Effects of rare kidney diseases on kidney failure: a longitudinal analysis of the UK National Registry of Rare Kidney Diseases (RaDaR) cohort.

BACKGROUND: Individuals with rare kidney diseases account for 5-10% of people with chronic kidney disease, but constitute more than 25% of patients receiving kidney replacement therapy. The National Registry of Rare Kidney Diseases (RaDaR) gathers longitudinal data from patients with these conditions, which we used to study disease progression and outcomes of death and kidney failure. METHODS: People aged 0-96 years living with 28 types of rare kidney diseases were recruited from 108 UK renal care facilities. The primary outcomes were cumulative incidence of mortality and kidney failure in individuals with rare kidney diseases, which were calculated and compared with that of unselected patients with chronic kidney disease. Cumulative incidence and Kaplan-Meier survival estimates were calculated for the following outcomes: median age at kidney failure; median age at death; time from start of dialysis to death; and time from diagnosis to estimated glomerular filtration rate (eGFR) thresholds, allowing calculation of time from last eGFR of 75 mL/min per 1·73 m2 or more to first eGFR of less than 30 mL/min per 1·73 m2 (the therapeutic trial window). FINDINGS: Between Jan 18, 2010, and July 25, 2022, 27 285 participants were recruited to RaDaR. Median follow-up time from diagnosis was 9·6 years (IQR 5·9-16·7). RaDaR participants had significantly higher 5-year cumulative incidence of kidney failure than 2·81 million UK patients with all-cause chronic kidney disease (28% vs 1%; p<0·0001), but better survival rates (standardised mortality ratio 0·42 [95% CI 0·32-0·52]; p<0·0001). Median age at kidney failure, median age at death, time from start of dialysis to death, time from diagnosis to eGFR thresholds, and therapeutic trial window all varied substantially between rare diseases. INTERPRETATION: Patients with rare kidney diseases differ from the general population of individuals with chronic kidney disease: they have higher 5-year rates of kidney failure but higher survival than other patients with chronic kidney disease stages 3-5, and so are over-represented in the cohort of patients requiring kidney replacement therapy. Addressing unmet therapeutic need for patients with rare kidney diseases could have a large beneficial effect on long-term kidney replacement therapy demand. FUNDING: RaDaR is funded by the Medical Research Council, Kidney Research UK, Kidney Care UK, and the Polycystic Kidney Disease Charity.

Child Opportunity Index and Pediatric Extracorporeal Membrane Oxygenation Outcomes; the Role of Diagnostic Category.

OBJECTIVES: To study the impact of social determinants of health (SDoH) on pediatric extracorporeal membrane oxygenation (ECMO) outcomes. DESIGN, SETTING, AND PATIENTS: Retrospective study of children (< 18 yr) supported on ECMO (October 1, 2015 to March 1, 2021) using Pediatric Health Information System (44 U.S. children's hospitals). Patients were divided into five diagnostic categories: neonatal cardiac, pediatric cardiac, neonatal respiratory, pediatric respiratory, and sepsis. SDoH included the Child Opportunity Index (COI; higher indicates social advantage), race, ethnicity, payer, and U.S. region. Children without COI were excluded. Diagnostic category-specific clinical variables related to baseline health and illness severity were collected. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Children supported on ECMO experienced a 33% in-hospital mortality (2863/8710). Overall, children with lower COI, "other" race, Hispanic ethnicity, public insurance and from South or West regions had greater mortality. Associations between SDoH and ECMO outcomes differed between diagnostic cohorts. Bivariate analyses found that only pediatric cardiac patients had an association between COI or race and mortality. Multivariable logistic regression analyses examined relationships between SDoH, clinical variables and mortality within diagnostic categories. Pediatric cardiac patients had 5% increased odds of death (95% CI, 1.01-1.09) for every 10-point decrement in COI, while Hispanic ethnicity was associated with higher survival (adjusted odds ratio [aOR] 0.72 [0.57-0.89]). Children with heart disease from the highest COI quintile had less cardiac-surgical complexity and earlier cannulation. Independent associations with mortality were observed in sepsis for Black race (aOR 1.62 [1.06-2.47]) and other payer in pediatric respiratory patients (aOR 1.94 [1.23-3.06]). CONCLUSIONS: SDoH are statistically associated with pediatric ECMO outcomes; however, associations differ between diagnostic categories. Influence of COI was observed only in cardiac patients while payer, race, and ethnicity results varied. Further research should investigate differences between diagnostic cohorts and age groups to understand drivers of inequitable outcomes.

Limited exposure to pancreatic ERCP during advanced endoscopy training impacts competence and performance in independent practice.

BACKGROUND/OBJECTIVES: The impact of competency-based training programs on pancreatic endoscopic retrograde cholangiopancreatography (ERCP) performance remains unclear. This study aimed to describe the learning curves of pancreatic ERCP and subsequent performance during independent practice. METHODS: This was a multicenter prospective cohort study involving advanced endoscopy trainees (AETs). In the 1st phase, trainees were assessed on every 5th ERCP using the ERCP and EUS Skills Assessment Tool (TEESAT). Cumulative sum (CUSUM) analysis of pancreatic ERCP evaluations was used to establish learning curves. During the 2nd phase (1st year of independent practice), now-graduated participants documented their performance on key ERCP quality indicators. RESULTS: A total of 24 AETs (20 training programs) received sufficient evaluations for CUSUM analysis. Pancreatic ERCP accounted for 14.6 % (196/1339) of all ERCPs evaluated with 45 % of pancreatic ERCPs carrying a Grade 3 level of complexity. A minority of AETs (16.7 %) performed enough pancreatic ERCPs to generate meaningful learning curves with no AETs achieving competence in pancreatic cannulation, sphincterotomy, or stone clearance during Phase 1. In Phase 2, a total of 3620 ERCPs were performed, of which 281 (7.8 %) were pancreatic ERCPs. While the overall pancreatic duct cannulation rate was 92.2 %, the native papilla pancreatic duct cannulation rate was 85.7 %, which was below the recommended 90 % threshold. CONCLUSIONS: Advanced endoscopy training offers a low level of exposure to pancreatic ERCP, which is mirrored in independent practice, highlighting the inadequate training in pancreatic ERCP. Given the complexity of pancreatic ERCP, novel strategies are warranted to improve training in pancreatic ERCP.

Development of American Society for Gastrointestinal Endoscopy standards for training in advanced endoscopy within dedicated advanced endoscopy fellowship programs.

BACKGROUND AND AIMS: Training in interventional endoscopy is offered by nonaccredited advanced endoscopy fellowship programs (AEFPs). The number of these programs has increased dramatically with a concurrent increase in the breadth and complexity of interventional endoscopy procedures. Accreditation is governed by competency-based education, yet what constitutes a "high-quality" nonaccredited AEFP has not been defined. Using an evidence-based consensus process, we aimed to establish standards for AEFPs. METHODS: The RAND UCLA appropriateness method, a well-described modified Delphi process to develop quality indicators, was used. A task force established by the American Society for Gastrointestinal Endoscopy drafted potential quality indicators (structure, process, and outcome) in 6 categories: activity preceding training; structure of AEFPs; training in ERCP, EUS, and EMR; and luminal stent placement. Three rounds of iterative feedback from 20 experts were conducted. Round 0 involved discussion of project details. In round 1, experts independently ranked proposed quality indicators on a 9-point interval scale ranging from highly inappropriate (1) to highly appropriate (9). Next, proposed quality indicators were discussed and reworded in a group meeting followed by round 2, in which experts independently reranked proposed quality indicators and provided benchmarks (when applicable). The median score for each quality indicator was calculated. Mean absolute deviation from the median was calculated, and appropriateness of potential quality indicators was assessed using the BIOMED concerted action on appropriateness definition, P value method, and interpercentile range adjusted for symmetry definition. A quality indicator was deemed appropriate if the median score was ≥7 and met criteria for appropriateness using all 3 defined statistical methods. RESULTS: Of 89 proposed quality indicators, 37 statements met criteria as appropriate for a quality indicator (activity preceding training, 2; structure of AEFPs, 10; training in ERCP, 7; training in EUS, 8; training in EMR, 7; luminal stent placement, 3). Minimum thresholds were defined for 19 relevant quality indicators for number of trainers, procedures during fellowship, and procedures before assessment of competence. Among the final appropriate quality indicators were that all trainees should undergo qualitative and quantitative competence assessments using validated tools at least quarterly with documented feedback throughout the training period and that trainees should track outcomes and relevant quality metrics for specific procedures. CONCLUSIONS: This consensus process using validated methodology established standards for an AEFP in an effort to ensure adequate training in the most commonly taught interventional endoscopic procedures (ERCP, EUS, EMR, and luminal stent placement) during fellowship. An important component of an AEFP is the use of competency-based assessments that are compliant with the Accreditation Council for Graduate Medical Education's Next Accreditation System, with the goal of ensuring that trainees achieve specific milestones in their progression to achieving cognitive and technical competency.

Trends in analgesia-sedation of pediatric patients receiving I-131 MIBG in the pediatric intensive care unit: A report from the Pediatric Health Information System database.

BACKGROUND: Children with neuroblastoma receiving I-131 metaiodobenzylguanidine (MIBG) therapy require sedation-analgesia for strict radiation safety precautions during MIBG infusion and clearance. We evaluated the sedation-analgesia trends of patients undergoing MIBG therapy using the Pediatric Health Information System (PHIS) database. MATERIALS AND METHODS: Retrospective data from 476 patient encounters from the PHIS from 2010 to 2019. RESULTS: Total 240/476 (50.45%) children evaluated were under 6 years of age. Compared to 2010, in 2018 there was a decrease in benzodiazepine infusion use (60% vs. 40%, p < .04), as well as a decrease in use of opiate infusion (35% vs. 25%, p < .001). Compared to 2010, in 2018 we report an increase in the use of ketamine (from 5% to 10%, p < .002), as well as an increase in dexmedetomidine use (0% vs. 30%, p < .001). Dexmedetomidine was the most used medication in the 0-3 years age group compared to children older than 3 years of age (14.19% vs. 5.80%, p < .001). Opiate was the most used medication in children greater than 3 years compared to the 0-3-year age group (36.23 vs. 23.87, p < .05). CONCLUSION: Using PHIS data, we discovered considerable variability in the medications used for sedation in patients undergoing MIBG therapy. Although benzodiazepines and opioids were the most used agents, there was a trend toward decreasing use of benzodiazepines and opioids in these patients. Furthermore, there has been an increasing trend in the use of dexmedetomidine and ketamine.

Healthcare utilization disparities among children with high-risk neuroblastoma treated on Children's Oncology Group clinical trials.

INTRODUCTION: Disparities in relapse and survival from high-risk neuroblastoma (HRNBL) persist among children from historically marginalized groups even in highly standardized clinical trial settings. Research in other cancers has identified differential treatment toxicity as one potential underlying mechanism. Whether racial and ethnic disparities in treatment-associated toxicity exist in HRNBL is poorly understood. METHODS: This is a retrospective study utilizing a previously assembled merged cohort of children with HRNBL on Children's Oncology Group (COG) post-consolidation immunotherapy trials ANBL0032 and ANBL0931 at Pediatric Health Information System (PHIS) centers from 2005 to 2014. Race and ethnicity were categorized to reflect historically marginalized populations as Hispanic, non-Hispanic Black (NHB), non-Hispanic other (NHO), and non-Hispanic White (NHW). Associations between race-ethnicity and intensive care unit (ICU)-level care utilization as a proxy for treatment-associated toxicity were examined with log binomial regression and summarized as risk ratio (RR) and corresponding 95% confidence interval (CI). RESULTS: The analytic cohort included 370 children. Overall, 88 (23.8%) patients required ICU-level care for a median of 3.0 days (interquartile range [IQR]: 1.0-6.5 days). Hispanic children had nearly three times the risk of ICU-level care (RR 3.1, 95% CI: 2.1-4.5; fully adjusted RR [aRR] 2.5, 95% CI: 1.6-3.7) compared to NHW children and the highest percentage of children requiring cardiovascular-driven ICU-level care. CONCLUSION: Children of Hispanic ethnicity with HRNBL receiving clinical trial-delivered therapy were more likely to experience ICU-level care compared to NHW children. These data suggest that further investigation of treatment-related toxicity as a modifiable mechanism underlying outcome disparities is warranted.

Impoverished language in early childhood affects the development of complex sentence structure.

The hypothesis that impoverished language experience affects complex sentence structure development around the end of early childhood was tested using a fully randomized, sentence-to-picture matching study in American Sign Language (ASL). The participants were ASL signers who had impoverished or typical access to language in early childhood. Deaf signers whose access to language was highly impoverished in early childhood (N = 11) primarily comprehended structures consisting of a single verb and argument (Subject or Object), agreeing verbs, and the spatial relation or path of semantic classifiers. They showed difficulty comprehending more complex sentence structures involving dual lexical arguments or multiple verbs. As predicted, participants with typical language access in early childhood, deaf native signers (N = 17) or hearing second-language learners (N = 10), comprehended the range of 12 ASL sentence structures, independent of the subjective iconicity or frequency of the stimulus lexical items, or length of ASL experience and performance on non-verbal cognitive tasks. The results show that language experience in early childhood is necessary for the development of complex syntax. RESEARCH HIGHLIGHTS: Previous research with deaf signers suggests an inflection point around the end of early childhood for sentence structure development. Deaf signers who experienced impoverished language until the age of 9 or older comprehend several basic sentence structures but few complex structures. Language experience in early childhood is necessary for the development of complex sentence structure.

Trends in sedation-analgesia practices in pediatric liver transplant patients admitted postoperatively to the pediatric intensive care unit: An analysis of data from the pediatric health information system (PHIS) database.

BACKGROUND: Children admitted to the pediatric intensive care unit (PICU), after liver transplantation, frequently require analgesia and sedation in the immediate postoperative period. Our objective was to assess trends and variations in sedation and analgesia used in this cohort. METHODS: Multicenter retrospective cohort study using the Pediatric Health Information System from 2012 to 2022. RESULTS: During the study period, 3963 patients with liver transplantation were admitted to the PICU from 32 US children's hospitals with a median age of 2 years [IQR: 0.00, 10.00]. 54 percent of patients received mechanical ventilation (MV). Compared with patients without MV, those with MV were more likely to receive morphine (57% vs 49%, p < .001), fentanyl (57% vs 44%), midazolam (45% vs 31%), lorazepam (39% vs. 24%), dexmedetomidine (38% vs 30%), and ketamine (25% vs 12%), all p < .001. Vasopressor usage was also higher in MV patients (22% vs. 35%, p < .001). During the study period, there was an increasing trend in the utilization of dexmedetomidine and ketamine, but the use of benzodiazepine decreased (p < .001). CONCLUSION: About 50% of patients who undergo liver transplant are placed on MV in the PICU postoperatively and receive a greater amount of benzodiazepines in comparison with those without MV. The overall utilization of dexmedetomidine and ketamine was more frequent, whereas the administration of benzodiazepines was less during the study period. Pediatric intensivists have a distinctive opportunity to collaborate with the liver transplant team to develop comprehensive guidelines for sedation and analgesia, aimed at enhancing the quality of care provided to these patients.

Daily Diversity Flows: Racial and Ethnic Context Between Home and Work.

The racial and ethnic diversification of the U.S. population has transformed the demographic makeup of communities and rapidly increased exposure to diversity in American neighborhoods. Although diversity exposure occurs throughout people's daily lives, the conventional approach to describing diversity only at places of residence potentially understates the full extent of this phenomenon. In this study, we explore short-term, within-day changes in the diversity of different neighborhoods by considering U.S. workers' work and residential locations. Using estimates for daytime and nighttime populations among metropolitan census tracts, our empirical analyses investigate the extent to which the process of daytime mobility for work relates to changes in the racial and ethnic diversity of different spaces. Our results indicate widespread daily shifts toward diversity for most neighborhood types, especially those with residential (nighttime) populations that are predominantly Black, Latino, or Asian. We find that patterns of intraday diversification experienced minor declines across recent decades but are present in most metropolitan areas. Our findings also show that intraday changes in racial and ethnic diversity overlap with nonracial forms of daily diversity change. Further, average within-day changes in diversity are more pronounced in areas with greater residential segregation.

Race, Ethnicity, and Intensive Care Utilization for Common Pediatric Diagnoses: U.S. Pediatric Health Information System 2019 Database Study.

OBJECTIVES: Racial and ethnic disparities in healthcare delivery for acutely ill children are pervasive in the United States; it is unknown whether differential critical care utilization exists. DESIGN: Retrospective study of the Pediatric Health Information System (PHIS) database. SETTING: Multicenter database of academic children's hospitals in the United States. PATIENTS: Children discharged from a PHIS hospital in 2019 with one of the top ten medical conditions where PICU utilization was present in greater than or equal to 5% of hospitalizations. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Race and ethnicity categories included Asian, Black, Hispanic, White, and other. Primary outcomes of interest were differences in rate of PICU admission, and for children requiring PICU care, total hospital length of stay (LOS). One-quarter ( n = 44,200) of the 178,134 hospital discharges included a PICU admission. In adjusted models, Black children had greater adjusted odds ratio (aOR [95% CI]) of PICU admission in bronchiolitis (aOR, 1.08 [95% CI, 1.02-1.14]; p = 0.01), respiratory failure (aOR, 1.18 [95% CI, 1.10-1.28]; p < 0.001), seizure (aOR, 1.28 [95% CI, 1.08-1.51]; p = 0.004), and diabetic ketoacidosis (DKA) (aOR, 1.18 [95% CI, 1.05-1.32]; p = 0.006). Together, Hispanic, Asian, and other race children had greater aOR of PICU admission in five of the diagnostic categories, compared with White children. The geometric mean (± sd ) hospital LOS ranged from 47.7 hours (± 2.1 hr) in croup to 206.6 hours (± 2.8 hr) in sepsis. After adjusting for demographics and illness severity, children from families of color had longer LOS in respiratory failure, pneumonia, DKA, and sepsis. CONCLUSIONS: The need for critical care to treat acute illness in children may be inequitable. Additional studies are needed to understand and eradicate differences in PICU utilization based on race and ethnicity.

Trends and hospital practice variation for analgesia for children with sickle cell disease with vaso-occlusive pain episodes: An 11-year analysis.

This cross-sectional analysis of 86,111 visits for sickle cell disease and vaso-occlusive episodes (VOE) in U.S. pediatric emergency departments between 2013 and 2023 shows increased use of NSAIDs, ketamine, and acetaminophen, with unchanged opioid use. Hospitals with a higher volume of VOE visits more frequently administered opioids. BACKGROUND: Vaso-occlusive episodes (VOEs) are a hallmark of sickle cell disease (SCD), leading to frequent emergency department (ED) visits. Effective pain management is crucial, with guidelines recommending routine use of non-steroidal anti-inflammatory drugs (NSAIDs) with opioids, and emerging evidence supporting ketamine use. However, these recommendations are based on low-certainty evidence, and the impact of these guidelines on analgesia use over time remains unclear. OBJECTIVE: This study aimed to analyze trends in analgesia use over an 11-year period in pediatric SCD patients presenting to U.S. EDs with VOE and assess variations in treatment across hospitals. METHODS: A cross-sectional study was conducted using data from the Pediatric Health Information System covering 34 U.S. children's hospitals from January 1, 2013, to December 31, 2023. The primary outcomes were the proportions of visits where opioids, NSAIDs, acetaminophen, and/or ketamine were administered on the first calendar day of the initial visit. Secondary outcomes included the co-administration of NSAIDs with opioids. Logistic and linear regression models were used to assess trends and hospital-level variations. RESULTS: A total of 86,111 ED visits for VOE were analyzed. Opioids were administered in 82 % of encounters, NSAIDs in 72 %, acetaminophen in 17 %, and ketamine in 1 %. Co-administration of NSAIDs with opioids occurred in 59 % of the visits. Among discharged patients, there was a positive trend for NSAID use (slope: 1.68 %/year, 95 % CI: 0.91 %, 2.45 %) and NSAID-opioid co-administration (slope: 1.03 %/year, 95 % CI: 0.37 %, 1.69 %) over time. Acetaminophen use also increased over the study period (slope: 0.99 %/year, 95 % CI: 0.80 %, 1.17 %). In hospitalized patients, there was a significant upward trend for acetaminophen (slope: 1.29 %/year, 95 % CI: 0.69 %, 1.89 %) and ketamine (slope: 0.36 %/year, 95 % CI: 0.27 %, 0.45 %), while opioid use remained unchanged. Significant hospital-level variations were observed, with larger hospitals more likely to administer opioids but less likely to co-administer NSAIDs with opioids compared to medium-volume hospitals. CONCLUSION: Over the past decade, the use of NSAIDs, acetaminophen, and ketamine has increased in the management of VOE in pediatric SCD patients, while opioid use remains consistent. The co-administration of NSAIDs and opioids has also increased, reflecting guideline adherence. Variations in analgesia practices across hospitals underscore the need for standardizing pain management strategies in this population.

Guideline-Concordant Antiviral Treatment in Children at High Risk for Influenza Complications.

BACKGROUND: National guidelines recommend antiviral treatment for children with influenza at high risk for complications regardless of symptom duration. Little is known about concordance of clinical practice with this recommendation. METHODS: We performed a cross-sectional study of outpatient children (aged 1-18 years) at high risk for complications who were diagnosed with influenza during the 2016-2019 influenza seasons. High-risk status was determined using an existing definition that includes age, comorbidities, and residence in a long-term care facility. The primary outcome was influenza antiviral dispensing within 2 days of influenza diagnosis. We determined patient- and provider-level factors associated with guideline-concordant treatment using multivariable logistic regression. RESULTS: Of the 274 213 children with influenza at high risk for influenza complications, 159 350 (58.1%) received antiviral treatment. Antiviral treatment was associated with the presence of asthma (aOR, 1.13; 95% confidence interval [CI], 1.11-1.16), immunosuppression (aOR, 1.10; 95% CI, 1.05-1.16), complex chronic conditions (aOR, 1.04; 95% CI, 1.01-1.07), and index encounter in the urgent care setting (aOR, 1.3; 95% CI, 1.26-1.34). Factors associated with decreased odds of antiviral treatment include age 2-5 years compared with 6-17 years (aOR, 0.95; 95% CI, .93-.97), residing in a chronic care facility (aOR, .61; 95% CI, .46-.81), and index encounter in an emergency department (aOR, 0.66; 95% CI, .63-.71). CONCLUSIONS: Among children with influenza at high risk for complications, 42% did not receive guideline-concordant antiviral treatment. Further study is needed to elucidate barriers to appropriate use of antivirals in this vulnerable population.

Changing Landscape of Routine Pediatric Surgery for Rural and Urban Children: A Report From the Child Health Evaluation of Surgical Services (CHESS) Group.

OBJECTIVE: To describe the changes to routine pediatric surgical care over the past 2 decades for children living in urban and rural environments. BACKGROUND: A knowledge gaps exists regarding trends in the location where routine pediatric surgical care is provided to children from urban and rural environments over time. METHODS: Children (age 0-18) undergoing 7 common surgeries were identified using State Inpatient Databases (SID, 2002-2017). Rural-Urban Commuting Area codes were used to classify patient and hospital zip codes. Multivariable regression models for distance traveled >60 miles and transfer status were used to compare rural and urban populations, adjusting for year, age, sex, race, and insurance status. RESULTS: Among 143,467 children, 13% lived in rural zip codes. The distance traveled for care increased for both rural and urban children for all procedures but significantly more for the rural cohort (eg, 102% vs 30%, P <0.001, cholecystectomy). Transfers also increased for rural children (eg, transfers for appendectomy increased from 1% in 2002 to 23% in 2017, P <0.001). Factors associated with the need to travel >60 miles included year [adjusted odds ratio (aOR)=2.18, 95% CI: 1.94-2.46: 2017 vs 2002], rural residence (aOR=6.55, 95% CI: 6.11-7.01), age less than 5 years (aOR=2.17, 95% CI: 1.92-2.46), and Medicaid insurance (aOR=1.35, 95% CI: 1.26-1.45). Factors associated with transfer included year (aOR=5.77, 95% CI: 5.26-6.33: 2017 vs 2002), rural residence (aOR=1.47, 95% CI: 1.39-1.56), age less than 10 years (aOR=2.34, 95% CI: 2.15-2.54), and Medicaid insurance (aOR=1.49, 95% CI: 1.42-1.46). CONCLUSION: Rural children, younger age, and those on Medicaid disproportionately traveled greater distances and were more frequently transferred for common pediatric surgical procedures.

Varied Trainee Competence in Cold Snare Polypectomy: Results of the COMPLETE Randomized Controlled Trial.

INTRODUCTION: Cold snare polypectomy (CSP) is strongly recommended as the optimal technique for the complete removal of small polyps. Though significant variability in polypectomy technique and quality has been established, the learning curve and impact of targeted training on CSP are unknown. Video feedback has shown promise as an effective pedagogy to improve performance among surgical trainees. We aimed to compare CSP performance between trainees who received video-based feedback and those who received conventional apprentice-based concurrent feedback. We hypothesized that video-based feedback would accelerate competence. METHODS: We conducted a single-blinded, randomized controlled trial on competence for CSP of polyps <1 cm, comparing video-based feedback with conventional feedback. We randomly assigned deidentified consecutively recorded CSP videos to blinded raters to assess using the CSP Assessment Tool. We shared cumulative sum learning curves every 25 CSP with each trainee. The video feedback trainees also received biweekly individualized terminal feedback. Control trainees received conventional feedback during colonoscopy. The primary outcome was CSP competence. We also assessed competence across domains and change over polypectomy volume. RESULTS: We enrolled and randomized 22 trainees, 12 to video-based feedback and 10 to conventional feedback, and evaluated 2,339 CSP. The learning curve was long; 2 trainees (16.7%) in the video feedback achieved competence, after a mean of 135 polyps, and no one in the control ( P = 0.481) achieved competence. Overall and in all steps of CSP, a higher percentage of the video feedback group met competence, increasing 3% every 20 CSP ( P = 0.0004). DISCUSSION: Video feedback aided trainees to competence in CSP. However, the learning curve was long. Our findings strongly suggest that current training methods are not sufficient to support trainees to competency by the completion of their fellowship programs. The impact of new training methods, such as simulation-based mastery learning, should be assessed to determine whether such methods can result in achievement of competence at a faster rate; ClinicalTrials.gov : NCT03115008.

Acute-on-chronic liver failure before liver transplantation does not impact post-transplant survival in children with biliary atresia.

Acute-on-chronic liver failure (ACLF) occurs in children with biliary atresia (BA) awaiting liver transplantation (LT). However, data on transplant outcomes in ACLF are limited. Our aim was to characterize ACLF and determine its effect on transplant outcome and resource utilization. Using a linkage of the Scientific Registry of Transplant Recipients and Pediatric Health Information System, we identified children with BA between 3 months and 18 years at the time of listing who received a transplant from 2003 to 2018 and were hospitalized while waiting. ACLF was defined by the presence of at least 1 extra-hepatic organ failure during a pre-LT hospitalization. In all, 1044 patients (58% female, median age at listing 7.0 months IQR 5.0-14.0) were included. Thirty-four percent (351/1044) of the patients had at least 1 ACLF hospitalization. Patients with ACLF had longer waitlist times (114 [54-231] vs. 81 [35-181] days, p < 0.001), and were more likely to be listed as Status 1 (8% vs. 4%, p = 0.02). Pre-LT resource utilization was significantly higher in ACLF patients. There were no differences in mortality at 30 days (ACLF 3% vs. No ACLF 2%, p = 0.17), 90 days (ACLF 3% vs. No ACLF 2%, p = 0.24), 1 year (ACLF 3% vs. No ACLF 2%, p =0.23), 3 years (ACLF 4% vs. No ACLF 3%, p = 0.58), or 5 years (ACLF 5% vs. No ACLF 4%, p = 0.38) after LT. ACLF status was not associated with increased post-transplant mortality (adjusted HR 1.51, 95% CI 0.76-3.0, p =0.25). ACLF is an important morbidity in children with BA awaiting LT as it is associated with higher resource utilization and longer waitlist times. Further studies are needed to help understand the spectrum of ACLF and better prioritize critically ill children awaiting LT, as our study shows successful post-LT outcomes in children with BA and ACLF.

Neonates with acute liver failure have higher overall mortality but similar posttransplant outcomes as older infants.

Neonatal acute liver failure (ALF) carries a high mortality rate; however, little data exist on its peritransplant hospital course. This project aimed to identify factors associated with outcomes in neonates with ALF using large multicenter databases. Patients with International Classification of Diseases, Ninth Revision/International Classification of Diseases, Tenth Revision codes for liver failure (2004-2018) from linked Pediatric Health Information System and Scientific Registry of Transplant Recipients databases were assigned to two groups: neonates aged ≤30 days or older infants aged 31-120 days at admission. Billing data were used to assign diagnoses and assess patient comorbidities (sepsis, extracorporeal membrane oxygenation, total parenteral nutrition, intensive care unit, and cardiac/renal/respiratory failure). Statistical analysis included Kaplan-Meier survival curve analysis and univariate and multivariate analyses with the Cox proportional hazards model. We identified 1807 neonates and 890 older infants. Neonates had significantly lower survival to 90 days ( p = 0.04) and a lower rate of liver transplantation (2.0% vs. 6.4%; p < 0.001). Common risk factors associated with death or transplant were present between groups: diagnosis, respiratory failure, cardiac failure, and renal failure. Among neonates versus older infants who received a transplant, there was no significant differences in posttransplant lengths of stay (median 38 vs. 32 days; p = 0.53), posttransplant mortality (15% vs. 11%; p = 0.66), or graft loss (9.7% vs. 8.1%; p = 0.82). We present the largest multicenter study on peritransplant outcomes in neonatal ALF and show similar risk factors for death or transplant in neonates compared with older infants. Despite lower transplantation rates, neonates demonstrate similar posttransplant outcomes as older infants. Further studies are needed to better risk stratify neonates eligible for transplant and improve outcomes.

Steps on the Path to Clinical Translation: A workshop by the British and Irish Chapter of the ISMRM.

The British and Irish Chapter of the International Society for Magnetic Resonance in Medicine (BIC-ISMRM) held a workshop entitled "Steps on the path to clinical translation" in Cardiff, UK, on 7th September 2022. The aim of the workshop was to promote discussion within the MR community about the problems and potential solutions for translating quantitative MR (qMR) imaging and spectroscopic biomarkers into clinical application and drug studies. Invited speakers presented the perspectives of radiologists, radiographers, clinical physicists, vendors, imaging Contract/Clinical Research Organizations (CROs), open science networks, metrologists, imaging networks, and those developing consensus methods. A round-table discussion was held in which workshop participants discussed a range of questions pertinent to clinical translation of qMR imaging and spectroscopic biomarkers. Each group summarized their findings via three main conclusions and three further questions. These questions were used as the basis of an online survey of the broader UK MR community.

Tracheostomy, Feeding-Tube, and In-Hospital Postoperative Mortality in Children: A Retrospective Cohort Study.

BACKGROUND: Neuromuscular/neurologic disease confers increased risk of perioperative mortality in children. Some patients require tracheostomy and/or feeding tubes to ameliorate upper airway obstruction or respiratory failure and reduce aspiration risk. Empiric differences between patients with and without these devices and their association with postoperative mortality have not been previously assessed. METHODS: This retrospective cohort study using the Pediatric Health Information System measured 3- and 30-day in-hospital postsurgical mortality among children 1 month to 18 years of age with neuromuscular/neurologic disease at 44 US children's hospitals, from April 2016 to October 2018. We summarized differences between patients presenting for surgery with and without these devices using standardized differences. Then, we calculated 3- and 30-day mortality among patients with tracheostomy, feeding tube, both, and neither device, overall and stratified by important exposures, using Fisher exact test to test whether differences were significant. RESULTS: There were 43,193 eligible patients. Unadjusted 3-day mortality was 1.3% (549/43,193); 30-day mortality was 2.7% (1168/43,193). Most (79.1%) used neither a feeding tube or tracheostomy, 1.2% had tracheostomy only, 15.5% had feeding tube only, and 4.2% used both devices. Compared to children with neither device, children using either or both devices were more likely to have multiple CCCs, dysphagia, chronic pulmonary disease, cerebral palsy, obstructive sleep apnea, or malnutrition, and a prolonged intensive care unit (ICU) stay within the previous year. They were less likely to present for high-risk surgeries (33% vs 57%). Having a feeding tube was associated with decreased 3-day mortality overall compared to having neither device (0.9% vs 1.3%, P = .003), and among children having low-risk surgery, and surgery during urgent or emergent hospitalizations. Having both devices was associated with decreased 3-day mortality among children having low-risk surgery (0.8% vs 1.9%; P = .013), and during urgent or emergent hospitalizations (1.6% vs 2.9%; P = .023). For 30-day mortality, having a feeding tube or both devices was associated with lower mortality when the data were stratified by the number of CCCs. CONCLUSIONS: Patients requiring tracheostomy, feeding tube, or both are generally sicker than patients without these devices. Despite this, having a feeding tube was associated with lower 3-day mortality overall and lower 30-day mortality when the data were stratified by the number of CCCs. Having both devices was associated with lower 3-day mortality in patients presenting for low-risk surgery, and surgery during urgent or emergent hospitalizations.

Gentrification, Mobility, and Exposure to Contextual Social Determinants of Health.

This study uses individual level consumer trace data for 2006 residents of low- and moderate-income neighborhoods for the principal cities of the 100 largest metropolitan regions in the US using their location in 2006 and 2019 to examine exposure to the following four cSDOH: healthcare access (Medically Underserved Areas), socioeconomic condition (Area Deprivation Index), air pollution (NO2, PM 2.5 and PM10), and walkability (National Walkability Index). The results control for individual characteristics and initial neighborhood conditions. Residents of neighborhoods classified as gentrifying were exposed to more favorable cSDOH as of 2006 relative to residents of low- and moderate-income neighborhoods that were not gentrifying in terms of likelihood to be in a MUA, and level of local deprivation and walkability while experiencing similar level of air pollution. As a result of changes in neighborhood characteristics and differential mobility pattern, between 2006 and 2019, individuals who originally lived in gentrifying neighborhoods experienced worse changes in MUAs, ADI, and Walkability Index but a greater improvement in exposure to air pollutants. The negative changes are driven by movers, while stayers actually experience a relative improvement in MUAs and ADI and larger improvements in exposure to air pollutants. The findings indicate that gentrification may contribute to health disparities through changes in exposure to cSDOH through mobility to communities with worse cSDOH among residents of gentrifying neighborhoods although results in terms of exposure to health pollutants are mixed.

Mental Health Care Following Firearm and Motor Vehicle-related Injuries: Differences Impacting Our Treatment Strategies.

OBJECTIVE: To compare new mental health diagnoses (NMHD) in children after a firearm injury versus following a motor vehicle collision (MVC). BACKGROUND: A knowledge gap exists regarding childhood mental health diagnoses following firearm injuries, notably in comparison to other forms of traumatic injury. METHODS: We utilized Medicaid MarketScan claims (2010-2016) to conduct a matched case-control study of children ages 3 to 17 years. Children with firearm injuries were matched with up to 3 children with MVC injuries. Severity was determined by injury severity score and emergency department disposition. We used multivariable logistic regression to measure the association of acquiring a NMHD diagnosis in the year postinjury after firearm and MVC mechanisms. RESULTS: We matched 1450 children with firearm injuries to 3691 children with MVC injuries. Compared to MVC injuries, children with firearm injuries were more likely to be black, have higher injury severity score, and receive hospital admission from the emergency department ( P <0.001). The adjusted odds ratio (aOR) of NMHD diagnosis was 1.55 [95% confidence interval (95% CI): 1.33-1.80] greater after firearm injuries compared to MVC injuries. The odds of a NMHD were higher among children admitted to the hospital compared to those discharged. The increased odds of NMHD after firearm injuries was driven by increases in substance-related and addictive disorders (aOR: 2.08; 95% CI: 1.63-2.64) and trauma and stressor-related disorders (aOR: 2.07; 95% CI: 1.55-2.76). CONCLUSIONS: Children were found to have 50% increased odds of having a NMHD in the year following a firearm injury as compared to MVC. Programmatic interventions are needed to address children's mental health following firearm injuries.