Percent Body Fat and Weight Status of Youth Participating in Pediatric Weight Management Programs in the Pediatric Obesity Weight Evaluation Registry.

Factors associated with change in percent body fat (%BF) of children in pediatric weight management (PWM) care may differ from those associated with change in weight status. To describe %BF and weight status at initial visits to 14 PWM sites, identify differences by sex, and evaluate factors associated with change over 6 months. Initial visits of 2496 males and 2821 females aged 5-18 years were evaluated.

Evidence-Based Assessment of Congenital Heart Disease Genes to Enable Returning Results in a Genomic Study.

Background: Congenital heart disease (CHD) is the most common major congenital anomaly and causes significant morbidity and mortality. Epidemiologic evidence supports a role of genetics in the development of CHD. Genetic diagnoses can inform prognosis and clinical management.

Synergistic association of fluid overload and acute kidney injury on outcomes in pediatric cardiac ECMO: a retrospective analysis of the KIDMO database.

Background: Acute kidney injury (AKI) and fluid overload (FO) are associated with poor outcomes in children receiving extracorporeal membrane oxygenation (ECMO). Our objective is to evaluate the impact of AKI and FO on pediatric patients receiving ECMO for cardiac pathology.

Methods: We performed a secondary analysis of the six-center Kidney Interventions During Extracorporeal Membrane Oxygenation (KIDMO) database, including only children who underwent ECMO for cardiac pathology.

Design and rationale for the supermarket and web-based intervention targeting nutrition (SuperWIN) for cardiovascular risk reduction trial.

Purpose: The prevalence of chronic diseases is increasing largely due to suboptimal dietary habits. It is not known whether individualized, supermarket-based, nutrition education delivered by registered dietitians, utilizing the advantages of the in-store and online environments, and electronically collected purchasing data, can increase dietary quality.

Methods And Results: The supermarket and web-based intervention targeting nutrition (SuperWIN) for cardiovascular risk reduction trial is a randomized, controlled dietary intervention study.

Characteristics of paediatric weight management in the United States: Associations with program retention and BMI outcomes in the paediatric obesity weight evaluation registry (POWER).

Objective: To describe characteristics of paediatric weight management (PWM) programs across the United States and evaluate associations with program-specific retention rates and body mass index (BMI) outcomes at 6 months.

Methods: A program profile survey was administered to 33 programs within the Paediatric Obesity Weight Evaluation Registry (POWER) to assess program staffing, services, and treatment format. Patient retention and percent of the 95th BMI percentile (%BMIp95) changes were assessed for each program.

Belatacept for Simultaneous Calcineurin Inhibitor and Chronic Corticosteroid Immunosuppression Avoidance: Two-Year Results of a Prospective, Randomized Multicenter Trial.

Background And Objectives: Immunosuppressive therapy in kidney transplantation is associated with numerous toxicities. CD28-mediated T-cell costimulation blockade using belatacept may reduce long-term nephrotoxicity, compared with calcineurin inhibitor-based immunosuppression. The efficacy and safety of simultaneous calcineurin inhibitor avoidance and rapid steroid withdrawal were tested in a randomized, prospective, multicenter study.

Elucidation of de novo small insertion/deletion biology with parent-of-origin phasing.

The mechanisms underlying de novo insertion/deletion (indel) genesis, such as polymerase slippage, have been hypothesized but not well characterized in the human genome. We implemented two methodological improvements, which were leveraged to dissect indel mutagenesis. We assigned de novo variants to parent-of-origin (i.

Belatacept-based immunosuppression with simultaneous calcineurin inhibitor avoidance and early corticosteroid withdrawal: A prospective, randomized multicenter trial.

Simultaneous calcineurin inhibitor avoidance (CNIA) and early corticosteroid withdrawal (ESW) have not been achieved primarily due to excessive acute rejection. This trial compared 2 belatacept-based CNIA/ESW regimens with a tacrolimus-based ESW regimen. Kidney transplant recipients were randomized to receive alemtuzumab/belatacept, rabbit anti-thymocyte globulin (rATG)/belatacept, or rATG/tacrolimus.

Implementing a Novel Quality Improvement-Based Approach to Data Quality Monitoring and Enhancement in a Multipurpose Clinical Registry.

Objective: To implement a quality improvement based system to measure and improve data quality in an observational clinical registry to support a Learning Healthcare System.

Data Source: ImproveCareNow Network registry, which as of September 2019 contained data from 314,250 visits of 43,305 pediatric Inflammatory Bowel Disease (IBD) patients at 109 participating care centers.

Study Design: The impact of data quality improvement support to care centers was evaluated using statistical process control methodology.

Health Outcomes of Youth in Clinical Pediatric Weight Management Programs in POWER.

Objective: To describe treatment outcomes of children and adolescents enrolled in the Pediatric Obesity Weight Evaluation Registry, a consortium of multicomponent pediatric weight management programs in the US.

Study Design: This multicenter prospective observational cohort study, established in 2013, includes youth (2-18 years of age) with obesity enrolled from 31 Pediatric Obesity Weight Evaluation Registry (POWER) sites over a 2-year period and followed up to 12 months. Weight status was evaluated by the percentage of the 95th percentile for body mass index (%BMIp95).

Assessment of tacrolimus intrapatient variability in stable adherent transplant recipients: Establishing baseline values.

The purpose of this study was to determine the intrapatient (within the same patient) variability of tacrolimus in adherent patients. Daily tacrolimus trough levels were obtained at home using dried blood spot technology in kidney and liver transplant recipients. Patients were randomized to receive 3 formulations of tacrolimus, each for two 1-week periods.

Test-retest variability for quantitative two-dimensional and Doppler measurements in the fetus.

Objectives: Echocardiography is used to quantitatively characterize cardiovascular function in fetuses with cardiac abnormalities and inform decisions for fetal or perinatal interventions. It is clinically important to understand the reproducibility of these measures, particularly between testers. While studies have reported intra-observer variability and inter-observer variability, little is known about test-retest variability for these measures.

Creating a multi-center rare disease consortium - the Consortium of Eosinophilic Gastrointestinal Disease Researchers (CEGIR).

 Eosinophilic gastrointestinal disorders (EGIDs) affect various segments of the gastrointestinal tract. Since these disorders are rare, collaboration is essential to enroll subjects in clinical studies and study the broader population. The Rare Diseases Clinical Research Network (RDCRN), a program of the National Center for Advancing Translational Sciences (NCATS), funded the Consortium of Eosinophilic Gastrointestinal Disease Researchers (CEGIR) in 2014 to advance the field of EGIDs.

Fetal somatic growth trajectory differs by type of congenital heart disease.

BackgroundThe growth trajectories of common measurements, including estimated fetal weight (EFW), head circumference (HC), and abdominal circumference (AC), in fetuses with congenital heart disease (CHD) have not been described for different cardiac lesions. We hypothesized that (i) fetuses with CHD have differential growth in utero, and (ii) different categories of CHD demonstrate different in utero growth curves.MethodsWe performed a retrospective observational cohort study of pregnancies with known fetal CHD seen from January 2000 to June 2013.

Bioequivalence between innovator and generic tacrolimus in liver and kidney transplant recipients: A randomized, crossover clinical trial.

Background: Although the generic drug approval process has a long-term successful track record, concerns remain for approval of narrow therapeutic index generic immunosuppressants, such as tacrolimus, in transplant recipients. Several professional transplant societies and publications have generated skepticism of the generic approval process. Three major areas of concern are that the pharmacokinetic properties of generic products and the innovator (that is, "brand") product in healthy volunteers may not reflect those in transplant recipients, bioequivalence between generic and innovator may not ensure bioequivalence between generics, and high-risk patients may have specific bioequivalence concerns.

Impaired cerebral autoregulation in preoperative newborn infants with congenital heart disease.

Objectives: To characterize cerebral autoregulation (CA) in preoperative newborn infants with congenital heart disease (CHD).

Methods: This was a prospective, pilot study of term newborns with CHD who required intensive care. Continuous mean arterial blood pressure (MAP), cerebral tissue oxygen saturation (SO) via near-infrared spectroscopy, and arterial oxygen saturation (SaO) were collected.

Utility of Echocardiography in the Assessment of Left Ventricular Diastolic Function and Restrictive Physiology in Children and Young Adults with Restrictive Cardiomyopathy: A Comparative Echocardiography-Catheterization Study.

The aim of the study is to determine the utility of echocardiography in the assessment of diastolic function in children and young adults with restrictive cardiomyopathy (RCM). RCM is a rare disease with high mortality requiring frequent surveillance. Accurate, noninvasive echocardiographic measures of diastolic function may reduce the need for invasive catheterization.

Digoxin Use Is Associated With Reduced Interstage Mortality in Patients With No History of Arrhythmia After Stage I Palliation for Single Ventricle Heart Disease.

Background: Interstage mortality (IM) remains significant after stage 1 palliation (S1P) for single-ventricle heart disease (SVD), with many deaths sudden and unexpected. We sought to determine whether digoxin use post-S1P is associated with reduced IM, utilizing the multicenter database of the National Pediatric Cardiology Quality Improvement Collaborative (NPCQIC).

Methods And Results: From June 2008 to July 2013, 816 infants discharged after S1P from 50 surgical sites completed the interstage to stage II palliation, transplant, or IM.

Characteristics of Youth Presenting for Weight Management: Retrospective National Data from the POWER Study Group.

Background: There are no existing multisite national data on obese youth presenting for pediatric weight management. The primary aim was to describe BMI status and comorbidities among youth with obesity presenting for pediatric weight management (PWM) at programs within the Pediatric Obesity Weight Evaluation Registry (POWER).

Methods: Data were collected from 2009-2010 among 6737 obese patients ages 2-17.

Correlation of precordial voltages to left ventricular mass on echocardiogram in adolescent patients with hypertrophic cardiomyopathy compared with that in adolescent athletes.

Electrocardiograms continue to be part of screening programs for athletes and familial hypertrophic cardiomyopathy (HC). Whether electrocardiographic (ECG) findings of left ventricular (LV) hypertrophy can distinguish between healthy populations and those with HC remains unclear. We sought to (1) analyze the relation between ECG voltage and LV mass in patients with HC and (2) evaluate ECG characteristics of patients with phenotypical HC.

S-(-)equol production is developmentally regulated and related to early diet composition.

S-(-)7-hydroxy-3-(4'-hydroxyphenyl)-chroman, or S-(-)equol, a biologically active intestinally derived bacterial metabolite of the soy isoflavones daidzin/daidzein, is not produced in neonatal life. Because its synthesis is dependent on equol-producing bacteria, we hypothesized that early nutrition may influence equol production. This prospective 2.

Dietary factors influence production of the soy isoflavone metabolite s-(-)equol in healthy adults.

S-(-)equol, an intestinally derived metabolite of the soy isoflavone daidzein, is proposed to enhance the efficacy of soy diets. Adults differ in their ability to produce equol when consuming soy foods for reasons that remain unclear. Therefore, we performed a comprehensive dietary analysis of 143 macro- and micronutrients in 159 healthy adults in the United States (n = 89) and Australia (n = 70) to determine whether the intake of specific nutrients favors equol production.