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Background Adequate enteral nutrition may be difficult to achieve early in neonates after cardiac surgery, but it is essential for growth, wound healing, and immune function.Objective To assess caloric intake in neonates receiving enteral nutrition after surgery.Methods A retrospective chart review was conducted of daily enteral caloric intake in the cardiac intensive care unit of a tertiary children's hospital. Data on the institution of enteral feeding and the discontinuation of parenteral nutrition were assessed for full-term neonates who had undergone cardiac surgery.Results Caloric intake was assessed in 100 patients, 52 with biventricular cardiac defects and 48 with a functional single ventricle. The median duration of stay in the cardiac intensive care unit was 13 days (range, 4-69), and patients received enteral feeding exclusively for a median of 5 days (range, 1-43). In total, 705 patient days were evaluated. The median caloric intake per day was 93 kcal/kg (range, 43-142). A goal of 100 kcal/kg was achieved for 48.4% of patient days and 120 kcal/kg for only 19.7% of patient days. Median weight change for the period of enteral feeding was -20 g (range, -775 to 1485 g).Conclusions Enteral feeding alone is often suboptimal after neonatal cardiac surgery. New strategies to improve caloric intake may enhance postoperative recovery. [ABSTRACT FROM AUTHOR]

Purpose: To explore brachial plexus birth injury (BPBI)-affected adults' health-related quality of life (HRQoL) experiences informed by the theoretical framework of the International Classification of Functioning, Disability, and Health., Methods: This study applied a qualitative collective case study design. Twelve participants who participated in a prior survey study were recruited using maximum variation sampling. One-on-one semi-structured interviews focused on gaining in-depth understanding of participants' experiences with HRQoL. Interviews were recorded verbatim and thematically analysed., Results: We identified two themes. First, the theme biopsychosocial dimensions of the experience comprised the ways BPBI affects physical and emotional health and how BPBI is intertwined with affected individuals' identities, activities/participation, and social environments. Second, the theme lifelong and variable experience encompassed how BPBI is a chronic condition that changes over time and varies among affected individuals. The cross-case analysis derived a conceptual model of BPBI HRQoL to describe the BPBI experience through the lifespan. In this model, multiple dimensions of BPBI HRQoL expand through the lifespan while BPBI-specific health resources' availability contract., Conclusions: By providing insight into the many ways that BPBI affects and is affected by an individual's functioning and personal, social, and healthcare environmental factors, these findings underscore lifelong individualized care for BPBI-affected persons is needed.

Objective: In 2004, we reported improved early survival for patients with functional single ventricle anatomy and total anomalous pulmonary venous connection. This study sought to discover if outcomes have been ameliorated in the contemporary era., Methods: This was a single-center review of patients with single ventricle anatomy and total anomalous pulmonary venous connection who were admitted from 1984 to 2021. The cohort was divided into similarly sized groups by date of admission: Era 1: 1984 to 1992, Era 2: 1993 to 2007, and Era 3: 2008 to 2021. Survival was compared, and Cox proportional hazards models were used to evaluate the likelihood of mortality., Results: We included 190 patients with single ventricle anatomy and total anomalous pulmonary venous connection. Unbalanced atrioventricular canal defect (70%) was the most common primary diagnosis. The most common type of total anomalous pulmonary venous connection was supracardiac (49%). Approximately one-third (32%) of patients had pulmonary venous obstruction. There were no significant differences in patient characteristics across eras. Early survival after initial palliative operation improved between Eras 1 and 2, and then remained stable in Era 3. Overall survival improved from Era 1 to Eras 2 and 3 (P < .001), but not between Era 2 and 3. Survival to 10 years by Eras 1 to 3 was 15%, 51%, and 54%, respectively. The anatomic features associated with worse survival were hypoplastic left heart syndrome diagnosis (hazard ratio, 1.60; 1.04-2.57) and pulmonary venous obstruction (hazard ratio, 1.80; 1.24-2.69)., Conclusions: Overall survival for patients with single ventricle anatomy and total anomalous pulmonary venous connection has plateaued since the early 2000s. Even in the most recent era, survival to age 10 years remains less than 60%. Risk factors for mortality include the diagnosis of hypoplastic left heart syndrome and pulmonary venous obstruction. Further studies should focus on identification of the pathophysiological factors underlying the increased mortality., Competing Interests: Conflict of Interest Statement The authors reported no conflicts of interest. The Journal policy requires editors and reviewers to disclose conflicts of interest and to decline handling or reviewing manuscripts for which they may have a conflict of interest. The editors and reviewers of this article have no conflicts of interest., (Copyright © 2023 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Purpose: To describe the scope and nature of health concerns, functional impairments, and quality of life issues among adults with brachial plexus birth injury (BPBI)., Methods: A mixed methods study was conducted by surveying two social media networks of adults with BPBI using a combination of closed-ended and open-ended questions regarding the role of BPBI on ones' health, function, and quality of life. Closed-ended responses were compared across ages and genders. Open-ended responses were qualitatively analyzed to expand upon the close-ended responses., Results: Surveys were completed by 183 respondents (83% female, age range 20-87 years). BPBI was reported to impact hand and arm use in 80% of participants (including affected and unaffected limbs and bimanual tasks), overall health in 60% (predominantly pain), activity participation in 79% (predominantly activities of daily living and leisure), life roles in 76% (predominantly occupation and parenting), and overall quality of life in 73% (predominantly self-esteem, relationships, and appearance). Significantly more females than males reported other medical conditions and an impact on hand and arm use and life roles. No other responses varied by age or gender., Conclusions: BPBI affects many facets of health related quality of life in adulthood with variability among affected individuals.

Objectives: The primary objectives were to examine utilization of the Hybrid versus the Norwood procedure for patients with hypoplastic left heart syndrome or variants and the impact on hospital mortality. The Hybrid procedure was 1st used at our institution in 2004., Methods: Review of all subjects undergoing the Norwood or Hybrid procedure between 1 January 1984 and 31 December 2022. The study period was divided into 8 eras: era 1, 1984-1988; era 2, 1989-1993; era 3, 1994-1998; era 4, 1999-2003; era 5, 2004-2008; era 6, 2009-2014; era 7, 2015-2018 and era 8, 2019-2022. The primary outcome was in-hospital mortality. Mortality rates were computed using standard binomial proportions with 95% confidence intervals. Rates across eras were compared using an ordered logistic regression model with and adjusted using the Tukey-Kramer post-hoc procedure for multiple comparisons. In the risk-modelling phase, logistic regression models were specified and tested., Results: The Norwood procedure was performed in 1899 subjects, and the Hybrid procedure in 82 subjects. Use of the Hybrid procedure increased in each subsequent era, reaching 30% of subjects in era 8. After adjustment for multiple risk factors, use of the Hybrid procedure was significantly and positively associated with hospital mortality., Conclusions: Despite the increasing use of the Hybrid procedure, overall mortality for the entire cohort has plateaued. After adjustment for risk factors, use of the Hybrid procedure was significantly and positively associated with mortality compared to the Norwood procedure., (© The Author(s) 2024. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Objective: Volatile organic compounds (VOCs) are used in the sterilization and manufacture of medical equipment. These compounds have high vapor pressures with low water solubility and are emitted as gases from solids or liquids. They can be mutagenic, neurotoxic, genotoxic, and/or carcinogenic. Safe limits of exposure are not known for neonates. This study examined determinants of exposure in newborns undergoing cardiac surgery., Methods: Twenty metabolites of 16 VOCs (eg, xylene, cyanide, acrolein, acrylonitrile, N, N-dimethylformamide, 1,3-butadiene, styrene, and benzene) were measured as metabolites in daily urine samples collected from 10 neonates undergoing cardiac operations (n = 150 samples). Metabolites were quantified using reversed-phase ultra-high performance liquid chromatography and electrospray ionization tandem mass spectrometry. Repeated measures analysis of covariance was performed for each metabolite to examine associations with use of medical devices., Results: At least 3 metabolites were detected in every sample. The median number of metabolites detected in each sample was 14 (range, 3-15). In a model controlling for other factors, the use of extracorporeal membrane oxygenation was associated with significantly (P ≤ .05) greater metabolite levels of acrolein, acrylonitrile, ethylene oxide, propylene oxide, styrene, and ethylbenzene. Patients breathing ambient air had greater levels of metabolites of acrolein, xylene, N,N-dimethylformamide, methyl isocyanate, cyanide, 1,3-butadiene (all P ≤ .05)., Conclusions: Exposure to volatile organic compounds is pervasive in newborns undergoing cardiac surgery. Sources of exposure likely include medical devices and inhalation from the air in the intensive care unit. The contribution of VOC exposure during cardiac surgery in newborns to adverse outcomes warrants further evaluation., (Copyright © 2024. Published by Elsevier Inc.)

The purpose of this study was to establish the technical merit, feasibility, and generalizability of a new measure of understanding of informed consent for use with clinical research participants. A total of 109 teens/young adults at a large, pediatric medical center completed the consenting process of a hypothetical biobanking study. Data were analyzed using a combination of classical and modern theory analytic methods to produce a final set of 19 items referred to as the uConsent scale. A requirement of the scale was that each item mapped directly onto one or more of the Basic Elements of Informed Consent from the 2018 Final Rule. Descriptive statistics were computed for each item as well as the scale as a whole. Partial credit (Rasch) logistic modeling was then used to generate difficulty/endorsability estimates for each item. The final, 19-item uConsent scale was derived using inferential methods to yield a set of items that ranged across difficulty levels (-3.02 to 3.10 logits) with a range of point-measure correlations (0.12 to 0.50), within-range item- and model-fit statistics, varying item types mapped to both Bloom's Taxonomy of Learning and required regulatory components of the 2018 Final Rule. Median coverage rate for the uConsent scale was 95% for the 25 randomly selected studies from ClinicalTrials.gov. The uConsent scale may be used as an effective measure of informed consent when measuring and documenting participant understanding in clinical research studies today., (© 2023 The Authors. Clinical and Translational Science published by Wiley Periodicals LLC on behalf of American Society for Clinical Pharmacology and Therapeutics.)

Background: Infants with hypoplastic left heart syndrome (HLHS) or a variant are at risk of ventricular dysfunction (VD) and atrioventricular valve regurgitation (AVVR) prior to superior cavopulmonary connection (SCPC). Although the impact of these complications in isolation has been described, their effect in combination on attrition is poorly defined., Methods: A retrospective observational study of patients with HLHS or variants undergoing a Norwood procedure between 2008 and 2020 at a single center was performed. VD and AVVR were defined as moderate or severe when seen on 2 sequential echocardiograms outside the perioperative period. Attrition was defined as death, listing for heart transplant, or unsuitability for SCPC or transplant. Descriptive statistics and regression models were used for analysis., Results: A total of 397 patients were included, of whom 75% had HLHS and 57% had received a Blalock-Thomas-Taussig shunt. Isolated VD occurred in 9% of patients, AVVR occurred in 13%, and both occurred in 6%. Attrition prior to SCPC occurred in 19% of the overall cohort, in 52% of patients with combined VD and AVVR (odds ratio [OR], 5.2; 95% confidence interval [CI], 2.3-12.0; P  < .01), 26% of those with VD (OR, 1.5; 95% CI, 0.7-3.3; P  = .32), 25% of those with AVVR (OR, 1.5; 95% CI, 0.7-2.9; P  = .27), and 15% in those with neither (OR, 0.3; 95% CI, 0.2-0.6; P  < .01). Other factors associated with attrition included prematurity, total bypass time at Norwood, and extracorporeal membrane oxygenation after Norwood, whereas later year of Norwood was protective ( P  < .01 for all)., Conclusions: The presence of combined VD and AVVR markedly increases the likelihood of attrition prior to SCPC, identifying a high-risk group., Competing Interests: The authors reported no conflicts of interest. The Journal policy requires editors and reviewers to disclose conflicts of interest and to decline handling or reviewing manuscripts for which they may have a conflict of interest. The editors and reviewers of this article have no conflicts of interest., (© 2023 The Author(s).)

The purpose of this article is to propose and provide a blueprint for a graduate-level curriculum in clinical data science, devoted to the measurement, acquisition, care, treatment, and inferencing of clinical research data. The curriculum presented here contains a series of five required core courses, five required research courses, and a list of potential electives. The coursework draws from but does not duplicate content from the foundational areas of biostatistics, clinical medicine, biomedical informatics, and regulatory affairs, and may be reproduced by any institution interested in and capable of offering such a program. This new curriculum in "clinical" data science will prepare students for work in academic, industry, and government research settings as well as offer a unifying knowledge base for the profession., (© 2023 The Author. Clinical and Translational Science published by Wiley Periodicals LLC on behalf of American Society for Clinical Pharmacology and Therapeutics.)

Background: The AAP recommends "shared" reading from early infancy for healthy development. However, many families are uncertain how to read most enjoyably and effectively with infants, especially from underserved backgrounds. Shared reading quality (interactivity) moderates benefits yet is challenging to measure. SHARE/STEP is a new model of shared reading quality at this age incorporating evidence-based behaviors., Objective: To test the SharePR parent-report measure of caregiver-infant reading quality., Methods: This study involved mother-infant dyads in two unrelated trials in an obstetric (0-2 months old) and pediatric (6-9 months old) clinic. SharePR is a 10-item measure based on the SHARE/STEP model. Analyses involved descriptive statistics, measures of psychometric integrity, and correlations with home literacy environment (HLE)., Results: There were 99 dyads in the younger (1.2 + 0.5 months) and 108 dyads in the older groups (6.6 + 1.1 months). A majority were of non-white race (73%, 96%) and low-socioeconomic status (56%, 44% in-poverty). SharePR administration time was under 2 min and scores were normally distributed at each age. Psychometric properties were strong in terms of internal consistency and reliability. Scores were positively correlated with HLE for the older group (p < 0.05)., Conclusions: SharePR may be an efficient tool to quantify shared reading quality with infants, warranting further investigation., Clinical Trials: Data for these analyses were collected via two unrelated trials led by the lead author (J.S.H.). For the younger cohort, this is registered on the ClinicalTrials.gov website, ID# NCT04031235. For the older cohort, this is registered on the ClinicalTrials.gov website, ID# 2017-6856., Impact: The AAP recommends caregiver-child ("shared") reading beginning in infancy, yet many families are uncertain how to do so. Verbal and social-emotional interactivity during shared reading ("quality") moderates benefits and is often low in families from disadvantaged backgrounds, yet is challenging to measure. SharePR is a 10-item parent-report measure of shared reading quality based on a novel conceptual model incorporating evidence-based behaviors (SHARE/STEP). SharePR exhibited promising psychometric properties in two separate samples of mothers of younger and older infants. SharePR is a potentially useful measure of shared reading quality at this formative age, for research and to frame early reading guidance., (© 2022. The Author(s), under exclusive licence to the International Pediatric Research Foundation, Inc.)

Objective: To describe protocol adaptations to the Fibromyalgia Integrative Training for Teens (FIT Teens) randomized controlled trial in response to the COVID-19 pandemic. The overarching aims of the FIT Teens multi-site 3-arm comparative effectiveness trial are to assess whether a specialized neuromuscular exercise training intervention combined with cognitive-behavioral therapy (CBT) is superior to CBT alone or graded aerobic exercise alone., Design/methods: The trial was originally designed as an in-person, group-based treatment with assessments at baseline, mid- and post-treatment, and four follow-up time points. The original study design and methodology was maintained with specific modifications to screening, consenting, assessments, and group-based treatments to be delivered in remote (telehealth) format in response to COVID-19 restrictions., Results: Study enrollment was paused in March 2020 for five months to revise operations manuals, pilot remote treatment sessions for accuracy and fidelity, complete programming of REDCap assent/consent and assessment materials, train study staff for new procedures and obtain regulatory approvals. The trial was relaunched and has been successfully implemented in remote format since July 2020. Trial metrics thus far demonstrate a consistent rate of enrollment, strong attendance at remote treatment sessions, high retention rates and high treatment fidelity after protocol adaptations were implemented., Conclusions: Preliminary findings indicate that FIT Teens protocol adaptations from in-person to remote are feasible and allowed for sustained enrollment, retention, and treatment fidelity comparable to the in-person format. Methodologic and statistical considerations resulting from the adaptations are discussed as well as implications for interpretation of results upon completion of the trial., (© 2022 Published by Elsevier Inc.)

Background: Children with congenital heart defects have an increased risk of neurodevelopmental disability. The impact of environmental chemical exposures during daily life on neurodevelopmental outcomes in toddlers with congenital heart defects is unknown., Methods: This prospective study investigated the impacts of early childhood exposure to mixtures of environmental chemicals on neurodevelopmental outcomes after cardiac surgery. Outcomes were assessed at 18 months of age using The Bayley Scales of Infant and Toddler Development-III. Urinary concentrations of exposure biomarkers of pesticides, phenols, parabens, and phthalates, and blood levels of lead, mercury, and nicotine were measured at the same time point. Bayesian profile regression and weighted quantile sum regression were utilized to assess associations between mixtures of biomarkers and neurodevelopmental scores., Results: One-hundred and forty infants were enrolled, and 110 (79%) returned at 18 months of age. Six biomarker exposure clusters were identified from the Bayesian profile regression analysis; and the pattern was driven by 15 of the 30 biomarkers, most notably 13 phthalate biomarkers. Children in the highest exposure cluster had significantly lower adjusted language scores by -9.41 points (95%CI: -17.2, -1.7) and adjusted motor scores by -4.9 points (-9.5, -0.4) compared to the lowest exposure. Weighted quantile sum regression modeling for the overall exposure-response relationship showed a significantly lower adjusted motor score (β = -2.8 points [2.5th and 97.5th percentile: -6.0, -0.6]). The weighted quantile sum regression index weights for several phthalates, one paraben, and one phenol suggest their relevance for poorer neurodevelopmental outcomes., Conclusions: Like other children, infants with congenital heart defects are exposed to complex mixtures of environmental chemicals in daily life. Higher exposure biomarker concentrations were associated with significantly worse performance for language and motor skills in this population., Competing Interests: The authors have declared that no competing interests exist., (Copyright: This is an open access article, free of all copyright, and may be freely reproduced, distributed, transmitted, modified, built upon, or otherwise used by anyone for any lawful purpose. The work is made available under the Creative Commons CC0 public domain dedication.)

The Social and Emotional Assets and Resilience Scale (SEARS) is a promising instrument for prediction of resilience in youth; however, there is limited data to support its use. The purpose of the current study was to examine the factor structure, measurement invariance, internal consistency, and validity of the SEARS-Adolescent Report in youth 8 to 20 years of age. Two hundred and twenty-five childhood cancer survivors ( M age = 15.9, SD = 4.2; 51.4% male; 74.5% White) and 122 student controls without history of significant health problems ( M age = 14.2, SD = 3.5; 54.1% female; 79.5% White) 8 to 20 years of age completed the SEARS-A. The SEARS-A was found to have an adequate factor structure and model fit (χ 2 = 1215.5, p < .001; root mean square error of approximation = .057; comparative fit index = .95; standardized root mean square residual = .06) and demonstrated invariance across domains of age, health status, gender, race, and socioeconomic status (Δ comparative fit index < -0.01). It also demonstrated excellent internal reliability, criterion validity, and current validity when compared with another well-established measure of psychological adjustment. As such, the SEARS-A has potential to be a useful, valid, and psychometrically sound tool for predicting social-emotional adjustment outcomes among at-risk youth 8 to 20 years of age.

Aim: Digital media use is prevalent among children and linked to potential developmental and health risks, but validated measures of children's digital media use are lacking. The aim of this study was to validate the Portuguese version of the ScreenQ with three distinct children's age groups., Methods: Parents of children living in Portugal completed an online survey including the 16-item version of the ScreenQ and items related to home activities and digital media use. A combination of classical and modern theory (Rasch) methods was used for analysis., Results: A total of 549 mothers and 51 fathers of 325 girls and 322 boys from 6 months to 9 years and 11 months old responded to the survey. Point-measure correlations were all positive and endorsement of item values were within acceptable ranges. Cronbach's coefficient α was acceptable for a new measure, and test-retest reliability was high. Statistically significant correlations were found between ScreenQ total scores and relevant demographic, play-related, parenting and digital media use items., Conclusion: The Portuguese version of the ScreenQ exhibited sound psychometric properties, including internal consistency and concurrent validity referenced to external items. Higher ScreenQ scores were correlated with higher digital media multitasking, lower parent-child interaction, and higher concerns regarding child's learning and behaviour., (© 2022 The Authors. Acta Paediatrica published by John Wiley & Sons Ltd on behalf of Foundation Acta Paediatrica.)

Background: Assessment of bone health in young children has been hampered by limited reference values for bone mineral content (BMC) and areal bone mineral density (aBMD) by dual energy X-ray absorptiometry (DXA)., Objectives: To identify age, sex, and population ancestry effects on BMC and aBMD and develop smoothed reference ranges for BMC and aBMD in young children. To quantify precision of bone measurements and influence of height-for-age Z-scores on bone Z-scores., Methods: We recruited 484 healthy children ages 1 to 2 years or 4.5 to 5 years at 2 clinical centers, who were seen once or up to 7 times over a 3-year period. Lumbar spine, distal forearm, and whole-body subtotal (ages ≥ 3 years) BMC and aBMD were measured by DXA. These data were combined with data from the Bone Mineral Density in Childhood Study from children ages 5 to 8.9 years to create the smoothed reference curves., Results: For 1- to 5-year-olds, BMC and aBMD at all skeletal sites increased with age. Age trends differed by sex for BMC and aBMD of the spine, distal one-third radius, ultradistal radius, and by ancestry (Black vs non-Black) for all measures. BMC and aBMD precision (% coefficient of variation) ranged from 1.0% to 4.4%. Height Z-scores were positively associated with bone Z-scores and accounted for 4% to 45% of the variance., Conclusions: We demonstrate the feasibility of bone density measurements in young children and provide robust reference ranges and stature adjustments for calculation of bone Z-scores at multiple skeletal sites to enable bone health assessments., (© The Author(s) 2022. Published by Oxford University Press on behalf of the Endocrine Society. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Objective: We conducted a pre-post pilot trial to determine the feasibility and acceptability of a multi-component intervention (pre-clinic letter, shared decision making cards and follow-up phone call) designed to facilitate SDM in pediatric inflammatory bowel disease (IBD)., Methods: We recruited physicians (n = 11) caring for IBD patients and families (n = 36) expected to discuss anti-tumor necrosis treatment. We measured feasibility and acceptability of the intervention, observed SDM, perceived SDM, decision conflict, and regret. Medical records were used to assess clinical outcomes, time to decision and adherence. We compared all outcomes between the usual care and intervention study arms., Results: Two out of three intervention components were feasible. Visit length increased significantly in the intervention arm. Parents and patients rated the intervention as acceptable, as did most physicians. The intervention was associated with a higher-level of observed SDM. There was no difference perceived SDM, decision conflict, regret or quality of life outcomes between arms. Physician global assessment improved over time in the intervention arm., Conclusions: This pilot trial provides important guidance for developing a larger scale trial of a modified intervention., Practice Implications: Overall, our intervention shows promise in supporting SDM and engaging both parents and patients in pediatric IBD decisions., (Copyright © 2021 Elsevier B.V. All rights reserved.)

Objectives: The objective of this study was to investigate changes in serum biomarkers of acute brain injury, including white matter and astrocyte injury during chronic foetal hypoxaemia. We have previously shown histopathological changes in myelination and neuronal density in fetuses with chronic foetal hypoxaemia at a level consistent with CHD., Methods: Mid-gestation foetal sheep (110 ± 3 days gestation) were cannulated and attached to a pumpless, low-resistance oxygenator circuit, and incubated in a sterile fluid environment mimicking the intrauterine environment. Fetuses were maintained with an oxygen delivery of 20-25 ml/kg/min (normoxemia) or 14-16 ml/kg/min (hypoxaemia). Myelin Basic Protein and Glial Fibrillary Acidic Protein serum levels in the two groups were assessed by ELISA at baseline and at 7, 14, and 21 days of support., Results: Based on overlapping 95% confidence intervals, there were no statistically significant differences in either Myelin Basic Protein or Glial Fibrillary Acidic Protein serum levels between the normoxemic and hypoxemic groups, at any time point. No statistically significant correlations were observed between oxygen delivery and levels of Myelin Basic Protein and Glial Fibrillary Acidic Protein., Conclusion: Chronic foetal hypoxaemia during mid-gestation is not associated with elevated serum levels of acute white matter (Myelin Basic Protein) or astrocyte injury (Glial Fibrillary Acidic Protein), in this model. In conjunction with our previously reported findings, our data support the hypothesis that the brain dysmaturity with impaired myelination found in fetuses with chronic hypoxaemia is caused by disruption of normal developmental pathways rather than by direct cellular injury.

Little is known about the number of minors enrolled in clinical research today. IRB administrators at leading pediatric medical centers were surveyed regarding studies with minors. Analyses were descriptive in nature with adaptive Bayesian bootstrap imputation used with missing data. Officials from 17/41 (41.5%) pediatric research centers responded: 74,204 active studies were estimated, 29,078 (39%) included minors, and 6574 (23%) were "more than minimal risk." Minors accounted for 0.7-2.87M research subjects. Pediatric medicine desperately needs a more accurate and reliable reporting system for tracking the recruitment, retention, and involvement of minors in clinical research., Competing Interests: The authors have no conflicts of interest to declare., (© The Author(s) 2021.)

Objective: To estimate feasibility, usability and efficacy of a mobile parenting app (Rx for Success; RxS) to enhance reading guidance provided to parents of young children during well-visits., Methods: This trial was conducted at a clinic serving primarily families of Hispanic ethnicity and low-socioeconomic status (SES) where Reach Out and Read (ROR) is standard practice. It involved 252 parent-child dyads in 2 age groups (~6-months old, ~18-months old) randomized during well-visits to receive RxS or a children's book modeling alternatives to screen time (Control) by research coordinators. RxS involves videos, activities and "push" messages. Follow-up assessments were conducted approximately 6 months later, including impression and use, shared reading behaviors, child language and screen time., Results: A total of 217 dyads completed both visits (110 RxS, 107 Control). Time to introduce RxS was under 3 minutes and 32% of parents experienced largely minor performance issues. Parent impression of RxS was favorable for both age groups at baseline and follow-up, though use was infrequent, attributable to a desire for more relevant and updated content. Significant findings favoring RxS included shared reading as a favorite activity, more frequent shared reading reported at 12 months and higher language scores at 24 months. Screen time was equivalent between cohorts, exceeding American Academy of Pediatrics guidelines., Conclusions: A mobile app introduced to parents of young children from low-SES backgrounds was feasible during well-visits, rated as helpful, and effective to enhance shared reading at younger and language at older ages. While a potentially impactful enhancement to ROR, features needing improvement were identified., (Copyright © 2021 Academic Pediatric Association. Published by Elsevier Inc. All rights reserved.)

Objective: We investigated the incidence and predictors of failure to undergo the Fontan in children with hypoplastic left heart syndrome who survived superior cavopulmonary connection., Methods: The cohort consists of all patients with hypoplastic left heart syndrome who survived to hospital discharge after superior cavopulmonary connection between 1988 and 2017. The primary outcome was attrition, which was defined as death, nonsuitability for the Fontan, or cardiac transplantation before the Fontan. Subjects were excluded if they were awaiting the Fontan, were lost to follow-up, or underwent biventricular repair. The study period was divided into 4 eras based on changes in operative or medical management. Attrition was estimated with 95% confidence intervals, and predictors were identified using adjusted, logistic regression models., Results: Of the 856 hospital survivors after superior cavopulmonary connection, 52 died, 7 were deemed unsuitable for Fontan, and 12 underwent or were awaiting heart transplant. Overall attrition was 8.3% (71/856). Attrition rate did not change significantly across eras. A best-fitting multiple logistic regression model was used, adjusting for superior cavopulmonary connection year and other influential covariates: right ventricle to pulmonary artery shunt at Norwood (P < .01), total support time at superior cavopulmonary connection (P < .01), atrioventricular valve reconstruction at superior cavopulmonary connection (P = .02), performance of other procedures at superior cavopulmonary connection (P = .01), and length of stay after superior cavopulmonary connection (P < .01)., Conclusions: In this study spanning more than 3 decades, 8.3% of children with hypoplastic left heart syndrome failed to undergo the Fontan after superior cavopulmonary connection. This attrition rate has not decreased over 30 years. Use of a right ventricle to pulmonary artery shunt at the Norwood procedure was associated with increased attrition., (Copyright © 2020. Published by Elsevier Inc.)

Background: The contribution of neonatal cyanosis, inherent to cyanotic congenital heart disease, to the magnitude of neurologic injury during deep hypothermic circulatory arrest has not been fully delineated. This study investigates the impact of cyanosis and deep hypothermic circulatory arrest on brain injury., Methods: Neonatal piglets were randomised to placement of a pulmonary artery to left atrium shunt to create cyanosis or sham thoracotomy. At day 7, animals were randomised to undergo deep hypothermic circulatory arrest or sham. Arterial oxygen tension and haematocrit were obtained. Neurobehavioural performance was serially assessed. The animals were sacrificed on day 14. Brain tissue was assessed for neuronal necrosis using a 5-point histopathologic score., Results: Four experimental groups were analysed (sham, n = 10; sham + deep hypothermic circulatory arrest, n = 8; shunt, n = 9; shunt + deep hypothermic circulatory arrest, n = 7). Cyanotic piglets had significantly higher haematocrit and lower partial pressure of oxygen at day 14 than non-cyanotic piglets. There were no statistically significant differences in neurobehavioural scores at day 1. However, shunt + deep hypothermic circulatory arrest piglets had evidence of greater neuronal injury than sham animals (median (range): 2 (0-4) versus 0 (0-0), p = 0.02)., Discussion: Cyanotic piglets undergoing deep hypothermic circulatory arrest had increased neuronal injury compared to sham animals. Significant injury was not seen for either cyanosis or deep hypothermic circulatory arrest alone relative to shams. These findings suggest an interaction between cyanosis and deep hypothermic circulatory arrest and may partially explain the suboptimal neurologic outcomes seen in children with cyanotic heart disease who undergo deep hypothermic circulatory arrest.

Background and Objectives: The American Academy of Pediatrics recommends literacy and school readiness promotion during well visits. The Reading House (TRH) is a children's book-based screener of emergent literacy skills in preschool-aged children. Vocabulary, rhyming, and rapid naming are core emergent skills, and reading abilities are associated with thicker cortex in the left hemisphere. Our objective was to expand validity of TRH relative to these skills and explore association with cortical thickness., Methods: Healthy preschool-aged children completed MRI including a T1-weighted anatomic scan. Before MRI, TRH and assessments of rapid naming (Comprehensive Test of Phonological Processing, Second Edition), rhyming (Pre-Reading Inventory of Phonological Awareness), vocabulary (Expressive Vocabulary Test, Second Edition), and emergent literacy ( Get Ready to Read! ) were administered. Analyses included Spearman-ρ correlations ( r ρ ) accounting for age, sex, and socioeconomic status (SES). MRI analyses involved whole-brain measures of cortical thickness relative to TRH scores, accounting for covariates., Results: Seventy children completed assessments (36-63 months old; 36 female) and 52 completed MRI (37-63 months; 29 female). TRH scores were positively correlated with Comprehensive Test of Phonological Processing, Second Edition ( r ρ = 0.61), Expressive Vocabulary Test, Second Edition ( r ρ = 0.54), Get Ready to Read! ( r ρ = 0.87), and Pre-Reading Inventory of Phonological Awareness scores ( r ρ = 0.64; all P < .001). These correlations remained statistically significant across age, sex, and SES groups. TRH scores were correlated with greater thickness in left-sided language and visual cortex ( P -family-wise error <.05), which were similar for higher SES yet more bilateral and frontal for low SES, reflecting a less mature pattern ( P -family-wise error <.10)., Conclusions: These findings expand validation evidence for TRH as a screening tool for preschool-aged children, including associations with emergent skills and cortical thickness, and suggest important differences related to SES., Competing Interests: POTENTIAL CONFLICT OF INTEREST: Dr Hutton conceived, wrote, and edited the children’s book featured in this study. Dr Hutton is the founder of blue manatee press, the publisher and distributor of The Reading House, although he receives no salary or other compensation for this role. The Reading House is not intended for sale in retail, library, or other direct-to-consumer channels. Its intended use is as a screening tool, distributed at low cost to clinical practices, preschools, and organizations. Royalties, if any, are determined via intellectual property policies at Cincinnati Children’s Research Foundation; the other authors have indicated they have no potential conflicts of interest to disclose., (Copyright © 2021 by the American Academy of Pediatrics.)

Background The superior cavo-pulmonary connection was introduced at our institution in 1988 for infants undergoing surgery for hypoplastic left heart syndrome. Patients with hypoplastic left heart syndrome remain at high risk for mortality in the time period between the Norwood procedure and the superior cavo-pulmonary connection. The primary objectives of this study were to compare interstage mortality across 4 eras and analyze factors that may impact interstage mortality. Methods and Results Patients with hypoplastic left heart syndrome who underwent the Norwood procedure, were discharged from the hospital, and were eligible for superior cavo-pulmonary connection between January 1, 1988, and December 31, 2017, were included. The study period was divided into 4 eras based on changes in operative or medical management. Mortality rates were estimated with 95% CIs. Adjusted and unadjusted logistic regression models were used to identify risk factors for mortality. There were 1111 patients who met the inclusion criteria. Overall, interstage mortality was 120/1111 (10.8%). Interstage mortality was significantly lower in era 4 relative to era 1 (4.6% versus 13.4%; P =0.02) during the time that age at the superior cavo-pulmonary connection was the lowest (135 days; P <0.01) and the interstage monitoring program was introduced. In addition, use of the right ventricle to pulmonary artery shunt was associated with decreased interstage mortality ( P =0.02) and was more routinely practiced in era 4. Conclusions During this 30-year experience, the risk of interstage mortality decreased significantly in the most recent era. Factors that coincide with this finding include younger age at superior cavo-pulmonary connection, introduction of an interstage monitoring program, and increased use of the right ventricle to pulmonary artery shunt.

Objectives: The objective of this study was to estimate hospital mortality and length of stay (LOS) for children with hypoplastic left heart syndrome undergoing superior cavopulmonary connection (SCPC)., Methods: All hypoplastic left heart syndrome interstage survivors who underwent SCPC between 1 January 1988 and 31 December 2017 were included. The study period was divided into 4 eras based on changes in operative or medical management. Mortality rates were estimated using standard binomial proportions. Adjusted and unadjusted logistic regression models were used to identify risk factors for mortality and LOS., Results: The most common procedures for the cohort (n = 958) were Hemi-Fontan (57.3%) or Bidrectional Glenn shunt (35.7%). The mortality was 4.1% overall and decreased in all 3 later eras compared to era 1. Factors associated with mortality in a multiple covariate model included longer total support time, earlier gestational age, longer LOS at the Norwood Procedure and need for additional procedures. Overall, the median LOS was 7.0 days with a decrease from eras 1 to 2 and plateaued in eras 3 and 4. Predictors of longer LOS included genetic anomaly, longer Norwood LOS, additional procedures, lower weight at surgery and longer total support time. The type of SCPC was not associated with mortality or LOS., Conclusions: In this large cohort of patients with hypoplastic left heart syndrome undergoing SCPC, hospital mortality has decreased significantly. LOS initially declined but plateaued in recent eras. The risk factors for mortality and longer LOS are related to patient and procedural complexity, especially the need for additional procedures at the time of SCPC., (© The Author(s) 2020. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Background: Screen-based media use is prevalent in children and is associated with health risks. American Academy of Pediatrics (AAP) recommendations involve access to screens, frequency, content, and co-viewing. The aim of this study was to test the ScreenQ, a composite measure of screen-based media use., Methods: ScreenQ is a 15-item parent report measure reflecting AAP recommendations. Range is 0-26, higher scores reflecting greater non-adherence. With no "gold standard" available, four validated measures of skills and parenting practices cited as influenced by overuse were applied as the external criteria, including expressive language, speed of processing, emergent literacy, and cognitive stimulation at home. Psychometric analyses involved Rasch methods and Spearman's ρ correlations., Results: Sixty-nine families were administered ScreenQ. Child age ranged from 36 to 63 months old (52 ± 8; 35 girls). Mean ScreenQ score was 9.6 (±5.0; 1-22). Psychometric properties were strong (r Co-α  = 0.74). ScreenQ scores were negatively correlated with CTOPP-2 (Comprehensive Test of Phonological Processing, Second Edition) (r ρ  = -0.57), EVT-2 (Expressive Vocabulary Test, Second Edition) (r ρ  = -0.45), GRTR (Get Ready to Read!) (r ρ  = -0.30) and StimQ-P (r ρ  = -0.42) scores (all p ≤ 0.01)., Conclusions: ScreenQ shows potential as a composite measure of screen-based media use in young children in the context of AAP recommendations. ScreenQ scores were correlated with lower executive, language and literacy skills, and less stimulating home cognitive environment.

Background: Shared decision making (SDM) between families and physicians may facilitate informed, timely decisions to proceed with biologic therapy in children with inflammatory bowel disease (IBD). Our team previously developed an SDM tool to aid communication between physicians and families when considering biologic therapy for children with IBD., Objective: We are conducting a prospective, pre-post pilot trial of a new SDM tool. The primary aim of the study is to assess feasibility of both the intervention and trial procedures for a future large-scale trial., Methods: We are enrolling physicians with experience prescribing biologic therapy in the past year and families of children with IBD. Families in the intervention arm receive a 3-step intervention including a letter sent before trial consent or clinic appointment, an in-clinic decision tool and a follow-up phone call. Our primary trial outcome is a measure of feasibility, with measures of clinical and decision outcomes secondary. We seek to enroll 27 families in each of 2 arms (usual-care and intervention) and plan data collection at the time of the initial visit or hospital stay, and at 1 week, 3 months, and 6 months after the initial visit., Conclusion: This study protocol is designed to demonstrate that integrating novel consent procedures, including timing and multiple versions of written consent, may increase trial feasibility while maintaining scientific rigor and full protection of study participants.

Background: The consent process used in clinical research today falls markedly short of the ideal process envisioned nearly 30 years ago. Critics have suggested that the informed consent process has become challenging, formalistic, and incompletely understood by researchers and participants alike. Hence, the purpose of this pilot study was to identify and characterize important aspects of the informed consent process that teens believe impact their understanding of risks of participation in studies with genetic testing. Methods: The personal research experiences of 15 teens regarding consent/assent and research participation in studies with genetic testing were solicited through focus-group interviews. All participants had enrolled in at least one research study involving genetic testing in the prior 2 years. All groups were facilitated by the same experienced focus-group moderator. Themes and subthemes were identified, summarized, and interpreted using conventional qualitative content analysis. Results: Three overarching themes emerged from the interviews: fear of what could happen, need for additional information regarding risks, and need for autonomy and decision-making control throughout the consent process. Conclusion: Results of this pilot study provide preliminary evidence that teens can identify and characterize key issues in the informed consent/assent process when it comes to the risks of research participation. These findings are consistent with other research regarding teens' perceptions and recommendations for genetic testing research.

Objective: Hypoplastic left heart syndrome is one of the most common and challenging lesions requiring surgical intervention in the neonatal period. The Norwood procedure for hypoplastic left heart syndrome was first reported in 1983. The objective of this study was to describe early outcomes after the Norwood procedure at a single institution over 30 years., Methods: This retrospective cohort study included all patients with hypoplastic left heart syndrome (and variants) who underwent the Norwood procedure between January 1984 and May 2014 at a single institution. The study period was divided into 6 eras: era 1, 1984 to 1988; era 2, 1989 to 1993; era 3, 1994 to 1998; era 4, 1999 to 2003; era 5, 2004 to 2008; and era 6, 2009 to 2014. The primary outcome was in-hospital mortality after the Norwood procedure. Binomial point estimates complete with 95% confidence intervals (CL 0.95 ) were computed for the entire cohort and by era., Results: During the study period, 1663 infants underwent the Norwood procedure. Overall in-hospital mortality was 25.9% (CL 0.95 , 23.8-28.0). Mortality by chronologic era was 40.4% (CL 0.95 , 34.9-45.9), 33.6% (CL 0.95 , 29.2-37.9), 28.7% (CL 0.95 , 22.8-34.6), 14.9% (CL 0.95 , 10.4-19.3), 11.2% (CL 0.95 , 7.4-15.0), and 15.7% (CL 0.95 , 10.3-21.1). Survival was improved in eras 4 to 6 compared with eras 1 to 3 (P all < .03). Anomalous pulmonary drainage, moderate to severe atrioventricular valve regurgitation, lower birth weight, earlier era, younger gestational age, genetic anomaly, preterm birth, race other than white or African-American, and lower weight at the Norwood procedure were associated with increased mortality. Mortality was greatest in patients with 3 or more risk factors. In the best-fitting multiple covariate model, anomalous pulmonary venous drainage, gestational age in weeks, genetic anomaly, and race other than white and African American were statistically significant contributors, after adjusting for era., Conclusions: Survival after the Norwood procedure has plateaued despite improvements in diagnosis, perioperative care, and surgical techniques. Nonmodifiable patient characteristics are important determinants of the risk of mortality., (Copyright © 2019. Published by Elsevier Inc.)

Background: The American Academy of Pediatrics recommends literacy promotion and developmental assessment during well-child visits. Emergent literacy skills are well defined, and the use of early screening has the potential to identify children at risk for reading difficulties and guide intervention before kindergarten., Methods: The Reading House (TRH) is a children's book designed to screen emergent literacy skills. These are assessed by sharing the book with the child and using a 9-item, scripted scoring form. Get Ready to Read! (GRTR) is a validated measure shown to predict reading outcomes. TRH and GRTR were administered in random order to 278 children (mean: 43.1 ± 5.6 months; 125 boys, 153 girls) during well-child visits at 7 primary care sites. Parent, child, and provider impressions of TRH were also assessed. Analyses included Rasch methods, Spearman-ρ correlations, and logistic regression, including covariates age, sex, and clinic type., Results: Psychometric properties were strong, including item difficulty and reliability. Internal consistency was good for new measures ( r Co- α = 0.68). The mean TRH score was 4.2 (±2.9; range: 0-14), and mean GRTR was 11.1 (±4.4; range: 1-25). TRH scores were positively correlated with GRTR scores ( r s = 0.66; high), female sex, private practice, and child age ( P < .001). The relationship remained significant controlling for these covariates ( P < .05). The mean TRH administration time was 5:25 minutes (±0:55; range: 3:34-8:32). Parent, child, and provider impressions of TRH were favorable., Conclusions: TRH is a feasible, valid, and enjoyable means by which emergent literacy skills in 3- and 4-year-old children can be directly assessed during primary care., Competing Interests: POTENTIAL CONFLICT OF INTEREST: Dr Hutton conceived, wrote, and edited the children’s book used in this study. Dr Hutton is the founder of blue manatee press, the publisher of the book, although he receives no salary or other compensation for this role. The Reading House is not intended for sale in retail, library, or other direct-to-consumer channels. Its intended use is as a screening tool, distributed at low cost to clinical practices and organizations. Royalties, if any, will be determined via intellectual property policies at Cincinnati Children’s Hospital Medical Center; the other authors have indicated they have no potential conflicts of interest to disclose., (Copyright © 2019 by the American Academy of Pediatrics.)

Background: Family-based obesity treatment interventions can successfully reduce energy intake in preschoolers. An implicit goal of obesity treatment interventions is to improve diet quality, but diet quality has been less examined as a treatment outcome in studies of preschoolers. The purpose of this study was to conduct a secondary analysis comparing the change in diet quality and home food environment in preschoolers assigned to a behavioral family-based obesity intervention (LAUNCH), motivational interviewing (MI) condition, or standard care (STC) condition., Methods: Three 24-h dietary recalls were completed at baseline and 6-months and were analyzed using NDS-R software; diet quality was assessed using the Healthy Eating Index-2010 (HEI-2010). Availability of foods and beverages in the home was assessed through direct observation using the Home Health Environment tool that classifies foods and beverages as 'red' or 'green' based upon fat and sugar content. Repeated measures linear mixed effects models were used to examine changes in diet quality and home food environment between conditions (LAUNCH, MI, STC)., Results: At 6-months, preschoolers in the LAUNCH condition had a higher HEI-2010 total score (62.8 ± 13.7) compared to preschoolers in the MI (54.7 ± 13.4, P = 0.022) and STC (55.8 ± 11.6, P = 0.046) conditions. Regarding the home food environment, families in LAUNCH had significantly less 'red' foods in their home at 6-months (12.5 ± 3.4 'red' foods) compared to families in MI (14.0 ± 3.7 'red' foods, P = 0.030), and STC (14.3 ± 3.4 'red' foods, P = 0.006). There were no statistically significant differences across home food environments for number of 'green' foods., Conclusion: Family-based obesity treatment interventions for preschoolers can improve overall diet quality and alter the home food environment through reductions in 'red' foods., Trial Registration: Clinicaltrials.gov, NCT01546727 . Registered March 7, 2012.

Background: Industrial chemicals are increasingly recognized as potential developmental neurotoxicants. Di(2-ethylhexyl) phthalate (DEHP), used to impart flexibility and temperature tolerance to polyvinylchloride, and bisphenol A (BPA), used to manufacture polycarbonate, are commonly present in medical devices. The magnitude of exposure in neonates during hospitalization for cardiac operations is unknown., Methods: We quantified urinary concentrations of DEHP metabolites and BPA preoperatively and postoperatively in neonates undergoing cardiac operations and their mothers. Urinary concentrations of these biomarkers reflect recent exposures (half-lives are approximately 6 to 24 hours). Biomarker concentrations in mothers' and infants' preoperative and postoperative samples were compared., Results: Operations were performed in 18 infants (mean age, 5 ± 4 [SD] days). The maternal sample was obtained on postpartum day 4 ± 4. The preoperative urine sample was obtained on day-of-life 4 ± 2 and the postoperative sample on day-of-life 6 ± 4. Mean maternal concentrations for DEHP metabolites and BPA were at the 50th percentile for females in the United States general population. Infant preoperative concentrations of 1 DEHP metabolite and BPA were significantly higher than maternal concentrations. Postoperative concentrations for all DEHP metabolites were significantly greater than preoperative concentrations., Conclusions: There is considerable perioperative exposure to DEHP and BPA for neonates undergoing cardiac operations. Infant concentrations for both BPA and DEHP metabolites were significantly higher than maternal concentrations, consistent with the infant's exposure to medical devices. Further study is needed to determine the potential role of these suspect neurotoxicants in the etiology of neurodevelopmental disability after cardiac operations., (Copyright © 2019 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Veno-arterial extracorporeal membrane oxygenation is frequently used in patients with cardiac disease. We evaluated short-term outcomes and identified factors associated with hospital mortality in cardiac patients supported with veno-arterial extracorporeal membrane oxygenation., Methods: A retrospective review of patients supported with veno-arterial extracorporeal membrane oxygenation at a university-affiliated children's hospital was performed., Results: A total of 253 patients with cardiac disease managed with extracorporeal membrane oxygenation were identified; survival to discharge was 48%, which significantly improved from 39% in an earlier era (1995-2001) (p=0.01). Patients were categorised into surgical versus non-surgical groups on the basis of whether they had undergone cardiac surgery before or not, respectively. The most common indication for extracorporeal membrane oxygenation was extracorporeal cardiopulmonary resuscitation: 96 (51%) in the surgical group and 45 (68%) in the non-surgical group. In a multiple covariate analysis, single-ventricle physiology (p=0.01), duration of extracorporeal membrane oxygenation (p&lt;0.01), and length of hospital stay (p=0.03) were associated with hospital mortality. Weekend or night shift cannulation was associated with mortality in non-surgical patients (p=0.05)., Conclusion: We report improvement in survival compared with an earlier era in cardiac patients supported with extracorporeal membrane oxygenation. Single-ventricle physiology continues to negatively impact survival, along with evidence of organ dysfunction during extracorporeal membrane oxygenation, duration of extracorporeal membrane oxygenation, and length of stay.

Background: The American Academy of Pediatrics (AAP) recommends shared reading beginning as soon as possible after birth to promote healthy development. Shared reading quality can strongly influence outcomes, especially in children from low-SES households. Dialogic reading is a method developed to enhance verbal interactivity and engagement through book sharing, advocated by the AAP and clinic-based programs such as Reach Out and Read. There is no brief, validated, caregiver report measure of dialogic reading or shared reading quality currently available., Methods: This cross-sectional study involved 49 healthy mother-child dyads (mean child age 4.5 yrs., SD = 0.6 yrs.) from 2 separate MRI-based studies. The DialogPR was administered by trained research coordinators following MRI, along with the READ subscale of the validated StimQ-P measure of home cognitive environment. The DialogPR consists of eight items developed in consultation with experts in early literacy, based on the PEER/CROWD dialogic reading conceptual model. Estimated reading level is 6th grade. Descriptive statistics were computed at both the item and scale levels. Modern theory Rasch methods were used to analyze all eight DialogPR items along with preliminary estimates of reliability and validity., Results: Our combined sample involved 15 boys and 34 girls, and was diverse in terms of age, household income, and maternal education. DialogPR administration time was less than 2 min, with no problems reported. The DialogPR demonstrated strong internal consistency and reliability (Cronbach's alpha = 0.82), and criterion-related validity with the StimQ-P READ (Spearman's rho coefficient = 0.53). Rasch analysis revealed strong psychometric properties in terms of reliability, variability in item difficulty, and inter-item and item-measure correlations., Conclusions: Preliminary evidence suggests that the DialogPR may be an efficient means to assess shared reading quality and dialogic reading via caregiver report for clinical and research purposes, warranting further investigation.

Objectives: Pregnancies with congenital heart disease in the foetus have an increased prevalence of pre-eclampsia, small for gestational age and preterm birth, which are evidence of an impaired maternal-foetal environment (MFE)., Methods: The impact of an impaired MFE, defined as pre-eclampsia, small for gestational age or preterm birth, on outcomes after cardiac surgery was evaluated in neonates (n = 135) enrolled in a study evaluating exposure to environmental toxicants and neuro-developmental outcomes., Results: The most common diagnoses were transposition of the great arteries (n = 47) and hypoplastic left heart syndrome (n = 43). Impaired MFE was present in 28 of 135 (21%) subjects, with small for gestational age present in 17 (61%) patients. The presence of an impaired MFE was similar for all diagnoses, except transposition of the great arteries (P < 0.006). Postoperative length of stay was shorter for subjects without an impaired MFE (14 vs 38 days, P < 0.001). Hospital mortality was not significantly different with or without impaired MFE (11.7% vs 2.8%, P = 0.104). However, for the entire cohort, survival at 36 months was greater for those without an impaired MFE (96% vs 68%, P = 0.001). For patients with hypoplastic left heart syndrome, survival was also greater for those without an impaired MFE (90% vs 43%, P = 0.007)., Conclusions: An impaired MFE is common in pregnancies in which the foetus has congenital heart disease. After cardiac surgery in neonates, the presence of an impaired MFE was associated with lower survival at 36 months of age for the entire cohort and for the subgroup with hypoplastic left heart syndrome.

Objective: National health organizations and expert committees have issued recommendations for health behaviors related to obesity risk. Behavioral and family-based weight management interventions for preschoolers often target improving adherence to these recommendations, but it is unknown how the health behaviors of preschoolers with obesity enrolled in weight control treatments (WCTs) compare with these guidelines. In this study, the dietary intake, activity, and sleep behaviors of preschoolers with obesity enrolled in a family-based behavioral WCT are described and compared with national health behavior recommendations., Methods: Health behaviors of 151 preschoolers with obesity (M age = 4.60, SD = 0.93) enrolled in a clinical trial of a weight management program were measured at baseline through caregiver-report questionnaires, three 24-hr dietary recalls, and accelerometers., Results: In total, 70% of the sample exceeded daily caloric recommendations, only 10 and 5% met recommendations for fruit and vegetable intake, respectively, and only 30% met the recommendation of consuming no sugar-sweetened beverages. The majority of the sample met the daily recommendations for 60 min of moderate-to-vigorous activity (80%), < 2 hr of screen time (68%), and sleep duration (70%)., Conclusions: Behavioral weight management interventions for preschoolers with obesity should target the health behaviors where children are not meeting recommendations.

The paper-based pedigree is the current standard for family health history (FHH) documentation in genetic counseling. Several tools for electronic capture of family health data have been developed to improve re-use and accessibility, data quality and standardization, ease of updating, and integration with electronic medical records. One such tool, the tablet-based Proband application, provides a flexible approach to data capture in dynamic and diverse clinical settings. This study compared Proband FHH collection to paper-based methods and investigated the usability of Proband in a clinical setting. After one use by 23 genetic counselors and students, Proband had 91% accuracy with a FHH audio scenario, which was significantly less (p < 0.001) than paper's 96% accuracy. These differences were attributed to incorrect or missing ages of grandparents (p < 0.001) and great-aunts/uncles (p = 0.012) and missing documentation of consanguinity (p < 0.001). Possible explanations for these differences include greater experience with paper FHH documentation and pre-populated prompts for consanguinity on the paper template used. Proband's perceived usability increased with use, with individual System Usability Scores increasing between first and last use (p = 0.033). We conclude that tools for dynamic, provider-driven FHH documentation such as Proband show promise for improving risk assessment accuracy and quality patient care.

This study aimed to provide insight into the development of postural control abilities in youth. A total of 276 typically developing adolescents (155 males, 121 females) with a mean age of 13.23 years (range of 7.11-18.80) were recruited for participation. Subjects performed two-minute quiet standing trials in bipedal stance on a force plate. Center of pressure (COP) trajectories were quantified using Sample Entropy (SampEn) in the anterior-posterior direction (SampEn-AP), SampEn in the medial-lateral direction (SampEn-ML), and Path Length (PL) measures. Three separate linear regression analyses were conducted to predict the relationship between age and each of the response variables after adjusting for individuals' physical characteristics. Linear regression models showed an inverse relationship between age and entropy measures after adjusting for body mass index. Results indicated that chronological age was predictive of entropy and path length patterns. Specifically, older adolescents exhibited center of pressure displacement (smaller path length) and less complex, more regular center of pressure displacement patterns (lower SampEn-AP and SampEn-ML values) compared to the younger children. These findings support prior studies suggesting that developmental changes in postural control abilities may continue throughout adolescence and into adulthood., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Objectives: To investigate the prevalence of hearing loss after cardiac surgery in infancy, patient and operative factors associated with hearing loss, and the relationship of hearing loss to neurodevelopmental outcomes., Study Design: Audiologic and neurodevelopmental evaluations were conducted on 348 children who underwent repair of congenital heart disease at the Children's Hospital of Philadelphia as part of a prospective study evaluating neurodevelopmental outcomes at 4 years of age. A prevalence estimate was calculated based on presence and type of hearing loss. Potential risk factors and the impact of hearing loss on neurodevelopmental outcomes were evaluated., Results: The prevalence of hearing loss was 21.6% (95% CI, 17.2-25.9). The prevalence of conductive hearing loss, sensorineural hearing loss, and indeterminate hearing loss were 12.4% (95% CI, 8.8-16.0), 6.9% (95% CI, 4.1-9.7), and 2.3% (95% CI, 0.6-4.0), respectively. Only 18 of 348 subjects (5.2%) had screened positive for hearing loss before this study and 10 used a hearing aid. After adjusting for patient and operative covariates, younger gestational age, longer postoperative duration of stay, and a confirmed genetic anomaly were associated with hearing loss (all P < .01). The presence of hearing loss was associated with worse language, cognition and attention (P <.01)., Conclusions: These findings suggest that the prevalence of hearing loss in preschool children after heart surgery in infancy may be 20-fold higher than in the 1% prevalence seen in the general population. Younger gestational age, presence of a genetic anomaly, and longer postoperative duration of stay were associated with hearing loss. Hearing loss was associated with worse neurodevelopmental outcomes., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Objective: To test the hypotheses that an innovative skills-based behavioral family clinic and home-based intervention (LAUNCH) would reduce body mass index z score (BMIz) compared with motivational interviewing and to standard care in preschool-aged children with obesity., Study Design: Randomized controlled trial with children between the ages of 2 and 5 years above the 95 th percentile for body mass index for age and sex recruited from 27 pediatrician offices across 10 recruitment cycles between March 12, 2012 and June 8, 2015. Children were randomized to LAUNCH (an 18-session clinic and home-based behavioral intervention), motivational interviewing (delivered at the same frequency as LAUNCH), or standard care (no formal intervention). Weight and height were measured by assessors blinded to participant assignment. The primary outcome, BMIz at month 6 after adjusting for baseline BMIz, was tested separately comparing LAUNCH with motivational interviewing and LAUNCH with standard care using regression-based analysis of covariance models., Results: A total of 151 of the 167 children randomized met intent-to-treat criteria and 92% completed the study. Children were 76% White and 57% female, with an average age of 55 months and BMI percentile of 98.57, with no demographic differences between the groups. LAUNCH participants demonstrated a significantly greater decrease in BMIz (mean = -0.32, SD = ±0.33) compared with motivational interviewing (mean = -0.05, SD = ±0.27), P < .001, ω 2  = 0.74 and compared with standard care (mean = -0.13, SD = ±0.31), P < .004, ω 2  = 0.75., Conclusions: In preschool-age children, an intensive 6-month behavioral skills-based intervention is necessary to reduce obesity., Trial Registration: Clinicaltrials.gov NCT01546727., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Background: Left ventricular noncompaction (LVNC) can occur in isolation or can co-occur with a cardiomyopathy phenotype or cardiovascular malformation. The yield of cardiomyopathy gene panel testing in infants, children, and adolescents with a diagnosis of LVNC is unknown. By characterizing a pediatric population with LVNC, we sought to determine the yield of cardiomyopathy gene panel testing, distinguish the yield of testing for LVNC with or without co-occurring cardiac findings, and define additional factors influencing genetic testing yield., Methods and Results: One hundred twenty-eight individuals diagnosed with LVNC at ≤21 years of age were identified, including 59% with idiopathic pathogenesis, 32% with familial disease, and 9% with a syndromic or metabolic diagnosis. Overall, 75 individuals had either cardiomyopathy gene panel (n=65) or known variant testing (n=10). The yield of cardiomyopathy gene panel testing was 9%. The severity of LVNC by imaging criteria was not associated with positive genetic testing, co-occurring cardiac features, pathogenesis, family history, or myocardial dysfunction. Individuals with isolated LVNC were significantly less likely to have a positive genetic testing result compared with those with LVNC and co-occurring cardiomyopathy (0% versus 12%, respectively; P <0.01)., Conclusions: Genetic testing should be considered in individuals with cardiomyopathy co-occurring with LVNC. These data do not suggest an indication for cardiomyopathy gene panel testing in individuals with isolated LVNC in the absence of a family history of cardiomyopathy., (© 2017 American Heart Association, Inc.)

Objective: Extracorporeal membrane oxygenation is an important form of short-term mechanical support in children with cardiac disease, but information on long-term outcomes and quality of life is limited. The primary objective of this study was to determine the long-term outcomes of children previously supported by extracorporeal membrane oxygenation for cardiac etiologies., Design: A retrospective analysis was performed on patients with cardiac disease managed with extracorporeal membrane oxygenation between January 1, 1995, and December 31, 2012, at the Children's Hospital of Philadelphia. Survivors completed patient- and parent-reported verbal and written surveys, and univariate analyses assessed risk factors for long-term outcomes., Setting: Tertiary-care children's hospital., Patients: Patients with cardiac disease managed with extracorporeal membrane oxygenation., Interventions: None., Measurements and Main Results: Over 18 years, 396 patients were managed with extracorporeal membrane oxygenation with 43% survival to discharge. The median age at cannulation was 78 days. The majority had congenital heart disease (86%), surgery prior to extracorporeal membrane oxygenation (71%), and cardiopulmonary arrest as the primary extracorporeal membrane oxygenation indication (53%). With 6-year median follow-up, 66% are known to be deceased, including 38 deaths after hospital discharge. Among survivors at discharge, 65 (38%) completed the phone survey, and 33 (19%) completed the written survey. Negative clinical outcomes, defined as having at least significant physical limitations or "fair" or "poor" health, were present in 18% of patients. No patient- or extracorporeal membrane oxygenation-related variables were associated with negative outcomes in univariate analyses. There were significantly lower self-reported and parent-reported written Pediatric Quality of Life Inventory quality of life scores in children compared with healthy individual normative data but no differences in adolescents., Conclusions: In this series of pediatric cardiac patients supported by extracorporeal membrane oxygenation, mortality was 66% with 6-year median follow-up. The majority reported positive outcomes with respect to health and physical limitations, but children reported lower quality of life compared with healthy individuals.

Objectives: We sought to assess the effect of congenital heart disease requiring infant surgery with cardiopulmonary bypass on neurodevelopmental outcomes and growth at 4 years of age, while matching for gestational age, socioeconomic status, maternal gestational conditions, home environment, and parental intelligence by studying multiple-gestation births., Methods: We performed within-family comparison of 14 multiple-gestation births in which 1 child had congenital heart disease requiring surgery with cardiopulmonary bypass at ≤6 months of age. Between 4 and 5 years of age, a comprehensive neurodevelopmental assessment was performed. Paired comparisons were conducted between siblings with and without heart defects using a series of nonparametric tests., Results: On average, the children qualified as late preterm (mean gestational age 35.4 ± 2.6 weeks). At an average age of 4.8 ± 0.1 years, children with congenital heart disease weighed less than their siblings (median weight for age z score -0.4 vs 0.1, P = .02) and had worse performance for cognition (median full-scale IQ 99 vs 109, P = .02) and fine motor skills (median Wide Range Assessment of Visual Motor Ability, Fine Motor score 94.5 vs 107.5, P < .01)., Conclusions: After controlling for socioeconomic status, home environment, parental intelligence, and gestational factors by using multiple-gestation births, congenital heart disease requiring surgery with cardiopulmonary bypass at ≤6 months of age is associated with lower weight, cognitive abilities and fine motor skills at 4 years of age., (Copyright © 2017 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Background/objectives: Many children do not consume the recommended daily allowance of calcium. Inadequate calcium intake in childhood may limit bone accrual. The objective of this study was to determine if a behavioral modification and nutritional education (BM-NE) intervention improved dietary calcium intake and bone accrual in children., Subjects/methods: 139 (86 female) healthy children, 7-10 years of age, were enrolled in this randomized controlled trial conducted over 36 months. Participants randomized to the BM-NE intervention attended five sessions over a 6-week period designed to increase calcium intake to 1500 mg/day. Participants randomized to the usual care (UC) group received a single nutritional counseling session. The Calcium Counts Food Frequency Questionnaire was used to assess calcium intake; dual energy X-ray absorptiometry was used to assess areal bone mineral density (aBMD) and bone mineral content (BMC). Longitudinal mixed effects models were used to assess for an effect of the intervention on calcium intake, BMC and aBMD., Results: BM-NE participants had greater increases in calcium intake that persisted for 12 months following the intervention compared with UC. The intervention had no effect on BMC or aBMD accrual. Secondary analyses found a negative association between calcium intake and adiposity such that greater calcium intake was associated with lesser gains in body mass index and fat mass index., Conclusions: A family-centered BM-NE intervention program in healthy children was successful in increasing calcium intake for up to 12 months but had no effect on bone accrual. A beneficial relationship between calcium intake and adiposity was observed and warrants future study.

Obesity affects nearly 2 million preschool age children in the United States and is not abating. However, research on interventions for already obese preschoolers is limited. To address this significant gap in the literature, we developed an intervention targeting obesity reduction in 2 to 5year olds, Learning about Activity and Understanding Nutrition for Child Health (LAUNCH). This paper describes the rationale, design, participant enrollment, and implementation of a 3-arm randomized, parallel-group clinical trial comparing LAUNCH to a motivational-interviewing intervention (MI) and standard care (STC), respectively. Whereas LAUNCH was designed as a skills based intervention, MI focused on addressing the guardian's motivation to make changes in diet and activity and providing tools to do so at the guardian's level of readiness to implement changes. Child body mass index z-score was the primary outcome, assessed at pretreatment, posttreatment (Month 6), and 6 and 12month follow-ups (Months 12 and 18). Mechanisms of weight change (e.g., dietary intake, physical activity) and environmental factors associated with weight (e.g., foods available in the home, caregiver diet) were also assessed. This study is unique because it is one of the few randomized controlled trials to examine a developmentally informed, clinic and home skills based behavioral family intervention for preschoolers who are already obese. Being obese during the preschool years increases the likelihood of remaining obese as an adult and is associated with serious health conditions; if this intervention is successful, it has the potential to change the health trajectories for young children with obesity., (Copyright © 2016. Published by Elsevier Inc.)

Objectives: The aim of the study was to understand the association between parents' perceptions of the decision process and the decision outcomes in decisions about the use of biologics in pediatric chronic conditions., Methods: We mailed surveys to parents of children with inflammatory bowel disease or juvenile idiopathic arthritis who had started treatment with biologics in the prior 2 years and were treated at either of 2 children's hospitals. The survey included measures of the decision process, including decision control and physician engagement, and decision outcomes, including conflict and regret. We used means and frequencies to assess the response distributions. General linear models were used to test the associations between decision process and decision outcomes., Results: We had 201 respondents (response rate 54.9%). Approximately 47.0% reported using shared decision making. Each physician engagement behavior was experienced by the majority of parents, with the highest percentage reporting that their child's physician used language they understood and listened to them. Approximately 48.5% of parents had decisional conflict scores of 25 or greater, indicating high levels of conflict. Approximately 28.2% had no regret, 31.8% had mild regret, and the remaining 40.0% had moderate to severe regret. Shared decision making was not associated with improved decisional conflict, but physician engagement behaviors were associated with both decisional conflict and regret., Conclusions: Improving decision outcomes will require more than just focusing on who parents perceive as controlling the final decision. Developing interventions that facilitate specific physician engagement behaviors may decrease parents' distress around decision making and improve decision outcomes., Competing Interests: The authors have no conflicts of interest relevant to this article to disclose.

Objectives: Despite improved survival in children with hypoplastic left heart syndrome (HLHS), significant concern persists regarding their neurodevelopmental (ND) outcomes. Previous studies have identified patient factors, such as prematurity and genetic syndromes, to be associated with worse ND outcomes. However, no consistent relationships have been identified among modifiable management factors, including cardiopulmonary bypass strategies, and ND outcomes after cardiac surgery in infancy. Studies in immature animals, including primates, have demonstrated neurodegeneration and apoptosis in the brain after certain levels and extended durations of anesthetic exposure. Retrospective human studies have also suggested relationships between adverse ND effects and anesthetic exposure., Methods: Cumulative minimum alveolar concentration hours (MAC-hrs) of exposure to volatile anesthetic agents (VAA) (desflurane, halothane, isoflurane, and sevoflurane) were collected from an anesthetic database and medical record review for 96 patients with HLHS or variants. ND testing was performed between ages 4 and 5 years, including full-scale IQ, verbal IQ, performance IQ, and processing speed. Four generalized linear modes were hypothesized a priori and tested using a Gaussian (normal) distribution with an identity link., Results: Cumulative VAA exposure ranged from 0 to 35.3 MAC-hrs (median 7.5 hours). Using specified covariates identified previously as significant predictors of ND outcomes, statistically significant relationships were identified between total MAC-hrs exposure and worse full-scale IQ and verbal IQ scores (P's < .05) alone and after adjusting for relevant covariates., Conclusions: Increased cumulative MAC-hrs exposure to VAA is associated with worse ND outcomes in certain domains in children with HLHS and variants., (Copyright © 2016 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)