joint hypermobility, 18 patients had abdominal pain (p=0.027). Thirty-eight patients with orthostatic intolerance (OI) with MALS complained of abdominal pain vs 13 patients with OI and no abdominal pain (p=<0.0001)., Conclusion: Nearly half of patients with MALS had abdominal pain. Age, gender, weight, and the degree of stenosis had no statistically significant influence on symptom occurrence. OI, specifically postural orthostatic tachycardia syndrome (POTS), and joint hypermobility on exam predicted a higher propensity for abdominal pain in patients with MALS., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2024, Kakavand et al.)

Introduction:  Gastroschisis feeding practices vary. Standardized neonatal feeding protocols have been demonstrated to improve nutritional outcomes. We report outcomes of infants with gastroschisis that were fed with and without a protocol., Materials and Methods:  A retrospective study of neonates with uncomplicated gastroschisis at 11 children's hospitals from 2013 to 2016 was performed.Outcomes of infants fed via institutional-specific protocols were compared with those fed without a protocol. Subgroup analyses of protocol use with immediate versus delayed closure and with sutured versus sutureless closure were conducted., Results:  Among 315 neonates, protocol-based feeding was utilized in 204 (65%) while no feeding protocol was used in 111 (35%). There were less surgical site infections (SSI) in those fed with a protocol (7 vs. 16%, p  = 0.019). There were no differences in TPN duration, time to initial oral intake, time to goal feeds, ventilator use, peripherally inserted central catheter line deep venous thromboses, or length of stay. Of those fed via protocol, less SSIs occurred in those who underwent sutured closure (9 vs. 19%, p  = 0.026). Further analyses based on closure timing or closure method did not demonstrate any significant differences., Conclusion:  Across this multi-institutional cohort of infants with uncomplicated gastroschisis, there were more SSIs in those fed without an institutional-based feeding protocol but no differences in other outcomes., Competing Interests: None declared., (Thieme. All rights reserved.)

Background: Management of ovarian torsion has evolved toward ovarian preservation regardless of ovarian appearance during surgery. However, patients with torsion and an ovarian neoplasm undergo a disproportionately high rate of oophorectomy. Our objectives were to identify factors associated with ovarian torsion among females with an ovarian mass and to determine if torsion is associated with malignancy., Methods: A retrospective review of females aged 2-21 y who underwent an operation for an ovarian cyst or neoplasm between 2010 and 2016 at 10 children's hospitals was performed. Multivariate logistic regression was used to assess factors associated with torsion. Imaging data were assessed for sensitivity, specificity, and predictive value in identifying ovarian torsion., Results: Of 814 girls with an ovarian neoplasm, 180 (22%) had torsion. In risk-adjusted analyses, patients with a younger age, mass size >5 cm, abdominal pain, and vomiting had an increased likelihood of torsion (P < 0.01 for all). Patients with a mass >5 cm had two times the odds of torsion (odds ratio: 2.1; confidence interval: 1.2, 3.6). Imaging was not reliable at identifying torsion (sensitivity 34%, positive predictive value 49%) or excluding torsion (specificity 72%, negative predictive value 87%). The rates of malignancy were lower in those with an ovarian mass and torsion than those without torsion (10% versus 17%, P = 0.01). Among the 180 girls with torsion and a mass, 48% underwent oophorectomy of which 14% (n = 12) had a malignancy., Conclusions: In females with an ovarian neoplasm, torsion is not associated with an increased risk of malignancy and ovarian preservation should be considered., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Objective: To provide generalizable estimates for expected outcomes of simple gastroschisis (SG) and complex gastroschisis (CG) patients from a large multi-institutional cohort for use during counseling., Study Design: A retrospective study of 394 neonates with gastroschisis at 11 children's hospitals from January 2013 to March 2017 was performed. Analysis by Fisher's exact tests and Wilcoxon rank sum tests were performed. Outcomes of complex and simple gastroschisis are reported., Result: There were 315 (80%) SG and 79 (20%) CG. CG had increased time from birth to closure (6 vs 4.4 days), closure to goal feeds (69 vs 23 days), ventilator use (90% vs 73%), SSIs (31% vs 11%), NEC (14% vs 6%), PN use (71 vs 24 days), LOS (104.5 vs 33 days), and mortality (11% vs 0%)., Conclusion: This study provides generalizable estimates for expected outcomes of patients with both SG and CG that can be utilized during counseling. CG has significantly worse in-hospital outcomes., (© 2021. The Author(s), under exclusive licence to Springer Nature America, Inc.)

Background: Pediatric lung lesions are a group of mostly benign pulmonary anomalies with a broad spectrum of clinical disease and histopathology. Our objective was to evaluate the characteristics of children undergoing resection of a primary lung lesion and to identify preoperative risk factors for malignancy., Methods: A retrospective cohort study was conducted by using an operative database of 521 primary lung lesions managed at 11 children's hospitals in the United States. Multivariable logistic regression was used to examine the relationship between preoperative characteristics and risk of malignancy, including pleuropulmonary blastoma (PPB)., Results: None of the 344 prenatally diagnosed lesions had malignant pathology ( P < .0001). Among 177 children without a history of prenatal detection, 15 (8.7%) were classified as having a malignant tumor (type 1 PPB, n = 11; other PPB, n = 3; adenocarcinoma, n = 1) at a median age of 20.7 months (interquartile range, 7.9-58.1). Malignancy was associated with the DICER1 mutation in 8 (57%) PPB cases. No malignant lesion had a systemic feeding vessel ( P = .0427). The sensitivity of preoperative chest computed tomography (CT) for detecting malignant pathology was 33.3% (95% confidence interval [CI]: 15.2-58.3). Multivariable logistic regression revealed that increased suspicion of malignancy by CT and bilateral disease were significant predictors of malignant pathology (odds ratios of 42.15 [95% CI, 7.43-340.3; P < .0001] and 42.03 [95% CI, 3.51-995.6; P = .0041], respectively)., Conclusions: In pediatric lung masses initially diagnosed after birth, the risk of PPB approached 10%. These results strongly caution against routine nonoperative management in this patient population. DICER1 testing may be helpful given the poor sensitivity of CT for identifying malignant pathology., Competing Interests: POTENTIAL CONFLICT OF INTEREST: The authors have indicated they have no potential conflicts of interest to disclose., (Copyright © 2021 by the American Academy of Pediatrics.)

Background/purpose: This study evaluated compliance with a multi-institutional quality improvement management protocol for Type-C esophageal atresia with distal tracheoesophageal fistula (EA/TEF)., Methods: Compliance and outcomes before and after implementation of a perioperative protocol bundle for infants undergoing Type-C EA/TEF repair were compared across 11 children's hospitals from 1/2016-1/2019. Bundle components included elimination of prosthetic material between tracheal and esophageal suture lines during repair, not leaving a transanastomotic tube at the conclusion of repair (NO-TUBE), obtaining an esophagram by postoperative-day-5, and discontinuing prophylactic antibiotics 24 h postoperatively., Results: One-hundred seventy patients were included, 40% pre-protocol and 60% post-protocol. Bundle compliance increased 2.5-fold pre- to post-protocol from 17.6% to 44.1% (p < 0.001). After stratifying by institutional compliance with all bundle components, 43.5% of patients were treated at low-compliance centers (<20%), 43% at medium-compliance centers (20-80%), and 13.5% at high-compliance centers (>80%). Rates of esophageal leak, anastomotic stricture, and time to full feeds did not differ between pre- and post-protocol cohorts, though there was an inverse correlation between NO-TUBE compliance and stricture rate over time (ρ = -0.75, p = 0.029)., Conclusions: Compliance with our multi-institutional management protocol increased 2.5-fold over the study period without compromising safety or time to feeds and does not support the use of transanastomotic tubes., Level of Evidence: Level II., Type of Study: Treatment Study., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Purpose: To report outcomes of sutured and sutureless closure for gastroschisis across a large multi-institutional cohort., Methods: A retrospective study of infants with uncomplicated gastroschisis at 11 children's from 2014 to 2016 was performed. Outcomes of sutured and sutureless abdominal wall closure were compared., Results: Among 315 neonates with uncomplicated gastroschisis, sutured closure was performed in 248 (79%); 212 undergoing sutured closure after silo and 36 undergoing primary sutured closure. Sutureless closure was performed in 67 (21%); 37 primary sutureless closure, 30 sutureless closure after silo placement. There was no significant difference in gestational age, gender, birth weight, total days on TPN, and time from closure to initial oral intake or goal feeds. Sutureless closure patients had less general anesthetics, ventilator use/time, time from birth to final closure, antibiotic use after closure, and surgical site/deep space infections. Subgroup analysis demonstrated primary sutureless closure had less ventilator use and anesthetics than primary sutured closure. Sutureless closure after silo led to less ventilator use/time, anesthetics, and antibiotics compared to those with sutured closure after silo., Conclusion: Sutureless abdominal wall closure of neonates with gastroschisis was associated with less general anesthetics, antibiotic use, surgical site/deep space infections, and decreased ventilator time. These findings support further prospective study by our group., Level of Evidence: Level III., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Introduction: The purpose of this study was to develop a multi-institutional registry to characterize the demographics, management, and outcomes of a contemporary cohort of children undergoing congenital lung malformation (CLM) resection., Methods: After central reliance IRB approval, a web-based, secure database was created to capture retrospective cohort data on pathologically-confirmed CLMs performed between 2009 and 2015 within a multi-institutional research collaborative., Results: Eleven children's hospitals contributed 506 patients. Among 344 prenatally diagnosed lesions, the congenital pulmonary airway malformation volume ratio was measured in 49.1%, and fetal MRI was performed in 34.3%. One hundred thirty-four (26.7%) children had respiratory symptoms at birth. Fifty-eight (11.6%) underwent neonatal resection, 322 (64.1%) had surgery at 1-12 months, and 122 (24.3%) had operations after 12 months. The median age at resection was 6.7 months (interquartile range, 3.6-11.4). Among 230 elective lobectomies performed in asymptomatic patients, thoracoscopy was successfully utilized in 102 (44.3%), but there was substantial variation across centers. The most common lesions were congenital pulmonary airway malformation (n = 234, 47.3%) and intralobar bronchopulmonary sequestration (n = 106, 21.4%)., Conclusion: This multicenter cohort study on operative CLMs highlights marked disease heterogeneity and substantial practice variation in preoperative evaluation and operative management. Future registry studies are planned to help establish evidence-based guidelines to optimize the care of these patients., Level of Evidence: Level II., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Purpose: Chest tube (CT) management for pediatric primary spontaneous pneumothorax (PSP) is associated with long hospital stays and high recurrence rates. To streamline management, we explored simple aspiration as a test to predict need for surgery., Methods: A multi-institution, prospective pilot study of patients with first presentation for PSP at 9 children's hospitals was performed. Aspiration was performed through a pigtail catheter, followed by 6 h observation with CT clamped. If pneumothorax recurred during observation, the aspiration test failed and subsequent management was per surgeon discretion., Results: Thirty-three patients were managed with simple aspiration. Aspiration was successful in 16 of 33 (48%), while 17 (52%) failed the aspiration test and required hospitalization. Twelve who failed aspiration underwent CT management, of which 10 (83%) failed CT management owing to either persistent air leak requiring VATS or subsequent PSP recurrence. Recurrence rate was significantly greater in the group that failed aspiration compared to the group that passed aspiration [10/12 (83%) vs 7/16 (44%), respectively, P=0.028]., Conclusion: Simple aspiration test upon presentation with PSP predicts chest tube failure with 83% positive predictive value. We recommend changing the PSP management algorithm to include an initial simple aspiration test, and if that fails, proceed directly to VATS., Type of Study: Prospective pilot study LEVEL OF EVIDENCE: Level III., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Purpose: The purpose of this study was to determine the diagnostic accuracy of tumor markers for malignancy in girls with ovarian neoplasms., Methods: A retrospective review of girls 2-21 years who presented for surgical management of an ovarian neoplasm across 10 children's hospitals between 2010 and 2016 was performed. Patients who had at least one concerning feature on imaging and had tumor marker testing were included in the study. Sensitivity, specificity, and negative and positive predictive values (PPV) of tumor markers were calculated., Results: Our cohort included 401 patients; 22.4% had a malignancy. Testing for tumor markers was inconsistent. AFP had high specificity (98%) and low sensitivity (42%) with a PPV of 86%. The sensitivity, specificity, and PPV of beta-hCG was 44%, 76%, and 32%, respectively. LDH had high sensitivity (95%) and Inhibin A and Inhibin B had high specificity (97% and 92%, respectively)., Conclusions: Tumor marker testing is helpful in preoperative risk stratification of ovarian neoplasms for malignancy. Given the variety of potential tumor types, no single marker provides enough reliability, and therefore a panel of tumor marker testing is recommended if there is concern for malignancy. Prospective studies may help further elucidate the predictive value of tumor markers in a pediatric ovarian neoplasm population., Type of Study: Retrospective Cohort Review., Level of Evidence: Level III., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Purpose:  The purpose of this study is to describe a single institution's 11-year experience treating children with congenital pulmonary airway malformations (CPAMs) and pleuropulmonary blastoma (PPB)., Materials and Methods:  An institutional database was sampled for all patients aged 0 to 18 years from January 1, 2005, to December 31, 2015. Patients with a pathologic diagnosis of CPAM or PPB during this period were reviewed., Results:  A total of 51 patients with a pathologic diagnosis of CPAM ( n  = 45; 88.2%) or PPB ( n  = 6; 11.8%) underwent surgical resection. Among patients treated for PPB, one death occurred approximately 13 months after diagnosis. Although four patients with PPB (four out of six; 66.7%) had radiographic indicators highly suggestive of malignancy prior to surgery, two had a preoperative diagnosis of CPAM (two out of six; 33.3%). Twenty-four patients (24 out of 45; 53.3%) with CPAM underwent resection after developing symptoms and 21 (21 out of 45; 46.7%) were symptomatic at the time of surgery. Mann-Whitney's tests revealed a statistically significant difference in postoperative length of stay (median: 6 vs. 3 days; p  < 0.001) and days with thoracostomy tube in place (median 3 vs. 2 days; p  = 0.003) for symptomatic versus asymptomatic patients, respectively., Conclusion:  CPAM patients appear to recover faster from surgery, if performed before the onset of symptoms. There may be a benefit to waiting until at least 3 months of age to complete resection in the asymptomatic patient. A low threshold for resection should be maintained in patients where delineating CPAM from PPB is difficult., Competing Interests: None., (Georg Thieme Verlag KG Stuttgart · New York.)

Background: Available evidence supports ovary-sparing surgery for benign ovarian neoplasms; however, preoperative risk stratification of pediatric ovarian masses can be difficult. Our objective of this study was to characterize the surgical management of pediatric ovarian neoplasms across 10 children's hospitals and to identify factors that could potentially aid in the preoperative risk stratification of these lesions., Methods: A retrospective review of girls and women aged 2 to 21 years who underwent surgery for an ovarian neoplasm between 2010 and 2016 at 10 children's hospitals was performed. Multivariable logistic regression was used to examine the relationships between the preoperative cohort characteristics, procedure performed, and risk of malignancy., Results: Among 819 girls and women undergoing surgery for an ovarian neoplasm, malignant lesions were identified in 11%. The overall oophorectomy rate for benign disease was 33% (range: 15%-49%) across institutions. Oophorectomy for benign lesions was independently associated with provider specialty ( P = .002: adult gynecologist, 45%; pediatric surgeon, 32%; pediatric gynecologist, 18%), premenarchal status ( P = .02), preoperative suspicion for malignancy ( P < .0001), larger lesion size ( P < .0001), and presence of solid components ( P < .0001). Preoperative findings independently associated with malignancy included increasing size ( P < .0001), solid components ( P = .003), and age ( P < .0001)., Conclusions: The rate of oophorectomy for benign ovarian disease remains high within the pediatric population. Identification of factors associated with the choice of procedure and the risk of malignancy may allow for improved preoperative risk stratification and fewer unnecessary oophorectomies. These results have been used to develop and validate a multidisciplinary preoperative risk stratification algorithm that is currently being studied prospectively across 10 institutions., Competing Interests: POTENTIAL CONFLICT OF INTEREST: The authors have indicated they have no potential conflicts of interest to disclose., (Copyright © 2019 by the American Academy of Pediatrics.)

Purpose: The purpose of this study was to evaluate the clinical presentation and operative outcomes of patients with congenital lobar emphysema (CLE) within a large multicenter research consortium., Methods: After central reliance IRB-approval, a retrospective cohort study was performed on all operatively managed lung malformations at eleven participating children's hospitals (2009-2015)., Results: Fifty-three (10.5%) children with pathology-confirmed CLE were identified among 506 lung malformations. A lung mass was detected prenatally in 13 (24.5%) compared to 331 (73.1%) in non-CLE cases (p < 0.0001). Thirty-two (60.4%) CLE patients presented with respiratory symptoms at birth compared to 102 (22.7%) in non-CLE (p < 0.0001). The most common locations for CLE were the left upper (n = 24, 45.3%), right middle (n = 16, 30.2%), and right upper (n = 10, 18.9%) lobes. Eighteen (34.0%) had resection as neonates, 30 (56.6%) had surgery at 1-12 months of age, and five (9.4%) had resections after 12 months. Six (11.3%) underwent thoracoscopic excision. Median hospital length of stay was 5.0 days (interquartile range, 4.0-13.0)., Conclusions: Among lung malformations, CLE is associated with several unique features, including a low prenatal detection rate, a predilection for the upper/middle lobes, and infrequent utilization of thoracoscopy. Although respiratory distress at birth is common, CLE often presents clinically in a delayed and more insidious fashion., Level of Evidence: Level III., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Recent events in the United States have shown the vulnerability of water quality in certain communities. Accordingly, we conducted a survey in a large community in north central West Virginia (US) to explore the factors that influence the perceptions of water quality. We sought to assess whether respondent's proximity to a mine, gas/oil well, or bodies of water would affect their perceived health risks and environmental concern. Additionally, we aimed at understanding how these perceptions were affected by the density of these sites and the presence of these sites within defined distances. As West Virginia is rapidly expanding its natural gas production, there is no research that has objectively associated water quality perceptions with geographic location in regard to oil and gas extraction sites. With small effect sizes, our results add some evidence to the link between unconventional oil and gas extraction, geographic location, and water quality perceptions. This study suggests the need for further water quality monitoring and increased public communication about water management practices in West Virginia., (Copyright © 2018 Elsevier Ltd. All rights reserved.)

Background: When evaluating a pediatric patient with abdominal pain, identification of a small bowel-to-small bowel intussusception (SBI) on radiologic imaging can create a diagnostic dilemma. The clinical significance and need for surgical exploration of SBI is highly variable, as most of them are considered clinically insignificant. We hypothesize that combination of clinical and radiologic factors in an exclusively SBI population will yield factors that guide the clinician in making operative decisions., Methods: A comprehensive database from a pediatric tertiary hospital was reviewed from January 1, 2011, to December 31, 2016, for any radiographic study mentioning intussusception. Results were reviewed for patients having only SBI (i.e., not ileocolic intussusception), and this comprised the study cohort. The electronic medical records for these patients were reviewed for clinical presentation variables, need for operative intervention, and identification of the intussusception during surgery. Patients with SBI due to enteral feeding tubes were excluded from the study., Results: Within the study period, 139 patients were identified with an SBI on radiologic imaging. Univariate analysis yielded numerous clinical and radiologic factors highly predictive of the need for surgical intervention. However, upon multivariate analysis, only a history of prior abdominal surgery (odds ratio [OR]: 7.2; CI: 1.1-46.3), the presence of focal abdominal pain (OR: 22.1; CI: 4.2-116.3), and the intussusception length (cm; OR: 10.6; CI: 10.3-10.8) were correlated with the need for surgical intervention., Conclusions: SBI is a disease process with a highly variable clinical significance. The presence of focal abdominal pain, a history of prior abdominal surgery, and the intussusception length are the greatest predictors of the need for operative intervention., Level of Evidence: Level II., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Research on place attachment suggests that place identity and place dependence differ between recreationists with varying levels of specialization, recreating in different settings and with different resource proximities to their home. To further explore this relationship, we compared place attachment and recreation specialization of whitewater boaters in four different river settings. Data were collected on three rivers in the US and one in Austria. Place attachment was measured using four place identity and four place dependence items. Recreation specialization was treated as a multivariate construct consisting of the three dimensions; behavior, skill, and enduring involvement. The results of a cluster analysis revealed three specialization clusters. Two ANOVAs were performed by using place dependence and place identity as dependent variables and specialization clusters and the sampling rivers as independent variables. Place identity was not expressed differently between rivers but differed in specialization clusters. Place dependence was different between rivers but not between specialization clusters. Findings suggest that place attachment dimensions vary in river setting and specialization levels. Management should take into account that boaters exhibit different place attachment based on the specialization level and resource proximity to their home.

West Virginia has had a history of water quality issues. In parallel, the world is facing a plastic pollution crisis. In order to better understand behavioral responses to perceived water quality, a survey was conducted at a major research university to ask participants about water quality perceptions and drinking water behaviors. A total of 4,188 students completed the survey during the Spring 2017 semester. Logistic regression analyses were used to predict behaviors. Results indicated that a third of the student population primarily used bottled water for drinking purposes at home, while 39% used a filter at home and 26% drank water directly from the tap. On campus, bottled water use was reported by 36% of the students, water fountain use represented 31%, and 29% of the students brought their own water with reusable cups/bottles. Health risk perceptions, organoleptic perceptions (i.e., taste, odor, color), and environmental concern were predictors of the different behaviors. Students originally from West Virginia had a higher propensity of using bottled water. We argue that bottled water consumption should be reduced in areas where water quality is not an issue. In this sense, there is a need for education among the student population in West Virginia.

Purpose: Perioperative management of infants with esophageal atresia and tracheoesophageal fistula (EA/TEF) is frequently based on surgeon experience and dogma rather than evidence-based guidelines. This study examines whether commonly perceived important aspects of practice affect outcome in a contemporary multi-institutional cohort of patients undergoing primary repair for the most common type of esophageal atresia anomaly, proximal EA with distal TEF., Methods: The Midwest Pediatric Surgery Consortium conducted a multicenter, retrospective study examining selected outcomes on infants diagnosed with proximal EA with distal TEF who underwent primary repair over a 5-year period (2009-2014), with a minimum 1-year follow up, across 11 centers., Results: 292 patients with proximal EA and distal TEF who underwent primary repair were reviewed. The overall mortality was 6% and was significantly associated with the presence of congenital heart disease (OR 4.82, p=0.005). Postoperative complications occurred in 181 (62%) infants, including: anastomotic stricture requiring intervention (n=127; 43%); anastomotic leak (n=54; 18%); recurrent fistula (n=15; 5%); vocal cord paralysis/paresis (n=14; 5%); and esophageal dehiscence (n=5; 2%). Placement of a transanastomotic tube was associated with an increase in esophageal stricture formation (OR 2.2, p=0.01). Acid suppression was not associated with altered rates of stricture, leak or pneumonia (all p>0.1). Placement of interposing prosthetic material between the esophageal and tracheal suture lines was associated with an increased leak rate (OR 4.7, p<0.001), but no difference in the incidence of recurrent fistula (p=0.3). Empiric postoperative antibiotics for >24h were used in 193 patients (66%) with no difference in rates of infection, shock or death when compared to antibiotic use ≤24h (all p>0.3). Hospital volume was not associated with postoperative complication rates (p>0.08). Routine postoperative esophagram obtained on day 5 resulted in no delayed/missed anastomotic leaks or a difference in anastomotic leak rate as compared to esophagrams obtained on day 7., Conclusion: Morbidity after primary repair of proximal EA and distal TEF patients is substantial, and many common practices do not appear to reduce complications. Specifically, this large retrospective series does not support the use of prophylactic antibiotics beyond 24h and empiric acid suppression may not prevent complications. Use of a transanastomotic tube was associated with higher rates of stricture, and interposition of prosthetic material was associated with higher leak rates. Routine postoperative esophagram can be safely obtained on day 5 resulting in earlier initiation of oral feeds., Study Type: Treatment study., Level of Evidence: III., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Researches on water quality perceptions have used various techniques and models to explain relationships between specific variables. Surprisingly, Structural Equation Modeling (SEM) has received little attention in water quality perceptions studies, and reporting has been inconsistent among existing studies. One objective of this article is to provide readers with a methodological example for conducting and reporting SEM. Another objective is to build a model that explains the different relationships among the diverse factors highlighted by previous studies on water quality perceptions. Our study focuses on the factors influencing people's perceptions of water quality in the Appalachian region. As such, researchers have conducted a survey in a mid-sized city in northcentral West Virginia to assess residents' perceptions of water quality for drinking and recreational purposes. Specifically, we aimed to understand the relationships between perceived water quality, health risk perceptions, organoleptic perceptions, environmental concern, area satisfaction and perceptions of surface water quality. Our model provided a good fit that explained about 50% of the variance in health risk perceptions and 43% of the variance in organoleptic perceptions. Environmental concern, area satisfaction and perceived surface water quality are important factors in explaining these variances. Perceived water quality was dismissed in our analysis due to multicollinearity. Our study demonstrates that risk communication needs to be better addressed by local decision-makers and water managers., (Copyright © 2017 Elsevier Ltd. All rights reserved.)

A questionnaire survey was conducted in order to assess residents' perceptions of water quality for drinking and recreational purposes in a mid-sized city in northcentral West Virginia. Two logistic regression analyses were conducted in order to investigate the factors that influence bottle use and filter use. Results show that 37% of respondents primarily use bottled water and that 58% use a household filter when drinking from the tap. Respondents with lower levels of environmental concern, education levels, and lower organoleptic perceptions were most likely to perceive health risks from tap water consumption, and were most likely to use bottled water. Income, age, and organoleptic perceptions were predictors of water filter use among respondents. Clean water for recreational purposes was not found to be significant with either of these models. Our results demonstrate that bottle use and filter use are explained differently. We argue that more education and better communication about local tap water quality would decrease the use of bottled water. We demonstrate that household filters could be used as an alternative to bottled water.

Whitewater boaters often choose a river based on their preferences for attributes important for their trip experience. This study explored whether preferences and tradeoffs of whitewater boaters for social, resource, and managerial attributes of riverscapes differ among a high and a low use river in the United States by employing a stated choice approach. River trip scenarios were displayed using verbal descriptions and computer-generated photographs. Results indicate that use levels were more important for boaters on the low use river, whereas river difficulty and river access fee was of higher importance for the high use river boaters, who are more involved in this whitewater activity. Preferences for waiting times and trip length did not differ between the samples. Findings suggest that whitewater boaters of high and low use rivers have a different tradeoff behavior among river setting attributes, which has implications for river recreation management., Competing Interests: The authors declare that they have no conflict of interest.

To reduce CO2 emission into the atmosphere, particularly from coal-fired power stations, post combustion capture (PCC) using amine-based solvents to chemically absorb CO2 has been extensively developed. From an infrastructure viewpoint, the faster the absorption of CO2, the smaller the absorber required. The use of catalysts for this process has been broadly studied. In this manuscript, a study of the catalytic efficiencies of inorganic oxoanions such as arsenite, arsenate, phosphite, phosphate, and borate is described. The kinetics of the accelerated CO2 absorption at 25 °C was investigated using stopped-flow spectrophotometry. The catalytic rate constants of these anions for the reaction of CO2 with H2O were determined to be 137.7(3), 30.3(7), 69(2), 32.7(9), and 13.66(7) M(-1)s(-1), respectively. A new mechanism for the catalytic reaction of oxoanions with CO2 has also been proposed. The applicability of these catalysts to PCC was further studied by simulation of the absorption process under PCC conditions using their experimental catalytic rate constants. Arsenite and phosphite were confirmed to be the best catalysts for CO2 capture. However, considering the toxicological effect of arsenic and the oxidative instability of phosphite, phosphate would be the most promising inorganic catalyst for PCC process from the series of inorganic oxoanions studied.

Replaced right hepatic artery is a well-described normal anatomical variant, not previously associated with acute or chronic abdominal symptoms or long-term sequelae. We report a 15-year-old girl presenting with nearly a decade of symptoms secondary to external compression of the proximal duodenum by the ventral passage of a replaced right hepatic artery. Surgical bypass of the duodenal obstruction significantly improved her symptoms. Replaced right hepatic artery related duodenal compression should be considered in the differential diagnosis of proximal duodenal obstruction. The presence of the replaced right hepatic artery should be clearly communicated to allow optimal presurgical planning.

Purpose: Traumatic pancreatic transection is uncommon. The role of laparoscopy in the setting of this injury has not been well described., Patients and Methods: Six large-volume pediatric trauma centers contributed patients <18 years of age who underwent a distal pancreatectomy for traumatic pancreatic transection from 2000 to 2010., Results: Twenty-one patients without another indication for emergency laparotomy underwent a distal pancreatectomy for Grade III pancreatic injuries, of which 7 underwent laparoscopic distal pancreatectomy. Mean (±SD) age was 8.6±4.7 years, and 67% were male. There was no difference in the presence of other injuries between the two groups (43% in each group). Computed tomography revealed a transected pancreas in 85% of the laparoscopic patients and 75% of the open group (P=1.0). Mean operative time was 218±101 minutes with laparoscopy compared with 195±111 minutes with the open procedure (P=.7). Median duration of hospitalization was 6 days (range, 6-18 days) in the laparoscopic group compared with 11 days (range, 5-26 days) in the open group (P=0.3). Postoperative morbidity was not different between the two groups (57% versus 21% for laparoscopic versus open, P=.2)., Conclusions: Laparoscopy is equivalent to open distal pancreatectomy in children with select traumatic pancreatic injuries.

Kremens are high-affinity receptors for Dickkopf 1 (Dkk1) and regulate the Wnt/β-catenin signaling pathway by down-regulating the low-density lipoprotein receptor-related protein 6 (LRP6). Dkk1 competes with Wnt for binding to LRP6; binding of Dkk1 inhibits canonical signaling through formation of a ternary complex with Kremen. The majority of down-regulated clathrin-mediated endocytic receptors contain short conserved regions that recognize tyrosine or dileucine sorting motifs. In this study, we found that Kremen1 is internalized from the cell surface in a clathrin-dependent manner. Kremen1 contains an atypical dileucine motif with the sequence DXXXLV. Mutation of LV to AA in this motif blocked Kremen1 internalization; as reported previously for other proteins, the aspartic acid residue in Kremen1 is not critical. Inhibition of expression of the adaptor protein 2 (AP-2) or inhibition of clathrin by pitstop 2 also blocked Kremen1 internalization. The novel amino acid sequence identified in Kremen1 is similar to the motif previously identified in hydra, yeast, and other organisms known to signal from the trans-Golgi network to the endosomal compartment.

Objective: The goals of this study were to describe the clinical and anatomic features of infants undergoing Kasai portoenterostomy (KPE) for biliary atresia (BA) and to examine associations between these parameters and outcomes., Methods: Infants enrolled in the prospective Childhood Liver Disease Research and Education Network, who underwent KPE were studied. Patients enrolled in a blinded, interventional trial were excluded from survival analysis. Primary endpoints were successful surgical drainage (total bilirubin less than 2 mg/dL within the first 3 months), transplant-free survival (Kaplan-Meier), and time to transplant/death (Cox regression)., Results: KPE was performed in 244 infants (54% female; mean age: 65 ± 29 days). Transplant-free survival was 53.7% and 46.7% at 1 and 2 years post-KPE. The risk of transplant/death was significantly lower in the 45.6% of patients who achieved successful bile drainage within 3 months post-KPE (HR: 0.08, P < 0.001). The risk of transplant/death was increased in patients with porta hepatis atresia (Ohi type II and III vs type I; HR: 2.03, P = 0.030), nonpatent common bile duct (Ohi subtype: b, c, and d vs a; HR: 4.31, P = 0.022), BA splenic malformation syndrome (HR: 1.92, P = 0.025), ascites > 20 mL (HR: = 1.90, P = 0.0230), nodular liver appearance compared to firm (HR: = 1.61, P = 0.008), and age at KPE ≥ 75 days (HR: 1.73, P < 0.002). Outcome was not associated with gestational age, gender, race, ethnicity, or extent of porta hepatis dissection., Conclusion: Anatomic pattern of BA, BASM, presence of ascites and nodular liver appearance at KPE, and early postoperative jaundice clearance are significant predictors of transplant-free survival.

The mammalian embryo represents a fundamental paradox in biology. Its location within the uterus, especially early during development when embryonic cardiovascular development and placental blood flow are not well-established, leads to an obligate hypoxic environment. Despite this hypoxia, the embryonic cells are able to undergo remarkable growth, morphogenesis, and differentiation. Recent evidence suggests that embryonic organ differentiation, including pancreatic β-cells, is tightly regulated by oxygen levels. Since a major determinant of oxygen tension in mammalian embryos after implantation is embryonic blood flow, here we used a novel survivable in utero intracardiac injection technique to deliver a vascular tracer to living mouse embryos. Once injected, the embryonic heart could be visualized to continue contracting normally, thereby distributing the tracer specifically only to those regions where embryonic blood was flowing. We found that the embryonic pancreas early in development shows a remarkable paucity of blood flow and that the presence of blood flow correlates with the differentiation state of the developing pancreatic epithelial cells in the region of the blood flow., (Copyright © 2010 Elsevier Inc. All rights reserved.)

Organic-soluble transition metal-substituted Dawson compounds [(n-C(4)H(9))(4)N](9)[P(2)W(17)O(61)M(Br)] (M(n+) = Co(2+), Ni(2+), Cu(2+) and Zn(2+)), [(n-C(4)H(9))(4)N](7)[HP(2)W(17)O(61)M(Br)] (M(n+) = Cr(3+), Mn(3+) and Fe(3+)) and [K/(n-C(4)H(9))(4)N](10-n)[P(2)W(17)O(61)M(H(2)O)] (M(n+) = Ir(4+), Ru(3+) and Pd(2+)) have been investigated as oxygen transfer agents for H(2)O(2) to a series of primary allylic alcohols to generate epoxides under biphasic reaction conditions (1,2-dichloroethane/H(2)O) at 30 or 35 degrees C, such that the effect of variations in the substituted transition metals could be evaluated. The allylic alcohols involved the species R(1)R(2)C=C(R(3))CH(2)-OH (where R(1), R(2) and R(3) = H or Me), as well as cyclic (2-cyclohexen-1-ol), bicyclic [(R-)-(-)-myrtenol and (R-)-(-)-nopol] and species with two unsaturated sites (geraniol and nerol). The reactions are highly chemoselective and regioselective. The order of reactivity for the M(II)-substituted species is Pd(II) > Zn(II) > Co(II) > Ni(II), and for M(III) and M(IV) substitution is Mn(III) approximately Ir(IV) > Fe(III) > Cr(III). The observed orders are consistent with the formation of metal(n+)-alcohol species as part of the reaction mechanism. For the more polarizing Ir(IV), however, Ir(IV)-alcoholate species are likely involved in the mechanism. Formation constants for the Mn(III) and Co(II)-phosphopolyoxotungstate-alcohol species with all of the above alcohols have been evaluated in 1,2-dichloroethane at 25 degrees C and range from 19.0-3.5 M(-1). The most likely transition state involves coordination of the alcohol to the transition metal substituted at the lacunary site, or alkoxide in the case of Ir(IV), along with interaction of the double bond of the alcohol with a peroxo group located at a W(VI) site adjacent to the substituted transition metal.

The trend in synchrotron radiation (x-rays) is towards higher brilliance. This may lead to a very high power density, of the order of hundreds of watts per square millimetre at the x-ray optical elements. These elements are, typically, windows, polarizers, filters and monochromators. The preferred material for Bragg diffracting optical elements at present is silicon, which can be grown to a very high crystal perfection and workable size as well as rather easily processed to the required surface quality. This allows x-ray optical elements to be built with a sufficient degree of lattice perfection and crystal processing that they may preserve transversal coherence in the x-ray beam. This is important for the new techniques which include phase-sensitive imaging experiments like holo-tomography, x-ray photon correlation spectroscopy, coherent diffraction imaging and nanofocusing. Diamond has a lower absorption coefficient than silicon, a better thermal conductivity and lower thermal expansion coefficient which would make it the preferred material if the crystal perfection (bulk and surface) could be improved. Synthetic HPHT-grown (high pressure, high temperature) type Ib material can readily be produced in the necessary sizes of 4-8 mm square and with a nitrogen content of typically a few hundred parts per million. This material has applications in the less demanding roles such as phase plates: however, in a coherence-preserving beamline, where all elements must be of the same high quality, its quality is far from sufficient. Advances in HPHT synthesis methods have allowed the growth of type IIa diamond crystals of the same size as type Ib, but with substantially lower nitrogen content. Characterization of this high purity type IIa material has been carried out with the result that the crystalline (bulk) perfection of some of the HPHT-grown materials is approaching the quality required for the more demanding applications such as imaging applications and imaging applications with coherence preservation. The targets for further development of the type IIa diamond are size, crystal perfection, as measured by the techniques of white beam and monochromatic x-ray diffraction imaging (historically called x-ray topography), and also surface quality. Diamond plates extracted from the cubic growth sector furthest from the seed of the new low strain material produces no measurable broadening of the x-ray rocking curve width. One measures essentially the crystal reflectivity as defined by the intrinsic reflectivity curve (Darwin curve) width of a perfect crystal. In these cases the more sensitive technique of plane wave topography has been used to establish a local upper limit of the strain at the level of an 'effective misorientation' of 10(-7) rad.

We have previously reported that fibroblast growth factor 10 (FGF10) is crucial for the survival and proliferation of progenitor cells during embryonic gastrointestinal development. We sought to characterize the potential role of FGF10 signaling in the adaptive response following small bowel resection. Adult wild-type and Fgf10(LacZ) mice underwent 50% small bowel resection (SBR) or sham operation. Tissues were harvested 24 or 48 hr after surgery for histology, immunohistochemistry, and in situ hybridization. After SBR, Fgf10 expression was demonstrated in the epithelium at the base of the crypts. Moreover, there was a statistically significant increase in proliferating cells and goblet cells after SBR. In vitro studies using rat intestinal epithelial crypt (IEC-6) cells exposed to medium with or without recombinant FGF10 showed increased proliferation and phosphorylation of Raf and AKT with the addition of FGF10. Our results suggest that FGF10 may play a therapeutic role in diseases involving intestinal failure.

beta-Klotho, a newly described membrane protein, regulates bile acid synthesis. Fibroblast growth factor-15 (FGF-15) and FGF receptor-4 (FGFR4) knockout mice share a similar phenotype with beta-Klotho-deficient mice. FGF-15 secretion by the intestine regulates hepatic bile acid biosynthesis. The effects of beta-Klotho and FGF-15 on the ileal apical sodium bile transporter (ASBT) are unknown. beta-Klotho siRNA treatment of the mouse colon cancer cell line, CT-26, and the human intrahepatic biliary epithelial cells (HIBEC) resulted in upregulation of endogenous ASBT expression that was associated with reduced expression of the farnesoid X receptor (FXR) and the short heterodimer partner (SHP). Silencing beta-Klotho activated the ASBT promoter in CT-26, Mz-ChA-1 (human cholangiocarcinoma), and HIBEC cells. Site-directed mutagenesis of liver receptor homolog-1 (mouse) or retinoic acid receptor/retinoid X receptor (RAR/RXR) (human) cis-elements attenuated the basal activity of the ASBT promoter and abrogated its response to beta-Klotho silencing. siSHP, siFXR, or dominant-negative FXR treatment also eliminated the beta-Klotho response. FGF-15 secretion into cell culture media by CT-26 cells was diminished after siFGF-15 or sibeta-Klotho treatment and enhanced by chenodeoxycholic acid. Exogenous FGF-19 repressed ASBT protein expression in mouse ileum, gallbladder, and in HIBEC and repressed ASBT promoter activity in Caco-2, HIBEC, and Mz-ChA-1 cells. Promoter repression was dependent on the expression of FGFR4. These results indicate that both beta-Klotho and FGF-15/19 repress ASBT in enterocytes and cholangiocytes. These novel signaling pathways need to be considered in analyzing bile acid homeostasis.

Background/purpose: Primary gastric tumors are rare in infancy and childhood. Because of the infrequent occurrence of these tumors, the clinician may be unfamiliar with optimal management strategies. We review our experience over the past 54 years and the current literature., Methods: During the period extending from 1952 to 2006, 21 infants and children with primary gastric tumors were treated at Children's Hospital Los Angeles. The series includes 8 cases previously reported and 13 additional cases seen since the initial report. Follow-up information is included., Results: There were 12 males and 9 females, aged 12 days to 18 years, who were diagnosed with gastric tumors. The patients presented primarily with weight loss, vomiting, or an abdominal mass. Morphological analysis revealed gastric stromal tumors (n = 6), teratomas (n = 4), lymphomas (n = 4), adenocarcinomas (n = 2), inflammatory myofibroblastic tumors (n = 2), embryonal rhabdomyosarcoma (n = 1), and hamartomas (n = 3). There were 16 patients still alive (mean follow-up, 22.3 months), whereas 6 died from active disease despite multimodal treatment. The deaths occurred in patients with stromal tumors, adenocarcinomas, lymphomas, and rhabdomyosarcoma., Conclusions: Gastric tumors in children are rare. A high index of suspicion is needed to diagnose these tumors. Most malignant tumors present at an advanced stage and carry a substantial rate of mortality. They should be completely resected whenever feasible. In the case of some malignancies, chemotherapy may play a major role. Metastatic evaluation should be performed in all patients with malignant gastric tumors.

Management of children with intestinal failure is optimized by interdisciplinary coordination of parenteral and enteral nutrition support, medical management of associated complications, surgical lengthening procedures, and intestinal transplantation. Three hundred eighty-nine pediatric patients have been referred to our center for interdisciplinary assessment of intestinal failure since 1996 (median age=1 year; range 1 day-28.8 years). Factors predictive of weaning from parenteral nutrition without transplantation included increased mean bowel length for patients with gastroschisis (44 vs. 23 cm, p<0.05) and atresia (35 vs. 20 cm, p<0.01) and lower mean total bilirubin for patients with NEC (6.1 vs. 12.7 mg/dL, p<0.05). Others were also more likely to survive if referred with a lower mean total bilirubin (NEC, 7.9 vs. 12.7 mg/dL, p<0.05; pseudo-obstruction, 2.3 vs. 16.3 mg/dL, p<0.01). Patients weaned from parenteral nutrition by 2.5 years after referral achieved 95% survival at 5 years vs. 52% for those not weaned. Bowel lengthening procedures were performed on 25 patients. Eight subsequently weaned from parenteral nutrition without transplantation. Aggressive medical and nutritional intervention along with early referral, intestinal lengthening procedures, and intestinal transplantation in children with intestinal failure dependent on parenteral nutrition can result in the achievement of enteral autonomy and improved survival.

Primary choriocarcinoma of the liver is an extremely rare childhood malignancy frequently associated with clinical instability at initial presentation. It often mimics other benign and malignant childhood liver tumors. Prompt diagnosis and initiation of treatment are necessary to attain a successful outcome. We describe a critically ill infant with metastatic choriocarcinoma whose diagnosis was based on radiographic and tumor marker findings, without an initial biopsy, and her successful management with neo-adjuvant chemotherapy and delayed surgery. She is currently in continuous remission 24 months from diagnosis.