Clinical Correlates and Prevalence of Food Selectivity in Children with Autism Spectrum Disorder.

OBJECTIVE: To examine clinical correlates and prevalence of food selectivity (FS) - ie, self-restricted diet, reluctance to try new foods - in children with autism spectrum disorder (ASD) ascertained from a general outpatient autism clinic. STUDY DESIGN: A multidisciplinary team (pediatric nurse practitioner, psychologist and dietitian) assessed medical and psychosocial histories and dietary habits in 103 children with ASD (mean age = 5.8 ± 2.2 years; range 2-10). Parents rated child mealtime behavior on the Brief Autism Mealtime Behavior Inventory (BAMBI) and disruptive behavior on the Aberrant Behavior Checklist (ABC). Height and weight measurements were collected. Children were classified as FS or no FS based on parent reported intake and mealtime behavior. A 24-hour dietary recall was used to record intake percentages < 80%. Logistic regression and multivariable modeling were used to evaluate clinical correlates with FS. RESULTS: Of 103 children, 45.6% (n = 47) were classified as FS; 54.4% (n = 56) no FS. After adjusting for potential confounders, the odds of FS increased by 1.91 (95% CI: 1.38, 2.64, P < .001) for every half-SD increase in BAMBI total score and by 1.35 (95% CI: 1.05, 1.74, P = .020) for every half-SD increase in ABC Hyperactivity/Noncompliance. No group differences in anthropometrics or nutritional intake were identified. CONCLUSIONS: Food selectivity (FS) in children with ASD was strongly associated with greater severity of disruptive mealtime and hyperactivity/noncompliance behaviors. FS was not associated with anthropometrics or nutritional intake.

Microbiome and Growth in Infants with Congenital Heart Disease.

OBJECTIVES: To profile the gut microbiome in infants with congenital heart disease undergoing cardiac surgery compared with matched infants and to investigate the association with growth (weight, length, and head circumference). STUDY DESIGN: A prospective study in the Cardiac Intensive Care Unit at Children's Healthcare of Atlanta and newborn nursery within the Emory Healthcare system. Characteristics including weight, length, head circumference, and surgical variables were collected. Fecal samples were collected pre-surgery (T1), post-surgery (T2), and before discharge (T3), and once for controls. 16S rRNA V4 gene was sequenced from fecal samples and classified into taxonomy using Silva v138. RESULTS: There were 34 children with congenital heart disease (cases) and 34 controls. Cases had higher alpha-diversity, and beta-diversity showed significant dissimilarities compared with controls. Gut microbiome was associated with lower weight and smaller head circumference (z-score <2). Lower weight was associated with less Acinetobacter, Clostridioides, Parabacteroides, and Escherichia-Shigella. Smaller head circumference with more Veillonella, less Acinetobacter, and less Parabacteroides. CONCLUSIONS: Significant differences in gut microbiome diversity and abundance were observed between infants with congenital heart disease and control infants. Lower weight and smaller head circumference were associated with distinct gut microbiome patterns. Further study is needed to understand the longitudinal effect of microbial dysbiosis on growth in children with congenital heart disease.

Outcome measures in pediatric chronic inflammatory demyelinating polyradiculoneuropathy.

INTRODUCTION/AIMS: Objective outcome measures in children undergoing treatment for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) are lacking. The aim of the study was to record serial grip strength and motor nerve conduction studies to assess interval change. METHODS: This was a retrospective review of 16 children (8 females and 8 males; median age, 9.7 years; interquartile range, 6-13 years) with CIDP followed at a tertiary children's hospital from 2013 to 2021. Subjects were treated with intravenous immunoglobulin (IVIG). Right and left grip strength measurements were obtained at each clinic visit using a handheld dynamometer. Annual right median motor nerve conduction study data were recorded during the study period. RESULTS: Mean duration of follow-up was 2.9 years. Grip strength (right: 0.19 kg/month, p < 0.001; left 0.23 kg/month, p < 0.001) and median F-wave latencies (-0.23/month, p = 0.015) showed significant improvement over time. Akaike information criterion showed time + IVIG frequency <21 days as best fit for grip strength and distal compound muscle action potential amplitude. DISCUSSION: Our study results indicate serial grip strength measurements are a feasible and objective way to assess motor strength improvement in children with CIDP receiving immunotherapy.

Assessing the US treatment landscape for paediatric feeding disorder: A survey of multidisciplinary providers.

BACKGROUND: Paediatric feeding disorder (PFD) is a common childhood condition, estimated to impact one in 37 American children under the age of five. Such high prevalence occurs against a backdrop of limited understanding of the community treatment landscape in the United States. METHOD: To better understand the community treatment landscape for PFD in the United States and identify provider and treatment delivery characteristics, we collected primary data through a web-based survey targeting providers from all four PFD domains (i.e., medical, nutritional, feeding skill, and/or psychosocial) between January 2022 and March 2022. The 71-item cross sectional survey focussed on patient, provider and treatment characteristics. We distributed the survey using an electronic survey tool through Feeding Matters listserv followed by solicitation to discipline specific listservs and professional networks. The analytic approach involved descriptive statistics compared across settings and provider types, focussing on respondents within the United States. RESULTS: Eighty-three percent of respondents reported practicing in the United States. Most of the US sample (74.3%) involved providers from the feeding skill domain (speech-language pathologist - SLP, occupational therapist - OT) who reported delivering care through early intervention or outpatient settings using responsive and sensory based approaches. These approaches lack rigorous empirical evaluation. CONCLUSIONS: Survey results suggest a need to support community providers in engagement with research activity to promote a better understanding of treatment approaches and outcomes associated with a large cohort of providers delivering care (i.e. SLPs, OTs) to patients with PFD.

A comparison of rapid cycle deliberate practice and traditional reflective debriefing on interprofessional team performance.

BACKGROUND: In simulation-based education, debriefing is necessary to promote knowledge acquisition and skill application. Rapid Cycle Deliberate Practice (RCDP) and Traditional Reflective Debriefing (TRD) are based in learning theories of deliberate practice and reflective learning, respectively. In this study, we compared the effectiveness of TRD versus RCDP on acquisition of conceptual knowledge and teamwork skills among interdisciplinary learners in the pediatric emergency department. METHODS: One hundred sixty-four learners including emergency department attending physicians, fellows, nurses, medical technicians, paramedics, and respiratory therapists, participated in 28 in-situ simulation workshops over 2 months. Groups were quasi-randomized to receive RCDP or TRD debriefing. Learners completed a multiple-choice test to assess teamwork knowledge. The TEAM Assessment Tool assessed team performance before and after debriefing. Primary outcomes were teamwork knowledge and team performance. RESULTS: Average pre-intervention baseline knowledge assessment scores were high in both groups (TRD mean 90.5 (SD 12.7), RCDP mean 88.7 (SD 15.5). Post-test scores showed small improvements in both groups (TRD mean 93.2 (SD 12.2), RCDP mean 89.9 (SD 13.8), as indicated by effect sizes (ES = 0.21 and 0.09, for TRD and RCDP, respectively). Assessment of team performance demonstrated a significant improvement in mean scores from pre-assessment to post-assessment for all TEAM Assessment skills in both TRD and RCDP arms, based on p-values (all p < 0.01) and effect sizes (all ES > 0.8). While pre-post improvements in TEAM scores were generally higher in the RCDP group based on effect sizes, analysis did not indicate either debriefing approach as meaningfully improved over the other. CONCLUSIONS: Our study did not demonstrate that either TRD versus RCDP was meaningfully better in teamwork knowledge acquisition or improving skill application and performance. As such, we propose Reflective Deliberate Practice as a framework for future study to allow learners to reflect on learning and practice in action.

Regional Anesthesia as an Alternative to Procedural Sedation for Forearm Fracture Reductions in the Pediatric Emergency Department.

BACKGROUND: Pediatric forearm fractures are common injuries in the pediatric emergency department (PED). Pediatric procedural sedation (PPS) is often required for forearm fracture reductions and pain control for casting. Bier blocks and hematoma blocks are types of regional anesthesia (RA) procedures that can be performed as a potential alternative to PPS. OBJECTIVE: The objective of this study is to compare the safety of RA with that of PPS. We hypothesized that RA has a safety profile that is equal or superior to PPS as well as a shorter duration of treatment in the PED. METHODS: Pediatric emergency department encounters in patients presenting with a diagnosis of radius fracture, ulna fracture, distal "both-bone" fracture, Monteggia fracture, and/or Galeazzi fracture were included. Outcomes of interest included patient adverse events (AEs), sedation medications used, PED duration of treatment (arrival time to disposition time), sedation failures, and reduction failures. RESULTS: Propensity matching was performed resulting in 632 well-matched RA-PPS pairs. The PPS cohort had 13% of encounters with at least 1 AE compared with 0.2% in the RA cohort, P < 0.001. The most common AE in the PPS group was hypoxia (9.8%), and the only AE in the RA group was an intravenous infiltrate (0.16%). Within the matched cohorts, PPS required more medications than RA (100% vs 60%, P < 0.001). Ketamine alone was more commonly used in the PPS group than the RA group (86% vs 0.2%, P < 0.001). Propofol was used only in the PPS group. The average duration of treatment was 205 (SD, 81) minutes in the PPS group and 178 (SD, 75) minutes in the RA group ( P < 0.001). There were no reduction failures in either group. CONCLUSIONS: Bier blocks and hematoma blocks are an acceptable alternative to PPS for children requiring forearm reductions. The AE rate is low and the reduction success rate is high. Duration of treatment in the PED is shorter for patients receiving RA compared with PPS.

A Mixed-methods Examination of Culturally Responsive Adaptation to an Evidence-based Parent-mediated Intervention Implemented for Autistic Children.

Parent-mediated interventions (PMIs) are considered an evidence-based practice for fostering social communication skills in young autistic children and for promoting parent responsivity and empowerment, yet barriers to caregiver engagement are evident when PMIs are implemented within historically underserved community settings. Issues of caregiver engagement can reflect a lack of fit between PMIs and the needs of diverse families. We used a mixed methods approach to examine barriers to participating in an evidence-based PMI, Project ImPACT (Ingersoll & Dvortcsak, 2019), within an outpatient setting, as well as strategies that clinicians reported using to deliver and adapt Project ImPACT for minoritized families. Participants included 134 caregivers of a child 13 to 48 months with autism or other social communication differences and six clinicians delivering Project ImPACT. Findings suggest that caregivers experience barriers to participating in Project ImPACT and that these barriers are associated with caregivers' ability to complete the program. Although quantitative findings indicate that adaptation to Project ImPACT did not differ by caregiver and child background, qualitative findings highlighted that clinicians attempt to deliver Project ImPACT to respond to the needs of families from minoritized backgrounds by actively considering the family's culture, psychosocial experiences, goals, and specific barriers. Further, both qualitative and quantitative findings suggest that culturally responsive care and adaptations may support caregiver engagement, including rapport, trust, buy-in, and attendance. Approaches to center cultural alongside contextual/psychosocial considerations within family-centered care in the implementation of PMIs are also highlighted.

Impact of the Magnitude and Timing of Fluid Overload on Outcomes in Critically Ill Children: A Report From the Multicenter International Assessment of Worldwide Acute Kidney Injury, Renal Angina, and Epidemiology (AWARE) Study.

OBJECTIVES: With the recognition that fluid overload (FO) has a detrimental impact on critically ill children, the critical care nephrology community has focused on identifying clinically meaningful targets for intervention. The current study aims to evaluate the epidemiology and outcomes associated with FO in an international multicenter cohort of critically ill children. The current study also aims to evaluate the association of FO at predetermined clinically relevant thresholds and time points (FO ≥ 5% and FO ≥ 10% at the end of ICU days 1 and 2) with outcomes. DESIGN: Prospective cohort study. SETTING: Multicenter, international collaborative of 32 pediatric ICUs. PATIENTS: A total of 5,079 children and young adults admitted consecutively to pediatric ICUs as part of the Assessment of the Worldwide Acute Kidney Injury, Renal Angina and Epidemiology Study. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: The FO thresholds at the time points of interest occurred commonly in the cohort (FO ≥ 5%Day1 in 38.1% [ n = 1753], FO ≥ 10%Day1 in 11.7% [ n = 537], FO ≥ 5%Day2 in 53.3% [ n = 1,539], FO ≥ 10%Day2 in 25.1% [ n = 724]). On Day1, multivariable modeling demonstrated that FO ≥ 5% was associated with fewer ICU-free days, and FO ≥ 10% was associated with higher mortality and fewer ICU and ventilator-free days. On multivariable modeling, FO-peak, Day2 FO ≥ 5%, and Day2 FO ≥ 10% were associated with higher mortality and fewer ICU and ventilator-free days. CONCLUSIONS: This study found that mild-to-moderate FO as early as at the end of ICU Day1 is associated with adverse outcomes. The current study fills an important void in the literature by identifying critical combinations of FO timing and quantity associated with adverse outcomes (FO ≥ 5%Day1, FO ≥10%Day1, FO ≥ 5%Day2, and FO ≥ 10%Day2). Those novel findings will help guide the development of interventional strategies and trials targeting the treatment and prevention of clinically relevant FO.

Multimodal phenotyping and correlates of pain following hematopoietic cell transplant in children with sickle cell disease.

INTRODUCTION: There is limited understanding of pain, patient-reported outcomes (PROs) of health-related quality of life (HRQoL), psychological factors, and experimental pain sensitivity before and following hematopoietic cell transplant (HCT) in children with sickle cell disease (SCD). METHODS: Individuals aged 8 years and older, English speaking, and scheduled for a HCT were invited to participate in an observational study where they completed assessments of pain, PROs, psychological factors, and qualitative interviews before and around 3 months, 6 months, 1 year, and 2 years post-HCT. An optional substudy of experimental pain sensitivity before and around 6 month, 1 year, and 2 years post-HCT was also offered. RESULTS: Data from eight participants (median age 13.5 years, 25% female) with sickle cell anemia (SCA) or similarly severe genotype, and successful donor-derived erythropoiesis post-HCT are reported. We found that collection of pain, PROs, psychological factors, and qualitative data were feasible in the context of HCT. We found moderate to large differences in pain and some PROs between baseline to 1 year and baseline to 2 year post-HCT based on effect sizes, but only some differences were statistically significant. We found moderate to large differences in pressure pain threshold and moderate differences in cold pain threshold between baseline to 1 year and baseline to 2 year post-HCT based on effect sizes, but these differences were not statistically significant. Qualitative data indicated an improvement in pain and HRQoL post-HCT. CONCLUSION: This study provides a framework for the conduct of multimodal pain assessments before and after HCT, which is feasible but faced with unique barriers.

Outcomes of children with life-threatening status asthmaticus requiring isoflurane therapy and extracorporeal life support.

BACKGROUND: Severe, refractory asthma is a life-threatening emergency that may be treated with isoflurane and extracorporeal life support. The objective of this study was to describe the clinical response to isoflurane and outcomes after discharge of children who received isoflurane and/or extracorporeal life-support for near-fatal asthma. METHODS: This was a retrospective descriptive study using electronic medical record data from two pediatric intensive care units within a single healthcare system in Atlanta, GA. RESULTS: Forty-five children received isoflurane, and 14 children received extracorporeal life support, 9 without a trial of isoflurane. Hypercarbia and acidosis improved within four hours of starting isoflurane. Four children died during the index admission for asthma. Twenty-seven percent had a change in Functional Status Score of three or more points from baseline to PICU discharge. Patients had median percent predicted FEV1 and FEV1/FVC ratios pre- and post-bronchodilator values below normal pediatric values. CONCLUSION: Children who received isoflurane and/or ECLS had a high frequency of previous PICU admission and intubation. Improvement in ventilation and acidosis occurred within the first four hours of starting isoflurane. Children who required isoflurane or ECLS may develop long-lasting deficits in their functional status. Children with near-fatal asthma are a high-risk group and require improved follow-up in the year following PICU discharge.

Infant viewing of social scenes is under genetic control and is atypical in autism.

Long before infants reach, crawl or walk, they explore the world by looking: they look to learn and to engage, giving preferential attention to social stimuli, including faces, face-like stimuli and biological motion. This capacity-social visual engagement-shapes typical infant development from birth and is pathognomonically impaired in children affected by autism. Here we show that variation in viewing of social scenes, including levels of preferential attention and the timing, direction and targeting of individual eye movements, is strongly influenced by genetic factors, with effects directly traceable to the active seeking of social information. In a series of eye-tracking experiments conducted with 338 toddlers, including 166 epidemiologically ascertained twins (enrolled by representative sampling from the general population), 88 non-twins with autism and 84 singleton controls, we find high monozygotic twin-twin concordance (0.91) and relatively low dizygotic concordance (0.35). Moreover, the characteristics that are the most highly heritable, preferential attention to eye and mouth regions of the face, are also those that are differentially decreased in children with autism (χ2 = 64.03, P < 0.0001). These results implicate social visual engagement as a neurodevelopmental endophenotype not only for autism, but also for population-wide variation in social-information seeking. In addition, these results reveal a means of human biological niche construction, with phenotypic differences emerging from the interaction of individual genotypes with early life experience.

HIV in youth prevention in the emergency department initiative: A survey of pediatric emergency medicine providers.

BACKGROUND: The incidence of HIV among adolescents remains high, and adolescents are known to participate in sexual behaviors that increase their risk for HIV, such as unprotected sex and sex with multiple partners. HIV pre-exposure prophylaxis (PrEP) has been shown to be effective at preventing HIV when taken daily and is approved by the FDA for use in adolescents. Efforts to screen patients in adult emergency departments and connect them with PrEP services have been validated. We surveyed pediatric emergency medicine (PEM) providers to determine their knowledge of PrEP, prescribing practices, willingness to prescribe, and barriers to a screening protocol in the pediatric emergency department (PED). METHODS: We administered a survey to a multidisciplinary group of PEM providers to measure knowledge, use, willingness, and implementation barriers to PrEP as well as elements needed for a successful referral system. RESULTS: A total of 87 responses were included for analysis. While 79.1% of all providers had heard of PrEP, only 14.8% of prescribing providers had ever discussed PrEP with a patient, and none had ever prescribed PrEP. Overall, 76.3% of all providers were knowledgeable about PrEP based on answers to true/false questions, with prescribing providers significantly more likely to be knowledgeable compared to nurses (p = 0.005). Knowledgeable providers had higher willingness scores to refer for PrEP compared to providers who were not knowledgeable. Ninety-two percent of providers felt a PrEP referral process from the PED would be feasible. Creation of an eligibility algorithm and educational materials were the most common efforts providers preferred to make them more likely to refer for PrEP. The most notable barriers perceived by providers included patient noncompliance with therapy (20.9%), acceptance of PrEP discussion among patients and parents (19.8%), and cost of therapy (15.1%). CONCLUSION: PEM providers are knowledgeable about PrEP but have little experience with discussing or prescribing PrEP. Their willingness to refer for PrEP and anticipated feasibility of a PrEP referral system is encouraging. These results support the need for future educational efforts among PEM providers and creation of referral systems for PrEP services from the PED.

Do Weightbearing Restrictions Cause Excess Weight Gain in Children With LEGG-Calvé-Perthes Disease?

BACKGROUND: Restricted weight bearing is commonly prescribed in Legg-Calvé-Perthes Disease (LCPD), raising concerns of causing overweight or obesity. This study utilizes prospectively collected data to address the following questions: (1) does body mass index (BMI) Z-score increase over the course of LCPD follow-up; (2) is having a BMI category of normal, overweight, or obese at baseline associated with BMI Z-score changes over the course of follow-up; and (3) is the duration of weight bearing restrictions (no restrictions, <3, 3 to <6, 6 to 9, or >9 mo) associated with BMI Z-score changes. METHODS: Data of 130 children aged 5 to 12 years with unilateral early-stage LCPD were extracted from an international database. Nation-specific BMI Z-scores and percentile-based weight categories were determined, and the duration of follow-up and weight bearing restrictions were calculated. Longitudinal changes in BMI Z-scores were evaluated for the 3 study questions using mixed effects linear regression models with surgery as a covariate. Sensitivity analyses were used to determine the influence of socio-cultural background (USA vs. India) for each study question. RESULTS: During the 35.5±15.9 months of follow-up, no statistically significant increase in BMI Z-scores was observed across the entire cohort, or following stratification by baseline weight categories or the duration of the weight bearing restriction. Sensitivity analyses indicated that patients in the USA had no change in their BMI Z-score. When stratified by weight categories, the normal weight of US children had a small increase in their BMI Z-score (0.005 per mo, 95% confidence interval: 0.0002, 0.009), but this was not seen in other BMI categories. The cohort of Indian children had a small but significant decrease in their BMI Z-score (-0.005/mo, 95% CI: -0.009, -0.0002). After stratification by weight categories, a small decrease of the BMI Z-score was observed only in the Indian overweight children (-0.016 per mo, 95% CI: -0.027, -0.005) and no other BMI category. CONCLUSIONS: Weightbearing restrictions over the course of follow-up for our cohort of children with early-stage LCPD were not associated with clinically meaningful increases of BMI Z-scores. Weight gain is multi-factorial and probably not caused by weight bearing restrictions alone. LEVEL OF EVIDENCE: III Diagnostic Study.

Factors Associated With Anal High-Grade Intraepithelial Lesions and Carcinoma Among Young Men Who Have Sex With Men and Transgender Women With HIV in Atlanta.

OBJECTIVE: This study aimed to evaluate factors associated with anal high-grade intraepithelial lesions (HSIL) and anal carcinoma among young men who have sex with men (MSM) and transgender women (TW) with HIV in Atlanta, GA, to better inform screening guidelines and preventative measures. MATERIALS AND METHODS: Cross-sectional retrospective chart review was completed for cisgender MSM and TW with HIV aged 13-25 years at the Grady Ponce and Family Youth Clinic in Atlanta, GA, from 2009 to 2020. High-grade anal disease was defined as anal intraepithelial neoplasia (AIN) 2, 3, or anal carcinoma (AIN 2+). Associations between clinical and demographic factors with AIN 2+ were estimated using logistic regression. Adjusted odds ratios (aORs) and associated 90% CIs are reported. RESULTS: One hundred nine MSM and TW with HIV who underwent anoscopy were included. One hundred three participants received anal biopsies, and 62% had AIN 2+. Being incompletely or unvaccinated against human papillomavirus (HPV, 0-2 doses) relative to being fully vaccinated (3 doses; aOR = 5.85; 90% CI = 1.28-26.83; p = .06) and having ever received surgical treatment for anogenital HPV (aOR = 2.89; 90% CI = 1.10-7.65; p = .07) were associated with AIN 2+, controlling for age and CD4 T-cell count at time of biopsy. CONCLUSIONS: Our study found a high prevalence of anal HSIL among young MSM and TW with HIV. Those who had ever received surgical treatment for anogenital HPV and those who were incompletely or unvaccinated against HPV were more likely to have HSIL. Our data emphasize the urgent need to improve HPV vaccination efforts and to pursue larger surveillance studies of anal HSIL and carcinoma among young MSM and TW with HIV.

Comparing Eosinophilic Esophagitis in a Black and Non-Black Pediatric Cohort.

OBJECTIVES: To compare presenting symptoms, comorbidities, disease, and treatment characteristics of a black pediatric eosinophilic esophagitis (EoE) group to a non-black pediatric EoE group. METHODS: A retrospective chart review consisting of pediatric patients diagnosed with EoE between the years of 2010 and 2018 at a single urban pediatric hospital system comprising 143 black pediatric patients compared with 142 non-black pediatric patients with similar distribution of age and sex. RESULTS: Both groups were majority male, and the median age of diagnosis between the black and non-black group was 5.1 and 6.7 years old, respectively. Comorbidities more commonly seen in the black group included food allergies, atopic dermatitis, asthma, and allergic rhinitis. Black patients were more likely to present with failure to thrive (FTT)/poor growth, whereas non-black patients were more likely to present with abdominal pain. There was no statistically significant difference between the groups in achieving remission using current therapies. The black group had higher rates of nonadherence to medical therapies. CONCLUSIONS: This is the largest study to date comparing a black versus non-black pediatric EoE population. The black population had more atopic comorbidities and FTT at presentation and had significantly more issues with nonadherence. This new knowledge describing EoE in a minority population will hopefully improve awareness, diagnosis, and management of EoE in this population.

Education and Psychosocial Factors Predict Odds of Death After Transfer to Adult health Care in Pediatric Liver Transplant Patients.

OBJECTIVES: To analyze demographic, psychosocial, and clinical factors in pediatric liver transplant recipients for their association with death or loss to follow up in adulthood. We aimed to better understand known health disparities in transplant outcomes and identify potentially modifiable risk factors prior to transfer. METHODS: A retrospective cohort study of children who underwent liver transplantation at a large tertiary transplant center and were transferred to adult care between 2000 and 2015. RESULTS: During the study period, 101 qualifying patients were transferred. Ninety-three individuals followed with an adult provider, while 8 were lost to follow up. In total 23 of 93 patients died after transfer (24.7%). Several childhood factors were associated with adult death: Black race [odds ratio (OR) 6.59, P < 0.001]; psychiatric illness or substance use (OR 2.81, P = 0.04); failure to graduate high school before transfer (OR 9.59, P < 0.001); posttransplant tacrolimus medication-level variability index >2.5 (OR 5.36, P = 0.04); provider documentation of medication nonadherence (OR 4.72, P = 0.02); acute cellular rejection (OR 4.44, P = 0.03); the presence of diabetes mellitus (OR 5.71, P = 0.001), and chronic kidney disease (OR 2.82, P = 0.04). Failure to graduate HS was associated with loss to follow up ( P < 0.001). On multivariate analysis, Black race, substance use, diabetes, and failure to graduate HS retained association with adult death (each P < 0.05). CONCLUSIONS: Complex, intertwined patient characteristics are associated with increased odds of death in pediatric liver transplant recipients transferred to adult care. Early recognition of high-risk patients and intervention for modifiable factors, such as improved HS graduation and substance use prevention, may improve long-term outcomes.

Triage Travel Screening in the Pediatric ED: A Cross-Sectional Analysis to Evaluate Current Use and Effectiveness in Identifying Travel Related Illness.

OBJECTIVES: In 2014 the Center for Disease Control and Prevention recommended emergency departments (EDs) implement triage travel screening to identify persons at risk for Ebola Virus Disease (EVD). EVD remains rare in the United States, and in practice the triage travel screen serves as a de facto screen for all travel-related illnesses. This study seeks to determine the current use and effectiveness of the triage travel screen to detect travel-related illness in the pediatric ED. METHODS: This was a retrospective, cross-sectional study of visits across three pediatrics EDs in 2019 in Atlanta, GA. Prevalences of travel-related illnesses were compared between patients with positive and negative travel screens. Patient charts with diagnoses of travel-related illness were then reviewed. RESULTS: Out of 244,841 patient encounters during the study period, 13 patients with travel-related illness were identified. 5/13 cases of travel-related illness were not diagnosed at the initial ED visit. Of these 5 cases, 2 had correctly negative travel screens (as travel was not within the specified timeframe) and 3 had correctly positive travel screens, but none had a clinician-documented travel history in the ED clinical notes. Of the 8/13 cases that were diagnosed at the initial ED visit, 7/8 had a clinician-documented travel history in the ED note. CONCLUSIONS: This study highlights the limitations of the current pediatric ED triage travel screen to detect travel-related illness and reinforces the importance of a provider-taken travel history. Strategies to increase provider-administered travel history documentation and revisions to increase triage travel-screen efficacy should be considered.

Cumulative Application of Creatinine and Urine Output Staging Optimizes the Kidney Disease: Improving Global Outcomes Definition and Identifies Increased Mortality Risk in Hospitalized Patients With Acute Kidney Injury.

OBJECTIVES: Acute kidney injury is diagnosed according to creatinine and urine output criteria. Traditionally, both are applied, and a severity stage (1-3) is conferred based upon the more severe of the two; information from the other criteria is discarded. Physiologically, however, rising creatinine and oliguria represent two distinct types of renal dysfunction. We hypothesized that using the information from both criteria would more accurately characterize acute kidney injury severity and outcomes. DESIGN: Prospective cohort study. SETTING: Multicenter, international collaborative of ICUs. PATIENTS: Three thousand four hundred twenty-nine children and young adults admitted consecutively to ICUs as part of the Assessment of the Worldwide Acute Kidney Injury, Renal Angina and Epidemiology Study. MEASUREMENTS AND MAIN RESULTS: The Kidney Disease: Improving Global Outcomes creatinine and urine output acute kidney injury criteria were applied sequentially, and the two stages were summed, generating an Acute Kidney Injury (AKI) Score ranging from 1 to 6. The primary outcome was 28-day mortality; secondary outcomes were time until ICU discharge and nonrecovery from acute kidney injury. Models considered associations with AKI Score, assessing the relationship unadjusted and adjusted for covariates. Twenty-eight-day mortality and nonrecovery from acute kidney injury were modeled using logistic regression. For 28-day ICU discharge, competing risks analysis was performed. Although AKI Scores 1-3 had similar mortality to no Acute Kidney Injury, AKI Scores 4-6 were associated with increased mortality. Relative to No Acute Kidney Injury, AKI Scores 1-6 were less likely to be discharged from the ICU within 28 days. Relative to AKI Score 1, AKI Scores 2-6 were associated with higher risk of nonrecovery. Within the traditional Kidney Disease: Improving Global Outcomes Stage 3 acute kidney injury cohort, when compared with AKI Score 3, AKI Scores 4-6 had increased mortality, AKI Scores 5-6 had prolonged time to ICU discharge, and AKI Score 6 experienced higher nonrecovery rates. CONCLUSIONS: Cumulative application of the creatinine and urine output criteria characterizes renal excretory and fluid homeostatic dysfunction simultaneously. This Acute Kidney Injury score more comprehensively describes the outcome implications of severe acute kidney injury than traditional staging methods.

African American Pediatric Liver Transplant Recipients Have an Increased Risk of Death After Transferring to Adult Healthcare.

OBJECTIVE: To analyze the long-term outcomes in pediatric liver transplant recipients after they have transferred to an adult provider and assess for racial disparities in health outcomes. STUDY DESIGN: This is a single-center, retrospective review of pediatric patients who underwent liver transplantation between July 1990 and August 2015 at a tertiary healthcare system with a large transplant center. Patient mortality and retransplantation were assessed after transfer to adult care. RESULTS: There were 120 patients who were transferred, of whom 19 did not meet the inclusion criteria. Of the remaining 101 patients, 64 (63%) transferred care to a nearby affiliated tertiary adult facility, 29 (29%) were followed by other healthcare systems, and 8 (8%) were lost to follow-up. Of the patients followed at our affiliated adult center, 18 of the 64 (28%) died. Of those 18 deaths, 4 (22%) occurred within the first 2 years after transfer, and 10 (55%) within 5 years of transfer. Four patients were retransplanted by an adult provider, of whom 2 eventually received a third transplant. African Americans had higher rates of death after transfer than patients of other races (44% mortality vs 16%, representing 67% of all cases of death; P = .032), with nearly 50% mortality at 20 years from time of transplantation. CONCLUSIONS: Death is common in pediatric liver transplant recipients after transfer to adult care, with African Americans having disproportionately higher mortality. This period of transition of care is a vulnerable time, and measures must be taken to ensure the safe transfer of young adults with chronic health care needs.

Validation of a new instrument to measure disease-related distress among patients with haemophilia.

INTRODUCTION: In patients with haemophilia, general psychological distress as measured by the National Comprehensive Cancer Network (NCCN) distress thermometer has been associated with pain, disability and increased healthcare utilization. AIMS: To develop and validate a measure of haemophilia-related distress. METHODS: After qualitative interviews, the Hemophilia-Related Distress Questionnaire (HRDq) was developed. To validate the HRDq, adults (≥18 years) with haemophilia were enrolled, reported demographic and clinical information, and completed the HRDq and other questionnaires that measured similar constructs. Analysis included factor analysis and assessment of internal consistency using Cronbach's α, convergent validity using Pearson's correlation coefficient, and discriminant validity by comparing subgroups of patients. Test-retest reliability was assessed using an intraclass correlation coefficient (ICC). RESULTS: Among 130 enrolled participants, 126 (median age=32.7 years) completed the 24 item HRDq in a median time of 5.4 minutes with overall HRDq scores ranging from 2 to 83 (median score=31.5; higher scores indicating higher distress). Assessment of convergent validity demonstrated a strong correlation (ρ>.60) of the HRDq total score with the NCCN Distress Thermometer, Haem-A-QoL total Score, and PROMIS-29 Profile social role domain and a mild to moderate correlation with all other questionnaire domains (.3-.59, p < .05). Distress was higher among those who had less education, were not employed, and were disabled and was not significantly different among those with severe compared with non-severe disease. Assessment of test-retest reliability demonstrated an ICC value of .84 (95% CI .71-.91) for the total score. CONCLUSIONS: The HRDq demonstrates good internal consistency, construct and discriminant validity, and retest reliability with a low responder burden.