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Venous thromboembolism (VTE) poses a significant risk to cancer patients receiving systemic therapy. The generalizability of pan-cancer models to lymphomas is limited. Currently, there are no reliable risk prediction models for thrombosis in patients with lymphoma. Our objective was to create a risk assessment model (RAM) specifically for lymphomas. We performed a retrospective cohort study to develop Fine and Gray sub-distribution hazard model for VTE and pulmonary embolism (PE)/ lower extremity deep vein thrombosis (LE-DVT) respectively in adult lymphoma patients from the Veterans Affairs national healthcare system (VA). External validations were performed at the Harris Health System (HHS) and the MD Anderson Cancer Center (MDACC). Time-dependent c-statistic and calibration curves were used to assess discrimination and fit. There were 10,313 (VA), 854 (HHS), and 1858 (MDACC) patients in the derivation and validation cohorts with diverse baseline. At 6 months, the VTE incidence was 5.8% (VA), 8.2% (HHS), and 8.8% (MDACC), respectively. The corresponding estimates for PE/LE-DVT were 3.9% (VA), 4.5% (HHS), and 3.7% (MDACC), respectively. The variables in the final RAM included lymphoma histology, body mass index, therapy type, recent hospitalization, history of VTE, history of paralysis/immobilization, and time to treatment initiation. The RAM had c-statistics of 0.68 in the derivation and 0.69 and 0.72 in the two external validation cohorts. The two models achieved a clear differentiation in risk stratification in each cohort. Our findings suggest that easy-to-implement, clinical-based model could be used to predict personalized VTE risk for lymphoma patients., (© 2024 Wiley Periodicals LLC.)

Introduction Neck adiposity has been related to cardiovascular risk in healthy and nonhealthy individuals. Our objective was to evaluate the utility of anatomic neck measurements extracted from computed tomography (CT) examinations as a predictor of cardiovascular disease and its risk factors. Methods We investigated patients who had a CT neck examination with intravenous contrast performed at two hospitals between 2013 and 2020. Patients with a neck malignancy, prior neck surgery, age <18 years, incomplete demographic information, and inadequate image quality were excluded. We performed 18 separate measurements of neck anatomy which were correlated with cardiovascular risk factors and disease, as well as relevant lab values and medications. All multivariable linear regressions were controlled for gender and BMI. Associations with p<0.05 were considered statistically significant. The measurements were then used to predict hypertension using random forest, a non-linear prediction algorithm. Results Approximately 20,000 neck CT examinations with contrast were performed between 2013-2020. After applying the inclusion criteria, 458 patients remained in the study population. Eight measurements (all of which include a component of neck adiposity) showed a statistically significant association between anatomic measurements and cardiovascular risk factors. The risk factor most often associated with increases in CT measurements was type 2 diabetes. Accordingly, patients on insulin treatment had a significantly higher average for all eight measurements. Significant measurement increases were also found in those previously diagnosed with hyperlipidemia and in those being treated with hypertension medications. The area under the receiver operating characteristic (AUROC) value of the random forest prediction algorithm was 0.68, meaning our measurements were a good predictor of hypertensive disease status. Conclusion Adipose tissue measurements extracted from CT examinations of the neck are associated with cardiovascular risk factors including hypertension, diabetes, and hyperlipidemia. Machine learning models of anatomic neck measurements can potentially identify patients at risk for cardiovascular disease., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Baylor College of Medicine Institutional Review Board issued approval H-47632. Animal subjects: All authors have confirmed that this study did not involve animal subjects or tissue. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Lopez et al.)

Background: The transition of the Accreditation Council for Graduate Medical Education (ACGME) to milestone assessment creates opportunities for collaboration and shared assessments across graduate medical programs. Breaking bad news is an essential communication skill that is a common milestone across almost every medical specialty. The purpose of this study was to develop and pilot an integrated milestone assessment (IMA) tool for breaking bad news using ACGME milestone criteria and to compare the IMA tool with the existing SPIKES protocol., Methods: The IMA tool was created using sub-anchors in professionalism and interpersonal communication skills that are applicable to every specialty and to the ability to break bad news. Two cases of breaking bad news, designed to be "easy" and "intermediate" in difficulty, were used to assess basic skills in breaking bad news in first-year medical residents from six residency specialties. Eight standardized patients were trained to portray the cases in sessions held in November 2013 and May 2014. Standardized patients completed an assessment checklist to evaluate each resident's performance in breaking bad news based on their use of the SPIKES protocol and IMA tool. Residents answered post-encounter questions about their training and comfort in breaking bad news. The association between SPIKES and IMA scores was investigated by simple linear regression models and Spearman rank correlations., Results: There were 136 eligible medical residents: 108 (79.4%) participated in the first session and 97 (71.3%) participated in the second session, with 96 (70.6%) residents participating in both sessions. Overall, we were able to identify residents that performed at both extremes of the assessment criteria using the integrated milestone assessment (IMA) and the SPIKES protocol. Interestingly, residents rated themselves below "comfortable" on average., Conclusion: We developed an integrated milestone assessment (IMA) that was better than the SPIKES protocol at assessing the skill of breaking bad news. This collaborative assessment tool can be used as supplement tool in the era of milestone transformation. We aim assess our tool in other specialties and institutions, as well as assess other shared milestones across specialties., (© 2024. The Author(s).)

Purpose: Performance status (PS), an essential indicator of patients' functional abilities, is often documented in clinical notes of patients with cancer. The use of natural language processing (NLP) in extracting PS from electronic medical records (EMRs) has shown promise in enhancing clinical decision-making, patient monitoring, and research studies. We designed and validated a multi-institute NLP pipeline to automatically extract performance status from free-text patient notes., Patients and Methods: We collected data from 19,481 patients in Harris Health System (HHS) and 333,862 patients from veteran affair's corporate data warehouse (VA-CDW) and randomly selected 400 patients from each data source to train and validate (50%) and test (50%) the proposed pipeline. We designed an NLP pipeline using an expert-derived rule-based approach in conjunction with extensive post-processing to solidify its proficiency. To demonstrate the pipeline's application, we tested the compliance of PS documentation suggested by the American Society of Clinical Oncology (ASCO) Quality Metric and investigated the potential disparity in PS reporting for stage IV non-small cell lung cancer (NSCLC). We used a logistic regression test, considering patients in terms of race/ethnicity, conversing language, marital status, and gender., Results: The test results on the HHS cohort showed 92% accuracy, and on VA data demonstrated 98.5% accuracy. For stage IV NSCLC patients, the proposed pipeline achieved an accuracy of 98.5%. Furthermore, our analysis revealed a documentation rate of over 85% for PS among NSCLC patients, surpassing the ASCO Quality Metrics. No disparities were observed in the documentation of PS., Conclusion: Our proposed NLP pipeline shows promising results in extracting PS from free-text notes from various health institutions. It may be used in longitudinal cancer data registries.

Transplantation-associated thrombotic microangiography (TA-TMA) is a disorder that causes severe complications after allogeneic hematopoietic cell transplantation (allo-HCT). Diagnosing TA-TMA is challenging because of the lack of standardized criteria. In this study, we aimed to evaluate the new TA-TMA consensus definition from the American Society for Transplantation and Cellular Therapy (ASTCT) panel as part of an ongoing prospective pediatric cohort study, and also to compare the impact and outcomes of using the current definition of clinical TMA (cTMA) versus the new consensus definition. We included patients age 0 to 18 years who underwent their first allo-HCT between May 2021 and January 2023 at Texas Children's Hospital. We compared the incidence, biomarkers, and outcomes of TA-TMA applying the previous and recently proposed screening algorithms and definitions. Whereas use of the classic microangiopathic hemolytic anemia (MAHA)-based cTMA definition led to an incidence of 12.7% by day 100 post-transplantation, the ASTCT-HR definition doubled the incidence to 28.5% by day 100. In contrast to patients with a concordant diagnosis (+/+), who had significantly worse post-transplantation survival, those reclassified as TA-TMA only by the new definition (-/+) had a significantly different prognosis (100% survival at day 100) despite the lack of TMA-directed therapy. Furthermore, biomarkers of the terminal and alternative complement pathways (sC5b9 and Ba, respectively) were significantly elevated compared with non-TMA patients around day 15 in the concordant group (+/+) but not in the discordant group (-/+). The recently proposed ASTCT consensus TA-TMA diagnosis is more sensitive and allows earlier recognition of manifestation that requires closer clinical monitoring but risks overdiagnosis and overtreatment. We recommend additional prospective validation., (Copyright © 2023 The American Society for Transplantation and Cellular Therapy. Published by Elsevier Inc. All rights reserved.)

Introduction: Chloral hydrate (CH) has long been utilized as a pediatric procedural sedation agent. However, very little is published describing CH use in a pediatric intensive care unit (PICU) setting. The aim of this retrospective observational cohort study was to investigate and describe the use of CH in mechanically-ventilated, critically ill children at a large pediatric tertiary referral hospital. Methods: Data were extracted from the hospital electronic medical record and a locally maintained registry of all children admitted to the PICU between 2012 and 2017. Patients admitted to the cardiovascular ICU were not included in this review. The clinical and pharmacy data for 3806 consecutive PICU admissions of mechanically-ventilated, critically ill children were examined. Results: 283 admissions received CH during their first ICU stay. CH-exposed children were younger (16 months vs. 35 months, p < 0.001), the median total dose of CH (indexed to duration of ventilation) was 11 mg/kg/day, the median time to first CH dose was 3 days and more CH doses were administered at night (1112 vs. 958, p < 0.001). We constructed a propensity score to adjust for the differences in patients with and without CH exposure using logistic regression including variables of age, sex, diagnosis, and PRISM3 score. After adjustment, the median length of mechanical ventilation was 5 days longer in the CH-exposed group (95% Confidence Interval [CI] 4-6) compared to unexposed CH patients. Similarly, the median length of ICU duration was 9.4 days longer (95% CI 7.1-11.6) and median length of hospital admission duration was 13.2 days longer (95% CI 7.8-18.6) in CH-exposed patients compared to CH-non-exposed. After adjustment, CH-exposed patients had a 9% higher median exposure to HFOV (95% CI 3.9-14.6), but did not have higher median exposures to new tracheostomy (95% CI -0.4-2.2) or ECMO (95% CI -0.2-5.0). Discussion: As part of an extended sedation regimen in mechanically-ventilated and critically ill children, CH is associated with somewhat higher complexity of illness and longer ICU durations., Competing Interests: Author JC was employed by Tableau Software. The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2023 Ettinger, Kiskaddon, McNeely, Craycraft, Rogers, Achuff, Guffey and Musick.)

Background: Juvenile localized scleroderma (LS) and systemic sclerosis (SSc) are rare pediatric conditions often associated with severe morbidities. Delays in diagnosis are common, increasing the risk for permanent damage and worse outcomes. This study explored caregiver perspectives on barriers they encountered while navigating diagnosis and care for their child's scleroderma., Methods: In this cross-sectional study, caregivers of juvenile LS or SSc patients were recruited from a virtual family scleroderma educational conference and a juvenile scleroderma online interest group. The survey queried respondents about their child's condition and factors affecting diagnosis and treatment., Results: The response rate was 61% (73/120), with 38 parents of LS patients and 31 parents of SSc patients. Most patients were female (80%) and over half were non-Hispanic white (55%). Most families had at least one person with a college education or higher (87%), traveled ≤ 2 h to see their rheumatologist (83%), and had private insurance (75%). Almost half had an annual household income ≥ $100,000 (46%). Families identified the following factors as barriers to care: lack of knowledge about scleroderma in the medical community, finding reliable information about pediatric scleroderma, long wait times/distances for a rheumatology/specialist appointment, balance of school/work and child's healthcare needs, medication side effects, and identifying effective medications. The barrier most identified as a major problem was the lack of knowledge about juvenile scleroderma in the medical community. Public insurance, household income less than $100,000, and Hispanic ethnicity were associated with specific barriers to care. Lower socioeconomic status was associated with longer travel times to see the rheumatologist/specialist. Diagnosis and systemic treatment initiation occurred at greater than one year from initial presentation for approximately 28% and 36% of patients, respectively. Families of LS patients were commonly given erroneous information about the disease, including on the need and importance of treating active disease with systemic immunosuppressants in patients with deep tissue or rapidly progressive disease., Conclusion: Caregivers of children with LS or SSc reported numerous common barriers to the diagnosis, treatment, and ongoing care of juvenile scleroderma. The major problem highlighted was the lack of knowledge of scleroderma within the general medical community. Given that most of the caregiver respondents to the survey had relatively high socioeconomic status, additional studies are needed to reach a broader audience, including caregivers with limited English proficiency, geographical limitations, and financial constraints, to determine if the identified problems are generalizable. Identifying key care barriers will help direct efforts to address needs, reduce disparities in care, and improve patient outcomes., (© 2023. The Author(s).)

Data on outcomes and interventions for children with sickle cell disease (SCD) admitted to a pediatric intensive care units (PICU) are unknown. We provide the first comprehensive multi-center report on PICU interventions associated with death, the need for invasive respiratory support or stroke among critically ill children with SCD. We collected retrospective multi-center cohort data from January 1, 2012 to December 31, 2019 utilizing the Virtual Pediatric Systems, LLC database. We identified 3388 unique children with SCD, accounting for a total of 5264 PICU admissions from 138 PICUs. The overall mortality rate for the PICU admissions cohort was 1.8% (95/5264 PICU admissions, 95/3388 [2.8%] of all unique patients), the rate of needing of needing Invasive Respiratory Support (IRS, a composite category of exposure) was 21.3% (872/4093 PICU admissions with complete data) and the overall rate of stroke (ischemic or hemorrhagic) was 12.5% (657/5264 PICU admissions). In multivariable analysis adjusting for admission age category, sex, race/ethnicity, PRISM-3 score at admission, exposure to IRS, quartile of unit volume of patients with SCD, and patient origin, admitted children who needed invasive respiratory support (IRS) had higher adjusted odds ratios for mortality (adjusted odds ratio [aOR], 19.72; 95% confidence interval [CI] 8.98-43.29; p < 0.001), although admitted children > 2 years old had decreased aOR for needing IRS (aOR 0.25-0.62; 95% CI 0.16-0.94; p < 0.001-0.025). By contrast, admitted children > 2 years old had a strikingly increased aOR for stroke (aOR 7.57-16.32; 95% CI 2.25-52.15; p < 0.001). These groups may represent PICU-specific subsets of patients with SCD who are at higher risk for more serious illness and should deserve early consideration for referral to a pediatric institution providing comprehensive care for patients with SCD., (© 2023. The Author(s).)

Children presenting with acute myocarditis may experience rapid clinical deterioration requiring extracorporeal membrane oxygenation (ECMO); however, our understanding of best practices and timing of ECMO initiation are lacking. We explored the relationships between pre-cannulation factors and survival in this high-acuity patient population., Design: Retrospective review of a large international registry. Primary outcome was survival to hospital discharge, stratified by incident cardiac arrest (CA) prior to ECMO and time to cannulation after intubation., Setting and Subjects: The Extracorporeal Life Support Organization registry was queried for patients less than or equal to 18 years old receiving ECMO support for myocarditis between 2007 and 2018. Exclusion criteria included being nonindex runs, non-venoarterial ECMO or missing data points for main variables studied., Interventions: None., Measurements and Main Results: Population characteristics and survival were compared using t test, Wilcoxon rank-sum test, or Fisher exact test. Multivariable logistic regression was used for significant factors in the unadjusted logistic regression. Among 506 index ECMO runs in pediatric patients with myocarditis, survival for the cohort was 72%, with no difference between early and late eras (2007-2012 vs 2013-2018; p = 0.69). Survivors demonstrated higher pre-ECMO pH levels as well as shorter intubation-to-cannulation (ITC) times (3 hr [interquartile range (IQR)], 1-14 hr vs 6 hr [IQR, 2-20 hr]; p = 0.021). CA occurred within 24 hours prior to ECMO cannulation, including extracorporeal cardiopulmonary resuscitation, in 54% of ECMO runs ( n = 273). Accounting for the interaction between pre-ECMO CA occurrence and ITC time, longer ITC time remained associated with lower survival for patients who did not experience a CA prior to ECMO, with adjusted odds ratio of 0.09 (IQR, 0.02-0.40; p = 0.002) for ITC time greater than or equal to 18 hours., Conclusions: The results of this multicenter analysis of ECMO utilization and outcomes for pediatric myocarditis suggest that patients approaching ECMO cannulation who have not experienced CA may have better survival outcomes if cannulated onto ECMO early after intubation., Competing Interests: Dr. Thiagarajan received funding from the Extracorporeal Life Support Organization and Society of Critical Care Medicine. Dr. Alexander’s institution received funding from Novartis. The remaining authors have disclosed that they do not have any potential conflicts of interest., (Copyright © 2022 The Authors. Published by Wolters Kluwer Health, Inc. on behalf of the Society of Critical Care Medicine.)

Background: Infants who undergo surgery for congenital heart disease are at risk of neurodevelopmental delay. Cardiac surgery-associated acute kidney injury (CS-AKI) is common but its association with neurodevelopment has not been explored., Methods: This was a single-center retrospective observational study of infants who underwent cardiac surgery in the first year of life who had neurodevelopmental testing using the Bayley Scale for Infant Development, third edition. Single and recurrent episodes of stages 2 and 3 CS-AKI were determined., Results: Of 203 children with median age at first surgery of 12 days, 31% had one or more episodes of severe CS-AKI; of those, 16% had recurrent CS-AKI. Median age at neurodevelopmental assessment was 20 months. The incidence of delay was similar for patients with and patients without CS-AKI but all children with recurrent CS-AKI had a delay in one or more domains and had significantly lower scores in all three domains, namely, cognitive, language, and motor., Conclusions: This study has assessed the association of CS-AKI with neurodevelopmental delay after surgery for congenital heart disease in infancy. Infants who have recurrent CS-AKI in the first year of life are more likely to be delayed and have lower neurodevelopmental scores., (Copyright © 2022 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

The epidemiology of cancer-associated thrombosis (CAT) among uninsured and vulnerable populations in the US is not well-characterized. We performed a retrospective cohort study for patients with newly diagnosed cancer from 2011 to 2020 at Harris Health System, which cares for uninsured residents in the Houston metropolitan area. Patient demographics, NCI comorbidity index, area of deprivation index (ADI), cancer histology, staging, and systemic therapy data were extracted. CAT included overall venous thromboembolism (VTE) or pulmonary embolism +/- lower extremity deep vein thrombosis (PE/LE-DVT) within 1 year of diagnosis. We used multivariable Fine-Gray models to assess the associations with CAT accounting for death as a competing risk. Among 15 342 patients, 74% were uninsured and 84% lived in socioeconomically disadvantaged neighborhoods. There were 16% Non-Hispanic White (NHW), 28% Non-Hispanic Black (NHB), 50% Hispanic (27% Mexican), and 6% Asian/Pacific Islanders (API). The 1-year CAT incidence rate was 14.6%. Overall VTE was lower for Hispanics versus NHW (SHR 0.87 [0.76-0.99]) and API versus NHW (SHR 0.58 [0.44-0.77]). PE/LE-DVT was higher for NHB versus NHW (SHR 1.18 [1.01-1.39]). CAT was also associated with chemotherapy-based regimens (+/- immunotherapy), age, obesity, cancer type/staging, VTE history, and recent hospitalization. NCI comorbidity and ADI scores were associated with mortality but not CAT. In a large cohort of underserved patients with cancer, we identified an elevated incidence of CAT with known and novel risk predictors. Hispanics had lower adjusted rates of CAT and mortality. Our findings highlight the need to investigate and incorporate vulnerable populations in clinical trials., (© 2022 Wiley Periodicals LLC.)

Background: Increasing numbers of adults with congenital heart disease (ACHD) undergo cardiac surgical procedures in children's hospitals, yet surgical outcomes data are limited. We sought to better understand the impact of preoperative risk factors on postoperative complications and cardiac intensive care unit (CICU) length of stay (LOS)., Methods: Surgical CICU admissions for patients aged 18 years and older in the Pediatric Cardiac Critical Care Consortium registry from August 2014 to January 2019 in 34 hospitals were included. Primary outcomes included prolonged LOS (defined as LOS ≥90th percentile) and major complications (cardiac arrest, extracorporeal membrane oxygenation, arrhythmia requiring intervention, stroke, renal replacement therapy, infection, and reoperation/reintervention)., Results: We analyzed 1764 surgical CICU admissions. Prolonged LOS was 7 days or longer. Eighteen patients (1.0%) died, of whom 9 (0.5%) died before the LOS cutoff and were excluded from analysis. Of 1755 CICU admissions, 8.8% (n = 156) had prolonged LOS, and 23.3% (n = 413) had 1 or more major complications. Several variables, including The Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery 4/5 operation, 3 or more previous sternotomies, and preoperative renal dysfunction/dialysis were independent risk factors for both prolonged LOS and major complications (P < .05). Preoperative ventilation was associated with increased odds of prolonged LOS and preoperative arrhythmia with major complications., Conclusions: This analysis of postoperative ACHD care in pediatric CICUs found high complexity operations, 3 or more previous sternotomies, preoperative arrhythmias, renal dysfunction, and respiratory failure are associated with prolonged LOS and/or major complications. Future quality improvement initiatives focused on preoperative optimization and implementation of adult-specific perioperative protocols may mitigate morbidity in these patients undergoing cardiac surgical procedures at children's hospitals., (Copyright © 2022 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Research on venous thromboembolism (VTE) that relies only on the International Classification of Diseases (ICD) can misclassify outcomes. Our study aims to discover and validate an improved VTE computable phenotype for people with cancer., Methods: We used a cancer registry electronic health record (EHR)-linked longitudinal database. We derived three algorithms that were ICD/medication based, natural language processing (NLP) based, or all combined. We then randomly sampled 400 patients from patients with VTE codes (n = 1111) and 400 from those without VTE codes (n = 7396). Weighted sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) were calculated on the entire sample using inverse probability weighting, followed by bootstrapped receiver operating curve analysis to calculate the concordance statistic (c statistic)., Results: Among 800 patients sampled, 280 had a confirmed acute VTE during the first year after cancer diagnosis. The ICD/medication algorithm had a weighted PPV of 95% and a weighted sensitivity of 81%, with a c statistic of 0.90 (95% confidence interval [CI], 0.89-0.91). Adding Current Procedural Terminology codes for inferior vena cava filter removal or early death did not improve the performance. The NLP algorithm had a weighted PPV of 80% and a weighted sensitivity of 90%, with a c statistic of 0.93 (95% CI, 0.92-0.94). The combined algorithm had a weighted PPV of 98% at the higher cutoff and a weighted sensitivity of 96% at the lower cutoff, with a c statistic of 0.98 (95% CI, 0.97-0.98)., Conclusions: Our ICD/medication-based algorithm can accurately identify VTE phenotype among patients with cancer with a high PPV of 95%. The combined algorithm should be considered in EHR databases that have access to such capabilities., (© 2022 The Authors. Research and Practice in Thrombosis and Haemostasis published by Wiley Periodicals LLC on behalf of International Society on Thrombosis and Haemostasis (ISTH).)

Objectives: To determine if indices of alveolar gas exchange preextubation predict postextubation respiratory support needs as well as the need for escalation of therapies following infant cardiac surgery., Design: Retrospective chart review., Setting: Pediatric cardiac ICU in a quaternary-care teaching hospital., Patients: Infants less than 1 year old who underwent biventricular repair from January 2015 to December 2017., Interventions: None., Measurements and Main Results: Preextubation alveolar-arterial gradient, oxygenation index, oxygen saturation index, Pao 2 /Fio 2 ratio, and dead space ventilation (analyzed with both end-tidal carbon dioxide gradient and dead space fraction) were evaluated for each patient. All but dead space ventilation were associated with a higher level of noninvasive respiratory support immediately postextubation. Furthermore, impaired preextubation gas exchange was independently associated with escalation of respiratory support within the first 48-hour postextubation., Conclusions: Validated measures of alveolar gas exchange can be used as a tool to assess postextubation respiratory support needs including the risk of escalation of respiratory support in the first 48-hour postextubation. Prospective study with implementation of extubation guidelines, both for readiness and determination of early postextubation support, is needed to validate these findings., Competing Interests: The authors have disclosed that they do not have any potential conflicts of interest., (Copyright © 2022 The Authors. Published by Wolters Kluwer Health, Inc. on behalf of the Society of Critical Care Medicine.)

Background: Social determinants of health (SDH) are known to impact hospital and intensive care unit (ICU) outcomes. Little is known about the association between SDH and pediatric rapid response (RR) events and understanding this impact will help guide future interventions aimed to eliminate health disparities in the inpatient setting., Objectives: The primary objective of this study is to describe the association between SDH and RR utilization (number of RR events, time to RR event, shift of event and caller). The secondary objective is to determine if SDH can predict hospital length of stay (LOS), ICU transfer, critical deterioration (CD), and mortality., Methods: A retrospective cohort study was conducted. We reviewed all RR events from 2016 to 2019 at a large, academic, pediatric hospital system including a level 1 trauma center and two satellite community campuses. All hospitalized patients up to age 25 who had a RR event during their index hospitalization were included. Exposure variables included age, gender, race/ethnicity, language, income, insurance status, chronic disease status, and repeat RR event. The primary outcome variables were hospital LOS, ICU transfer, CD, and mortality. The odds of mortality, CD events and ICU transfer were assessed using unadjusted and multivariable logistic regression. Associations with hospital LOS were assessed with unadjusted and multivariable quantile regression., Results: Four thousand five hundred and sixty-eight RR events occurred from 3,690 unique admissions and 3301 unique patients, and the cohort was reduced to the index admission. The cohort was largely representative of the population served by the hospital system and varied according to race and ethnicity. There was no variation by race/ethnicity in the number of RR events or the shift in which RR events occurred. Attending physicians initiated RR calls more for event for non-Hispanic patients of mixed or other race (31.6% of events), and fellows and residents were more likely to be the callers for Hispanic patients (29.7% of events, p = 0.002). Families who are non-English speaking are also less likely to activate the RR system (12% of total RR events, p = 0.048). LOS was longest for patients speaking languages other than Spanish or English and CD was more common in patients with government insurance. In adjusted logistic regression, Hispanic patients had 2.5 times the odds of mortality (95% CI: 1.43-4.53, p = 0.002) compared with non-Hispanic white patients., Conclusion: Disparities exist in access to and within the inpatient management of pediatric patients. Our results suggest that interventions to address disparities should focus on Hispanic patients and non-English speaking patients to improve inpatient health equity. More research is needed to understand and address the mortality outcomes in Hispanic children compared to other groups., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Lawson, Acorda, Guffey, Bracken, Bavare, Checchia and Afonso.)

Study Objectives: (1) To determine the sensitivity and specificity of the home sleep apnea test (HSAT) performed in typically developing children who were diagnosed with moderate to severe obstructive sleep apnea during overnight attended laboratory polysomnography (LPSG). (2) To determine the utility of a screening questionnaire to identify children at increased risk for obstructive sleep apnea., Methods: Participants completed 2 consecutive study nights, the first night with the HSAT followed by LPSG on the second night. The SHOOTS questionnaire, composed of 6 questions (snoring, hyperactivity, obesity, observed apnea, tonsillar hypertrophy, and sleepiness) concerning sleep-disordered breathing, was administered by the clinician before the first study night., Results: Thirty-eight participants completed both studies. The mean age was 13.8 ± 3.0 years. Twenty (53%) were male. Most participants were obese. The mean LPSG total sleep time was 7.34 ± 1.19 hours; the mean HSAT total recording time was 8.86 ± 1.73 hours ( P < .001). The median obstructive apnea-hypopnea index for LPSG and HSAT was 6.6 and 0.8 events/h, respectively. For an obstructive apnea-hypopnea index ≥ 3.1 events/h by HSAT, the sensitivity was 71.43% (95% confidence interval, 41.9-91.6) and the specificity was 95.83% (95% confidence interval, 78.9-99.9) for identifying those with an LPSG obstructive apnea-hypopnea index of ≥ 10 events/h. For a SHOOTS score with ≥ 4 "yes" responses, the sensitivity and specificity were 85.7% (95% confidence interval, 57.2-98.2) and 54.2% (95% confidence interval, 32.8-74.4), respectively, for identifying those with an LPSG obstructive apnea-hypopnea index ≥ 10 events/h., Conclusions: Using HSAT, we clinically applied cutoff values to identify moderate to severe obstructive sleep apnea in typically developing children. The SHOOTS questionnaire may aid in identifying children at risk for obstructive sleep apnea and who are candidates for HSAT., Citation: Revana A, Vecchio J, Guffey D, Minard CG, Glaze DG. Clinical application of home sleep apnea testing in children: a prospective pilot study. J Clin Sleep Med. 2022;18(2):533-540., (© 2022 American Academy of Sleep Medicine.)

Background: In 2018, approximately 1.6 million adolescents (aged 10-19) were living with HIV worldwide, with the highest HIV prevalence found in Eswatini. Adolescents and young adults living with HIV are a vulnerable population due to unique psychosocial challenges that come with having a stigmatizing disease. This group struggles more than other age-groups with medication adherence and requires novel approaches to supporting treatment, including peer-group encouragement, and self-expression., Methods: We piloted a theater camp for a group of adolescents and young adults enrolled at our HIV clinic in Mbabane, Eswatini, to determine the impact of having an outlet for creative expression and peer support on treatment and feelings of stigma. Pre- and post-camp surveys were administered to the participants to assess perceived stigma and impact of the camp. The results were analyzed using a Wilcoxon-signed rank test., Results: Twenty individuals (ages 12-23) living with HIV participated in the camp concurrently with standard treatment. 25% showed a substantial decrease in viral load within six months of completing the camp (> 0.1 log 10 change) while only 10% showed a substantial increase. Those who completed the survey felt the camp helped them with confidence, teamwork, and friendships. A comparison of pre- and post- surveys showed an overall decrease in personalized stigma. Quotes from participants reinforced these results., Conclusions: Adolescents and young adults living with HIV are an important population for further program development. Our study showed creative arts programming has beneficial psychosocial effects, aids in community building, and potentially enhances the effectiveness of medical treatment. Further programs and studies should continue to investigate creative arts as an avenue for self-expression and community building among vulnerable populations., (© 2021. The Author(s).)

Background: Previous studies suggest that birth before 39 weeks' gestational age (GA) is associated with higher perioperative mortality and morbidity after congenital heart surgery. The optimal approach to timing of cardiac operation in premature infants remains unclear. We investigated the impact of GA at birth and corrected GA at surgery on postoperative outcomes using the Pediatric Cardiac Critical Care Consortium (PC 4 ) database., Methods: Infants undergoing selected index cardiac operations before the end of the neonatal period were included (n = 2298). GA at birth and corrected GA at the time of the index cardiac operation were used as categorical predictors and fitted as a cubic spline to assess nonlinear relationships. The primary outcome was hospital mortality. Multivariable logistic regression models assessed the association between predictors and outcomes while adjusting for confounders., Results: Late-preterm (34-36 weeks) birth was associated with increased odds of mortality compared with full-term (39-40 weeks) birth, while early-term (37-38 weeks) birth was not associated with increased mortality. Corrected GA at surgery of 34 to 37 weeks compared with 40 to 44 weeks was associated with increased mortality. When analyzing corrected GA at surgery as a continuous predictor of outcome, odds of survival improve as patients approach 39 weeks corrected GA., Conclusions: Contrary to previous literature, we did not find an association between early-term birth and hospital mortality at PC 4 hospitals. Our analysis of the relationship between corrected GA and mortality suggests that operating closer to full-term corrected GA may improve survival., (Copyright © 2021 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Though commonly used for adjustment of risk, severity of illness and mortality risk prediction scores, based on the first 24 h of intensive care unit (ICU) admission, have not been validated in the pediatric extracorporeal membrane oxygenation (ECMO) population. We aimed to determine the association of Pediatric Index of Mortality 2 (PIM2), Pediatric Risk of Mortality Score III (PRISM III) and Pediatric Logistic Organ Dysfunction (PELOD) scores with mortality in pediatric patients on ECMO. This was a retrospective cohort study of children ≤18 years of age included in the Pediatric ECMO Outcomes Registry (PEDECOR) from 2014 to 2018. Logistic regression and Receiver Operating Characteristics (ROC) curves were used to calculate the area under the curve (AUC) to evaluate association of mortality with the scores. Of the 655 cases, 289 (44.1%) did not survive until hospital discharge. AUCs for PIM2, PRISM III, and PELOD predicting mortality were 0.52, 0.52, and 0.51 respectively. PIM2, PRISM III, and PELOD scores are not associated with odds of mortality for pediatric patients receiving ECMO. These scores for a general pediatric ICU population should not be used for prognostication or risk stratification of a select population such as ECMO patients., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Pinto, Guffey, Loftis, Bembea, Spinella and Hanson.)

Vaccine hesitancy remains a global health threat. Addressing parental vaccine hesitancy is essential to maintaining high vaccine coverage levels and preventing disease outbreaks; however, it is unknown if administering a vaccine hesitancy screening tool negatively impacts parental vaccine beliefs. We conducted a stratified randomized controlled trial in pediatric primary care practices. English-speaking parents ≥18 years of age seeking routine care for infants <3 months of age were eligible. Participants were randomized to receive 1 of 2 surveys - the Parent Attitudes about Childhood Vaccines (PACV) survey or a placebo survey. Six months after initial enrollment, all participants were asked to complete the PACV, regardless of initial randomization group. Our primary outcome was the proportion of hesitant to non-hesitant parents at 6-months between randomization groups. We examined associations between vaccine hesitancy and participant characteristics. We also evaluated the change in the proportion of vaccine-hesitant parents in the PACV group between baseline and 6-month follow up. We enrolled 1705 parents at baseline. At 6-month follow up, 819 parents completed the PACV (50.2% from PACV group vs. 54.1% from placebo group). The proportion of hesitant parents at 6 months did not differ between PACV and placebo groups (6.6% vs. 6.1%; p = .78) and the odds of hesitancy among PACV group participants was not higher than those in the placebo group (OR = 1.10; 95% CI: 0.63-1.93; p = .743). Race was the only characteristic significantly associated with vaccine hesitancy at 6-month follow up ( p = .003). Overall, administration of the PACV did not trigger vaccine hesitancy in this study population.

Competing Interests: The authors have no conflicts of interest to disclose.

The study examines the whole blood transcriptome profile before and after cardiopulmonary bypass (CPB) in neonates with hypoplastic left heart syndrome (HLHS), a severe form of congenital heart disease, that can develop low cardiac output syndrome (LCOS). Whole blood mRNA transcriptome profiles of 13 neonates with HLHS before and after their first palliative surgery were analyzed to determine differentially expressed genes and pathways. The median age and weight at surgery were 4 days and 3.2 kg, respectively. Of the 13 patients, 8 developed LCOS. There was no significant difference between CPB, aortic cross clamp, deep hypothermic cardiac arrest times between patients that develop LCOS and those that do not. Upon comparing differential gene expression profiles between patients that develop LCOS and those that do not in pre-operative samples, 1 gene was up-regulated and 13 were down regulated. In the post-operative samples, 4 genes were up-regulated, and 4 genes were down regulated when patients that develop LCOS were compared to those that do not. When comparing post-operative samples to pre-operative samples in the patients that do not develop LCOS, 1484 genes were up-regulated, and 1388 genes were down regulated; while patients that developed LCOS had 2423 up-regulated genes, and 2414 down regulated genes for the same pre to post-operative comparison. Pathway analysis revealed differential regulation of inflammatory pathways (IL signaling, PDGF, NOTCH1, NGF, GPCR) and metabolic pathways (heme metabolism, oxidative phosphorylation, protein metabolism including amino acid and derivatives, fatty acid metabolism, TCA cycle and respiratory electron transport chain). By identifying altered transcriptome profiles related to inflammation and metabolism in neonates with HLHS who develop LCOS after CPB, this study opens for exploration novel pathways and potential therapeutic targets to improve outcomes in this high-risk population.

In a human immunodeficiency virus (HIV) clinic for children and their families in Eswatini, we sought to understand the use of antibiotics and identify specific areas for improvement. We performed a retrospective patient chart review as part of a quality improvement (QI) initiative to assess antimicrobial use before and after implementation of a standardized antimicrobial guide. For each prescribing period, 100 random patient encounters were selected for review if the indication for antibiotics, duration, and dose were consistent with World Health Organization (WHO) guidelines. Two physicians reviewed each encounter using a structured abstraction tool, with a third resolving discrepancies. Results were analyzed using a chi-square test of proportions and a structured survey was performed to assess perceptions of the guide. After the implementation of an antimicrobial guide, there was a significant decrease in the proportion of clinic visits with an antibiotic prescribed (p < 0.001). Incorrect indication for antimicrobial use decreased from 20.4% in the initial period to 10.31% and 10.2% but did not reach significance (p = .0621) in the subsequent periods after implementation. Incorrect dose/duration decreased from 10.47% in the initial period to 7.37% and 3.1% in the subsequent periods, but this was also was not significant (p = 0.139). All prescribers who completed the survey felt that it positively impacted their prescribing. Our study found that an antimicrobial guide reduced and improved the prescription of antimicrobials, demonstrating practical solutions can have a lasting impact on prescribing in low resource settings., Competing Interests: The authors have declared that no competing interests exist.

Hyperferritinemia is associated with poor outcomes in critically ill patients with sepsis, hemophagocytic lymphohistiocytosis (HLH), macrophage activation syndromes (MAS) and coronavirus disease 19 (COVID-19). Autopsies of hyperferritinemic patients that succumbed to either sepsis, HLH, MAS or COVID-19 have revealed disseminated microvascular thromboses with von Willebrand factor (VWF)-, platelets-, and/or fibrin-rich microthrombi. It is unknown whether high plasma ferritin concentration actively promotes microvascular thrombosis, or merely serves as a prognostic biomarker in these patients. Here, we show that secretion of VWF from human umbilical vein endothelial cells (HUVEC) is significantly enhanced by 100,000 ng/ml of recombinant ferritin heavy chain protein (FHC). Ferritin fraction that was isolated by size exclusion chromatography from the plasma of critically ill HLH patients promoted VWF secretion from HUVEC, compared to similar fraction from non-critically ill control plasma. Furthermore, recombinant FHC moderately suppressed the activity of VWF cleaving metalloprotease ADAMTS-13. These observations suggest that a state of marked hyperferritinemia could promote thrombosis and organ injury by inducing endothelial VWF secretion and reducing the ADAMTS-13 activity.

The transition from internship to residency is a critical period for trainees. This study investigated factors that influence the transition of residents from postgraduate year 1 (PGY-1) to dermatology residency. In June 2019, a program director-specific survey and a resident-oriented survey were administered via Survey Monkey. An email containing survey hyperlinks was sent via the Association of Professors of Dermatology Listserv to program directors, who were asked to forward the email to their current trainees. A total of 44 dermatology residents and 33 program directors responded. More than 58% of program directors identified a preliminary year in internal medicine as most beneficial. Both residents and program directors indicated rheumatology, infectious disease, and allergy and immunology as the most relevant PGY-1 electives. Eighty-two percent of program directors expected <1 h/week or no independent preparation for dermatology residency during PGY-1. The stress of incoming residents as perceived by program directors was significantly less than the self-reported stress of residents during their transition to dermatology residency (Mann-Whitney U, two-sided, P = 0.011)., (Copyright © 2020 Baylor University Medical Center.)

Alagille syndrome (ALGS) and progressive familial intrahepatic cholestasis (PFIC) are inherited cholestatic disorders with risk of developing end-stage liver disease requiring liver transplantation (LT). We investigated aspartate aminotransferase-to-platelet ratio index (APRI), Fibrosis-4 score (FIB-4), and conjugated bilirubin as biomarkers to assess fibrosis severity and risk for LT among children with ALGS and PFIC. This multicenter, cross-sectional study included 64 children with ALGS or PFIC (per genetics or strict clinical criteria) with APRI, FIB-4, and conjugated bilirubin levels collected within ±90 days of their most recent liver biopsy. A single, blinded pathologist staged all biopsies (metavir; F0-F2: nonsevere, F3-F4: severe). Logistic regression and area under the receiver operating characteristic curve analysis (AUC) were used to assess biomarker associations with fibrosis severity and risk for LT. In ALGS, only APRI distinguished F3-F4 (AUC 0.72, P  = 0.012), with a cutoff greater than 2.97 demonstrating a sensitivity of 61.5% (95% confidence interval 0.32, 0.86) and specificity of 81.5% (0.62, 0.94). In ALGS, a 50% increase of APRI increased the odds of F3-F4 by 1.31-fold (1.04, 1.65; P  = 0.023). In ALGS, APRI (AUC 0.87; P  < 0.001) and FIB-4 (AUC 0.84; P  < 0.001) were able to predict risk for LT. In PFIC, only APRI distinguished F3-4 (AUC 0.74, P  = 0.039), with a cutoff greater than 0.99 demonstrating a sensitivity of 80% (0.44, 0.98) and specificity of 64.3% (0.35, 0.87). In PFIC, only FIB-4 was able predict risk for LT (AUC 0.80; P  = 0.002). In ALGS or PFIC, conjugated bilirubin could not distinguish F3-F4 or predict risk for LT. Conclusion: This liver biopsy-validated study suggests that APRI is able to distinguish F3-F4 from F0-F2 in ALGS and PFIC. APRI and FIB-4 may also serve as predictors of risk for LT in ALGS (APRI and FIB-4) and PFIC (FIB-4)., (© 2020 The Authors. Hepatology Communications published by Wiley Periodicals LLC on behalf of American Association for the Study of Liver Diseases.)

BACKGROUNDLiver disease in urea cycle disorders (UCDs) ranges from hepatomegaly and chronic hepatocellular injury to cirrhosis and end-stage liver disease. However, the prevalence and underlying mechanisms are unclear.METHODSWe estimated the prevalence of chronic hepatocellular injury in UCDs using data from a multicenter, longitudinal, natural history study. We also used ultrasound with shear wave elastography and FibroTest to evaluate liver stiffness and markers of fibrosis in individuals with argininosuccinate lyase deficiency (ASLD), a disorder with high prevalence of elevated serum alanine aminotransferase (ALT). To understand the human observations, we evaluated the hepatic phenotype of the AslNeo/Neo mouse model of ASLD.RESULTSWe demonstrate a high prevalence of elevated ALT in ASLD (37%). Hyperammonemia and use of nitrogen-scavenging agents, 2 markers of disease severity, were significantly (P < 0.001 and P = 0.001, respectively) associated with elevated ALT in ASLD. In addition, ultrasound with shear wave elastography and FibroTest revealed increased echogenicity and liver stiffness, even in individuals with ASLD and normal aminotransferases. The AslNeo/Neo mice mimic the human disorder with hepatomegaly, elevated aminotransferases, and excessive hepatic glycogen noted before death (3-5 weeks of age). This excessive hepatic glycogen is associated with impaired hepatic glycogenolysis and decreased glycogen phosphorylase and is rescued with helper-dependent adenovirus expressing Asl using a liver-specific (ApoE) promoter.CONCLUSIONOur results link urea cycle dysfunction and impaired hepatic glucose metabolism and identify a mouse model of liver disease in the setting of urea cycle dysfunction.TRIAL REGISTRATIONThis study has been registered at ClinicalTrials.gov (NCT03721367, NCT00237315).FUNDINGFunding was provided by NIH, Burroughs Wellcome Fund, NUCDF, Genzyme/ACMG Foundation, and CPRIT.

Background: Youth onset type 1 diabetes (T1D) and type 2 diabetes (T2D) is increasing and associated with earlier vascular complications and mortality. Dyslipidemia is an important modifiable cardiovascular (CVD) risk factor that is under-recognized and undertreated in youth with T1D and T2D. Given this, we evaluated the prevalence and associations between lipid concentrations and clinical CVD risk factors in youth with T1D compared to T2D at our large ethnically diverse diabetes center., Methods: A retrospective chart review was performed, evaluating patients with T1D or T2D seen at least once in clinic from 2015 to 2017, age 10-22 years of age, duration of diabetes at least 6 months on the date of most recent LDL-cholesterol (LDL-C) concentration, and not on statin therapy. We performed independent and multivariable linear regressions of LDL-C and HDL-cholesterol (HDL-C) concentrations., Results: There were 32.7% with T1D ( n  = 1701) and 47.7% with T2D ( n  = 298) with LDL-C above recommend goal (> 100 mg/dL/2.6 mmol/L). Furthermore, there were 9% with T1D and 16.4% with T2D with LDL > 130 mg/dL (> 3.4 mmol/L), who likely met criteria for starting statin therapy. Higher LDL-C and/or lower HDL-C were associated with increased age, diabetes duration, higher HbA1C, female sex, Hispanic ethnicity, obesity, and T2D. After adjusting for these risk factors in a multivariable linear regression model, the association of higher LDL-C and lower HDL-C was higher with T2D than T1D., Conclusions: This highlights the need for more aggressive dyslipidemia screening and treatment in youth with diabetes, especially T2D. At our institution we have created and instituted quality improvement algorithms to try to address this need., Competing Interests: Competing interestsThe authors declare that they have no financial and non-financial competing interests., (© The Author(s) 2020.)

Objective: To investigate the impact of feeding mode on neurodevelopmental outcomes in children with congenital heart defects., Design: A retrospective cohort study of 208 children with congenital heart disease (CHD), who had surgery from 1 January 2013 until 31 December 2016 at Texas Children's Hospital, Houston, TX, US., Settings: University Hospital, Developmental Outcome Clinic., Outcomes Measures: Standardized cognitive scores were assessed with Capute Scales and motor development with Revised Gesell Developmental Schedules. We analyzed anthropometrics, mode of feeding, surgical complexity, syndrome, and gender as predictors of developmental outcomes at four time points: hospital discharge, and 6, 12, and 24 months of age., Results: Mode of feeding is associated with neurodevelopmental outcome in children with CHD. Children on enteral feeding tubes had significantly lower developmental quotient (DQ) scores in cognition, communication, and motor function at 12 and 24 months compared to orally fed children. There were greater proportions of developmental delays (DQ < 70) in enteral tube fed children at the 6, 12, and 24 months visits. Further, there was a strong association between presence of enteral feeding tube, syndrome, and developmental outcome. Greater surgical complexity, weight gain and ethnicity were not associated with the developmental outcomes., Conclusions: Our findings suggest that the presence of an enteral feeding tube following corrective congenital heart surgery are at increased risk of neurodevelopmental delays at 12 and 24 months., (© 2019 Wiley Periodicals, Inc.)

Competing Interests: Competing interests: The authors have no conflicts of interest to disclose.

Background and Aims: Epidural analgesia is believed to be the most difficult technique to learn for a trainee. The reason for this is not only inexperience of the provider and the complexity of the technique but also patient factors like obesity, spinal deformity and others which makes the epidural placement difficult. The aim of this study was to evaluate some of the common risk factors for difficult epidural placement as perceived by the anesthesia providers during training, with varying level of experience., Material and Methods: This prospective observational study includes patients who received epidural placement for labor analgesia. Data recorded on these patients included age, height, weight, body mass index (BMI), ease of palpation of the spinous process, level of epidural placement, number of attempts, time taken for epidural placement and experience of the provider. The association between the variables were assessed using logistic regression for first attempt success and Cox proportional hazard ratio for time to epidural placement., Results: A total of 373 patients received epidural placement for labor analgesia. The mean BMI at the time of placement was 34. The first attempt success rate for the placement of epidural was 67% ( n = 273). Women with well palpable spinous process were 3.3 times more likely to have a successful first attempt placement irrespective of the provider experience or BMI [3.39 (1.77-6.51), P < 0.001]. The time to placement was shorter in patients with good anatomical landmarks [1.58 (1.20-2.07), P < 0.001) and when performed by a trainee who had performed a minimum of 20 epidural procedures [1.57 (1.26-1.94), P < 0.001)., Conclusion: Inability to palpate the spinous process contributes to multiple attempts at epidural placement when performed by a trainee., Competing Interests: There are no conflicts of interest., (Copyright: © 2019 Journal of Anaesthesiology Clinical Pharmacology.)

Objective: Our study retrospectively evaluated the implementation of an influenza vaccine best practice alert (BPA) in an electronic medical record within an integrated pediatric health care delivery system., Methods: An influenza BPA was implemented throughout a large pediatric health care delivery system in Houston, TX, to improve vaccine uptake. Outcomes were measured retrospectively over 3 years of BPA implementation and compared with a control year prior to BPA implementation. Primary outcomes were influenza vaccine uptake, distribution of influenza vaccines ordered by week, proportion of BPA displays ignored, and missed vaccination opportunities., Results: Influenza vaccine uptake declined from the pre-BPA year (47.2%; 95% confidence interval [CI]: 47.0, 47.4) to the last study year (45.1%; 95% CI: 44.9, 45.2). BPA displays were increasingly ignored by clinical staff throughout the study years from 59.6% in 2014-2015 to 72.5% in 2016-2017. For providers, BPA displays were ignored less frequently each year from 53.4% in 2014-2015 to 51.4% in 2017-2017. Within the primary care outpatient group, the proportion of missed vaccination opportunities in sick visits decreased from 86.8% during the pre-BPA year to 81.0, 79.8, and 82.7% during the subsequent study years 2014-2015, 2015-2016, and 2016-2017, respectively., Conclusion: Implementation of a widespread influenza BPA in an integrated pediatric health care delivery system did not produce meaningful increases in influenza vaccine uptake. Differences between clinical staff and providers on BPA use warrant further investigation., Competing Interests: None declared., (Georg Thieme Verlag KG Stuttgart · New York.)

Objective: Neurodevelopmental impairment is common after surgery for congenital heart disease (CHD) in infancy. While neurodevelopmental follow-up of high-risk patients has increased, the referral patterns for ancillary services following initial evaluation have not been reported. The aim of this study is to describe the rates and patterns of referral at the initial visit to our outcomes clinic of patients who underwent surgery for CHD during infancy., Outcomes Measures: The Cardiac Developmental Outcomes Program clinic at Texas Children's Hospital provides routine longitudinal follow-up with developmental pediatricians and child psychologists for children who required surgery for CHD within the first 3 months of life. Demographic, diagnostic, and clinical data, including prior receipt of intervention and referral patterns at initial presentation, were abstracted from our database., Results: Between April 2013 and May 2017, 244 infants under 12 months of age presented for initial evaluation at a mean age of 7 ± 1.3 months. At presentation, 31% (76/244) were referred for either therapeutic intervention (early intervention or private therapies), ancillary medical services, or both. Referral rates for low-risk (STAT 1-3) and high-risk (STAT 4-5) infants were similar (28 vs. 33%, P = .48). Referrals were more common in: Hispanic white infants (P = .012), infants with non-cardiac congenital anomalies (P = .001), history of gastrostomy tube placement (P < .001), and infants with prior therapy (P = .043). Infants of non-English speaking parents were three times more likely to be referred (95% CI = 1.5, 6.4; P = .002)., Conclusions: At the time of presentation, nearly 1 in 3 infants required referral. Referral patterns did not vary by traditional risk stratification. Sociodemographic factors and co-morbid medical conditions increased the likelihood of referral. This supports the need for routine follow-up for all post-surgical infants regardless of level of surgical complexity. Further research into the completion of referrals and long-term referral patterns is needed., (© 2019 Wiley Periodicals, Inc.)

Background: In asymptomatic children, screening echocardiography has been used to attempt to diagnose rheumatic heart disease (RHD) at an early stage (latent RHD). World Heart Federation guidelines have standardized categorization of "definite," "borderline," or no RHD by echo findings. The progression of RHD diagnosed through echo screening is not known. In 2014, we screened 1450 schoolchildren in Malawi., Objective: Our objective was to evaluate 2-year RHD evolution among those diagnosed through screening., Methods: Two-year follow-up echocardiograms of those diagnosed with latent RHD were read by a primary, secondary, then third reader if there was disagreement. Progression or regression of both definite and borderline groups were tabulated. Penicillin adherence, age, gender, number in home, and household income were compared between those with definite RHD who regressed to borderline and those that stayed definite. We utilized the local system used to track HIV defaulters in order to bring participants back into care. Comparisons were made using Fisher's exact and Wilcoxon rank-sum tests., Results: Of the 39 with borderline RHD, 1 was lost to follow-up (2.6%), 1 progressed to definite (2.6%), 19 remained borderline (48.7%), 17 (43.6%) regressed to normal, and 1 was reclassified as mitral valve prolapse (2.6%). Of the 11 with definite RHD, 6 (54.5%) remained definite, 4 regressed to borderline (36.4%), and 1 regressed to normal (9.1%). Two of 11 with definite RHD had penicillin adherence above 80% for the 2-year follow-up period. There were no differences in adherence, gender, age, household income, or number in household between those with definite RHD that regressed to borderline and those who did not (P > .19)., Conclusions: Borderlines had a very low progression rate to definite RHD. A strength of our study was a high retention rate (98%). Longer follow-up is needed to determine expected disease evolution., (© 2019 Wiley Periodicals, Inc.)

Introduction: Almost 6 million children suffer from food allergies with roughly 2 affected per classroom. Deficiencies in knowledge and discrepancies in attitudes within school staff when addressing food allergies are associated with barriers to care. In this study, school teacher knowledge, beliefs, and attitudes were measured before and after a food allergy educational session., Methods: Three hundred seventy-five personnel of similar age, socioeconomic status, ethnicity, and educational level completed the Chicago Food Allergy Research survey before and after a 1-hour educational session in 6 private schools in Houston randomly assigned into an intervention (n = 4) and control group (n = 2). Responses were measured using frequencies and percentages. The group score differences and survey question comparisons were evaluated with a linear mixed-effects model., Results: Posttest, the intervention group had knowledge scores 19.58% points higher than control (95% confidence interval = 16.62-22.53; P  < .001) with no differences pretest. Odds of agreeing that injectable epinephrine is important was higher in the intervention schools posteducation. Within the intervention group, personnel were more likely to agree to injectable epinephrine use for children posteducation., Conclusion: A 1-hour educational session improved knowledge and attitudes in personnel in the intervention schools. Given the growing prevalence of food allergy, the emphasis on food allergy education is crucial to allow for familiarization of the condition, early recognition of anaphylaxis, and promotion of injectable epinephrine use.

Background: Pre-transplant nutrition is a key driver of outcomes following liver transplantation in children. Patients with biliary atresia (BA) may have difficulty achieving satisfactory weight gain with enteral nutrition alone, and parenteral nutrition (PN) may be indicated. While PN has been shown to improve anthropometric parameters of children with BA listed for liver transplantation, less is known about the risks, particularly infectious, associated with this therapy among this specific group of patients., Aim: To describe the incidence, microbiology, and risk factors of central line-associated bloodstream infection (CLABSI) among children with BA listed for liver transplantation., Methods: Retrospective review of children aged ≤ 2-years of age with BA who were listed for primary liver transplantation at Texas Children's Hospital from 2008 through 2015 ( n = 96). Patients with a central line for administration of PN ( n = 63) were identified and details of each CLABSI event were abstracted. We compared the group of patients who experienced CLABSI to the group who did not, to determine whether demographic, clinical, or laboratory factors correlated with development of CLABSI., Results: Nineteen of 63 patients (30%, 95%CI: 19, 43) experienced 29 episodes of CLABSI during 4800 line days (6.04 CLABSI per 1000 line days). CLABSI was predominantly associated with Gram-negative organisms (14/29 episodes, 48%) including Klebsiella spp., Enterobacter spp., and Escherichia coli . The sole polymicrobial infection grew Enterobacter cloacae and Klebsiella pneumoniae . Gram-positive organisms (all Staphylococcus spp.) and fungus (all Candida spp.) comprised 9/29 (31%) and 6/29 (21%) episodes, respectively. No demographic, clinical, or laboratory factors were significantly associated with an increased risk for the first CLABSI event in Cox proportional hazards regression analysis., Conclusion: There is substantial risk for CLABSI among children with BA listed for liver transplantation. No clinical, demographic, or laboratory factor we tested emerged as an independent predictor of CLABSI. While our data did not show an impact of CLABSI on the short-term clinical outcome, it would seem prudent to implement CLABSI reduction strategies in this population to the extent that each CLABSI event represents potentially preventable hospitalization, unnecessary healthcare dollar expenditures, and may exact an opportunity cost, in terms of missed allograft offers., Competing Interests: Conflict-of-interest statement: All authors declare no conflicts-of-interest related to this article.

Background: To evaluate changes in population characteristics and outcomes in a large single-center pediatric patient cohort treated with continuous renal replacement therapy (CRRT) over a 10 year course, coincident with multiple institutional practice changes in CRRT delivery., Methods: A retrospective cohort study with comparative analysis of all patients treated from 2004 to 2013 with CRRT in the neonatal, pediatric, and cardiovascular intensive care units within a free-standing pediatric tertiary care hospital., Results: Three hundred eleven total patients were identified, 38 of whom received concurrent treatment with extracorporeal membrane oxygenation. 273 patients received CRRT only and were compared in two study eras (2004-2008 n = 129; 2009-2013 n = 144). Across eras, mean patient age decreased (9.2 vs 7.7 years, p = 0.08), and the most common principal diagnosis changed from cardiac to liver disease. There was an increase in patients treated with continuous renal replacement therapy between cohorts for acute kidney injury of multi factorial etiology (44% vs 56%) and a decrease in treated patients with sepsis (21% vs 11%, p = 0.04). There was no significant difference in survival to hospital discharge between eras (47% vs 49%). Improvement in outpatient follow-up after discharge amongst survivors was seen between study eras (33% vs 54%)., Conclusions: Despite multiple institutional practice changes in provision of CRRT, few changes were seen regarding patient demographics, diseases treated, indications for therapy, and survival over 10 years at a single tertiary care. Recognition of need for follow-up nephrology care following CRRT is improving. Ongoing assessment of the patient population in a changing landscape of care for critically ill pediatric patients remains important.

Background: Disclosure of medical errors is important to patients and physicians, but formal disclosure training during the graduate medical education curriculum is limited., Objective: We examined resident competence related to error disclosure, using standardized patient (SP) ratings of resident communication skills., Methods: All first-year residents from medicine, radiology, emergency medicine, orthopedic surgery, and neurological surgery completed a 20-minute simulated session in which they were provided background information on a medical error they had made and were asked to disclose the error to an SP acting as a family member. Residents were then debriefed and completed a postscenario questionnaire. The SPs completed an 11-item communication assessment and 3 milestone rating tools on professionalism (PROF-1, PROF-3) and interpersonal and communication skills (ICS-1)., Results: Ninety-six residents from a single institution participated toward the end of the intern year. Communication assessment scores ranged from 23% to 100% (mean [SD], 80.6 [17.0]). Mean (SD) milestone ratings across specialties were 2.80 ± 0.92 for PROF-1, 2.48 ± 0.92 for PROF-3, and 2.45 ± 0.92 for ICS-1. One-way analysis of variance revealed no significant differences among specialties on milestone or communication ratings. Residents who accepted personal responsibility for the error (84.55 [14.06]) received significantly higher communication ratings from SPs compared with residents who did not (66.67 [19.52], P < .001)., Conclusions: This SP assessment of error disclosure by first-year residents from multiple specialties was feasible and acceptable. It revealed areas of improvement as well as considerable variation in communication skills and professionalism among residents., Competing Interests: Conflict of interest: The authors declare they have no competing interests.

Objective: This study aims to compare the sensitivity of dual phase (non-contrast and arterial) versus single phase (arterial) CT for detection of hyper-functioning parathyroid glands in patients with primary hyperparathyroidism., Methods: The CT scans of thirty-two patients who have biochemical evidence of primary hyperparathyroidism, pathologically proven parathyroid adenomas, and pre-operative multiphase parathyroid imaging were evaluated retrospectively in order to compare the adequacy of single phase vs. dual phase CT scans for the detection of parathyroid adenomas., Results: The parathyroid adenomas were localized in 83% of cases on single arterial phase CT and 80% of cases on dual phase CT. The specificity for localization of parathyroid tumor was 96% for single phase CT and 97% for dual phase CT. The results were not significantly different ( p  = 0.695). These results are similar to those found in the literature for multiphase CT of 55-94%., Conclusions: Our study supports the use of a single arterial phase CT for the detection of hyperfunctioning parathyroid adenomas. Advances in knowledge: a single arterial phase CT has similar sensitivity for localizing parathyroid adenomas as dual phase CT and significantly reduces radiation dose to the patient., Competing Interests: The authors declare there are no competing interests.

Rheumatic heart disease (RHD) is the largest cardiac cause of morbidity and mortality in the world's youth. Early detection of RHD through echocardiographic screening in asymptomatic children may identify an early stage of disease, when secondary prophylaxis has the greatest chance of stopping disease progression. Latent RHD signifies echocardiographic evidence of RHD with no known history of acute rheumatic fever and no clinical symptoms., Objective: Determine the prevalence of latent RHD among children ages 5-16 in Lilongwe, Malawi., Design: This is a cross-sectional study in which children ages 5 through 16 were screened for RHD using echocardiography., Setting: Screening was conducted in 3 schools and surrounding communities in the Lilongwe district of Malawi between February and April 2014., Outcome Measures: Children were diagnosed as having no, borderline, or definite RHD as defined by World Heart Federation criteria. The primary reader completed offline reads of all studies. A second reader reviewed all of the studies diagnosed as RHD, plus a selection of normal studies. A third reader served as tiebreaker for discordant diagnoses. The distribution of results was compared between gender, location, and age categories using Fisher's exact test., Results: The prevalence of latent RHD was 3.4% (95% CI = 2.45, 4.31), with 0.7% definite RHD and 2.7% borderline RHD. There was no significant differences in prevalence between gender (P = .44), site (P = .6), urban vs. peri-urban (P = .75), or age (P = .79). Of those with definite RHD, all were diagnosed because of pathologic mitral regurgitation (MR) and 2 morphologic features of the mitral valve. Of those with borderline RHD, most met the criteria by having pathological MR (92.3%)., Conclusion: Malawi has a high rate of latent RHD, which is consistent with other results from sub-Saharan Africa. This study strongly supports the need for a RHD prevention and control program in Malawi., (© 2016 Wiley Periodicals, Inc.)

Background: Chromosome 15q13.3 represents a hotspot for genomic rearrangements due to repetitive sequences mediating nonallelic homologous recombination. Deletions of 15q13.3 have been identified in the context of multiple neurological and psychiatric disorders, but a prospective clinical and behavioral assessment of affected individuals has not yet been reported., Methods: Eighteen subjects with 15q13.3 microdeletion underwent a series of behavioral assessments, along with clinical history and physical examination, to comprehensively define their behavioral phenotypes., Results: Cognitive deficits are the most prevalent feature in 15q13.3 deletion syndrome, with an average nonverbal IQ of 60 among the patients studied. Autism spectrum disorder was highly penetrant, with 31% of patients meeting clinical criteria and exceeding cutoff scores on both ADOS-2 and ADI-R. Affected individuals exhibited a complex pattern of behavioral abnormalities, most notably hyperactivity, attention problems, withdrawal, and externalizing symptoms, as well as impairments in functional communication, leadership, adaptive skills, and activities of daily living., Conclusions: The 15q13.3 deletion syndrome encompasses a heterogeneous behavioral phenotype that poses a major challenge to parents, caregivers, and treating providers. Further work to more clearly delineate genotype-phenotype relationships in 15q13.3 deletions will be important for anticipatory guidance and development of targeted therapies.Genet Med 18 11, 1111-1118.

Currently no broadly accepted and standardized objective ranking system for dermatology residency programs exist. A new ranking system was recently developed in an attempt to address this issue. However, it remains unclear if the algorithm employed is based on factors that have a tangible impact on the quality of training provided by dermatology residency programs [1]. Our authors surveyed all current dermatology residents in order to investigate the factors that they feel are most important in determining the strength of training provided by a residency program.

Background. Peritoneal dialysis (PD) is an underutilized dialysis modality in the United States, especially in urban areas with diverse patient populations. Technique retention is a major concern of dialysis providers and might influence their approach to patients ready to begin dialysis therapy. Methods. Records from January 2009 to March 2014 were abstracted for demographic information, technique duration, and the reasons for withdrawal. Results. The median technique survival of the 128 incident patients during the study window was 781 days (2.1 years). The principle reasons for PD withdrawal were repeated peritonitis (30%); catheter dysfunction (18%); ultrafiltration failure (16%); patient choice or lack of support (16%); or hernia, leak, or other surgical complications (6%); and a total of 6 patients died during this period. Of the patients who did not expire and were not transplanted, most transferred to in-center hemodialysis and 8% transitioned to home-hemodialysis. Conclusions. Our findings suggest measures to ensure proper catheter placement and limiting infectious complications should be primary areas of focus in order to promote technique retention. Lastly, more focused education about home-hemodialysis as an option may allow those on PD who are beginning to demonstrate signs of technique failure to stay on home therapy.

Background: Guidelines for cardiopulmonary resuscitation recommend a chest compression rate of at least 100 compressions per minute. Animal and human studies have reported that blood flow is greatest with chest compression rates near 120/min, but few have reported rates used during out-of-hospital (OOH) cardiopulmonary resuscitation or the relationship between rate and outcome. The purpose of this study was to describe chest compression rates used by emergency medical services providers to resuscitate patients with OOH cardiac arrest and to determine the relationship between chest compression rate and outcome., Methods and Results: Included were patients aged ≥ 20 years with OOH cardiac arrest treated by emergency medical services providers participating in the Resuscitation Outcomes Consortium. Data were abstracted from monitor-defibrillator recordings during cardiopulmonary resuscitation. Multiple logistic regression analysis assessed the association between chest compression rate and outcome. From December 2005 to May 2007, 3098 patients with OOH cardiac arrest were included in this study. Mean age was 67 ± 16 years, and 8.6% survived to hospital discharge. Mean compression rate was 112 ± 19/min. A curvilinear association between chest compression rate and return of spontaneous circulation was found in cubic spline models after multivariable adjustment (P=0.012). Return of spontaneous circulation rates peaked at a compression rate of ≈ 125/min and then declined. Chest compression rate was not significantly associated with survival to hospital discharge in multivariable categorical or cubic spline models., Conclusions: Chest compression rate was associated with return of spontaneous circulation but not with survival to hospital discharge in OOH cardiac arrest.