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The UV/hydrogen peroxide (H2O2) advanced oxidation process (AOP) frequently employed to generate hydroxyl radical (•OH) to treat reverse osmosis permeate (ROP) in potable reuse treatment trains is ...

OBJECTIVE: To evaluate the relationship between degree of cognitive impairment and gray-matter density changes in the auditory cortex. STUDY DESIGN: Retrospective case-control. PATIENTS: Six hundred sixty-three patients of a tertiary referral center cognitive disorders clinic. INTERVENTION: Magnetic resonance imaging. MAIN OUTCOME MEASURES: Ratios of gray matter density of the primary auditory cortex (A1) to whole brain and auditory association cortex (AAC) to whole brain in patients with Alzheimer's disease (AD) compared with mild cognitive impairment (MCI) and patients with a mini-mental state exam (MMSE) scores ≤25 versus >25. RESULTS: After multivariate analysis, a statistically significant difference between AAC to brain ratios for patients with a MMSE ≤25 (n = 325) compared with >25 (n = 269) was found, with values -0.03 (95% CI -0.04 to -0.02, p 

INTRODUCTION: Albuterol can trigger supraventricular tachycardia (SVT). The clinical characteristics, incidence, and risk factors of SVT after inhaled SABA treatment in children are currently unknown. Through review of regional care delivery, we will describe cases of SVT during asthma treatment in hospital-based settings, define the incidence of SVT in our population, and evaluate risk factors of SABA-induced SVT. METHODS: We identified hospital-based care episodes of children 0–18 years old between 2006 and 2015 recorded in the Intermountain Healthcare EDW with either 1) diagnosis codes for both asthma and SVT or 2) both SABA and adenosine listed as billed medications. Controls were matched with cases by age and sex to determine risk factors for SVT after SABA using conditional logistic regression. RESULTS: Of 93 care episodes meeting criteria, we found 7 cases of SVT after SABA treatment in 6 patients over 10 years. In our population, the incidence of SVT is 3.9 per 10,000 episodes of SABA treatment, and 5.1 per 10,000 children with asthma receiving hospital-based asthma care. Two episodes of SVT followed treatment with only levalbuterol, three after only albuterol, and two after both albuterol and levalbuterol treatment. Five cases of SVT were converted to sinus rhythm with adenosine, one converted with synchronized electrical cardioversion, and one resolved spontaneously. No cases of SVT led to death. No examined variables were associated with SABA-induced SVT. CONCLUSIONS: SVT is rare during hospital-based treatment for acute asthma using inhaled SABAs and has low morbidity and mortality.

Oxidation catalysts called NewTAMLs, macrocyclic complexes with TAML carbonamido-N donors replaced by more nucleophile-resistant binders, sulfonamido-N, for example, [Fe{4-NO2C6H3-1,2-(NCOCMe2NSO2)...

Introduction: Previous studies have reported on the evaluation of patients diagnosed with appendicitis. Very little is known about all patients evaluated for suspected appendicitis. Patients evaluated beyond physical examination with laboratory and imaging testing, then found not to have appendicitis, are more difficult to identify. Data readily available in administrative databases may be used to identify these patients. Methods: A multidisciplinary team developed a surrogate definition for evaluating suspected appendicitis in children based on available administrative data. Appendicitis was “suspected” if the patient underwent ultrasonography of the appendix or had a chief complaint of abdominal pain with both complete blood count performed and the word “appendicitis” in the ED provider note. Performance characteristics described the surrogate definition’s ability to retrospectively identify patients evaluated for suspected appendicitis through comparison to a population identified via chart review. Results: Compared with manual chart review of 498 patients from June 2014, the surrogate definition identified patients evaluated beyond physical examination for suspected appendicitis with a sensitivity of 79.8%, a specificity of 96.3%, a positive predictive value of 83.3%, and a negative predictive value of 95.3%. Of the 94 patients evaluated beyond physical examination for suspected appendicitis, 37 (39%) underwent appendectomy. Conclusions: Health systems can retrospectively identify children evaluated beyond physical examination for appendicitis using discrete administrative data and a word search of clinical notes. This surrogate definition for evaluation of suspected appendicitis enables research in quality improvement efforts and health care resource utilization.

Part 2: E-Government Services and Open Government; International audience; Measuring customer service quality evaluations has been important since the rise of the service industry and many models in this area have been published. Most models focus on one outcome with a set of predictors. These outcomes are often ill defined and concepts are used interchangeably causing issues in creating good and consistent measures of quality. In this study we develop a new model combining multiple outcome variables and a series of predictors to show the interdependent nature of service outcomes. We test the model using machine learning based on survey responses from 3702 Dutch people. The results indicate that two types of outcome variables are important; quality of the outcome and satisfaction with the process. Each is predicted in different ways by four dimensions. This means governments could benefit from a better specification of the desired outcomes of service delivery and targeted measurement approaches.

Objectives: This study sought to determine the safety and feasibility of transcatheter pulmonary valve replacement (TPVR) using the Melody valve in native (nonconduit) right ventricular out...

AIM To describe neurobehavioral patterns in neonates with congenital heart disease (CHD). METHOD A cohort study describing neurobehavioral performance of neonates with CHD requiring cardiac surgery. The neonates were evaluated preoperatively and postoperatively with the Neonatal Intensive Care Unit Network Neurobehavioral Scale (NNNS) and scores were compared with published normative values. Clinical factors were obtained by chart review to assess their association with behavior. The CHD NNNS score pattern was compared with previously reported profiles in other high-risk populations. RESULTS NNNS evaluations were completed on 67 neonates with CHD, resulting in 97 evaluations (50 preoperative, 47 postoperative). Compared with normative values, the cohort with CHD demonstrated decreased attention, regulation, asymmetry, stress, arousal, and excitability, along with increased non-optimal reflexes, lethargy, and need for handling (p

Seizures are present in over 90% of infants and children with Wolf-Hirschhorn syndrome (WHS). When present, they significantly affect quality of life. The goal of this study was to use caregiver reports to describe the comparative efficacies of commonly used antiepileptic medications in a large population of individuals with WHS. A web-based, confidential caregiver survey was developed to capture seizure semiology and a chronologic record of seizure treatments as well as responses to each treatment. Adverse events for each drug were also cataloged. We received 141 complete survey responses (47% response rate) describing the seizures of individuals ranging in age from 4months to 61years (90 females: 51 males). Using the Early Childhood Epilepsy Severity Scale (E-Chess), WHS-associated seizures are demonstrably severe regardless of deletion size. The best-performing antiepileptic drugs (AEDs) for controlling seizures in this cohort were broad spectrum drugs clobazam, levetiracetam, and lamotrigine; whereas, the three commonly used carboxamide class drugs: carbamazepine, phenytoin, and oxcarbazepine, were reported to have little effect on, or even exacerbate, seizures. The carboxamide class drugs, along with phenobarbital and topiramate, were also associated with the highest rate of intolerance due to cooccurrence of adverse events. Levetiracetam, clobazam, and clonazepam demonstrated higher tolerability and comparatively less severe adverse events (Wilcoxon rank sum comparison between performance of levetiracetam and carboxamide class drugs gives a p

Children with acquired and congenital heart disease (CHD) are increasingly surviving to adulthood. Our aim was to determine the prevalence of coronary artery disease (CAD) risk factors in children at known risk for early CAD or with severe CHD. We recruited children (8-19 years) at risk for early CAD-Kawasaki disease (KD, N = 36) and coarctation (69) or severe CHD: > 1 cardiopulmonary bypass surgery (60), single ventricle (15), prosthetic valves (13). Anthropometric measurements, blood pressure, and fasting lipid data were compared with summaries from National Health and Nutrition Examination Survey (NHANES) publications (1999-2012). Relative risk (RR) ratios were calculated based on age classification and pooled to obtain overall RR. Of 174 subjects, 106 were male (61%) and 138 (79%) had CHD. Compared to NHANES data, hypertension and low HDL were higher in the study cohort [RR 11.7 (CI 6.34-21.6), p

Bereavement support is a core tenet of palliative care that may prove difficult for clinicians as it is time-consuming, emotionally charged, and not emphasized in pediatrics training. This project is intended to describe the opinions, knowledge, and practice of bereavement care among pediatric oncologists to identify gaps in clinical care.An internet-based survey instrument was pilot tested, refined, and distributed to pediatric oncologists in the United States. Statistical analysis was performed using SAS 9.2.Electronic surveys were distributed to 2,061 pediatric oncologists and 522 surveys (25%) were fully completed. Participants were asked how likely they are to engage in particular bereavement activities (phone calls, condolence cards, memorial services, family meetings, or referrals for counseling) following the death of a pediatric cancer patient. Eighty-two percent of participants, at least, sometimes engage in at least one of these activities. Being female, an attending physician, and increased time in clinical practice were predictive of active participation in bereavement care. Nearly all participants (96%) believe that bereavement care is part of good clinical care, while 8% indicate that bereavement support is not their responsibility. Lack of time and resources were the biggest barriers to providing bereavement support.The majority of pediatric oncologists engage in clinical practices to support bereaved families. Lack of time and physical resources pose significant barriers to clinician's efforts. Additional supports should be explored to increase pediatric oncology physician uptake of bereavement care practices.

Bis-sulfonamide bis-amide TAML activator [Fe{4-NO2 C6 H3 -1,2-(NCOCMe2 NSO2 )2 CHMe}]- (2) catalyzes oxidative degradation of the oxidation-resistant neonicotinoid insecticide, imidacloprid (IMI), by H2 O2 at pH 7 and 25 °C, whereas the tetrakis-amide TAML [Fe{4-NO2 C6 H3 -1,2-(NCOCMe2 NCO)2 CF2 }]- (1), previously regarded as the most catalytically active TAML, is inactive under the same conditions. At ultra-low concentrations of both imidacloprid and 2, 62 % of the insecticide was oxidized in 2 h, at which time the catalyst is inactivated; oxidation resumes on addition of a succeeding aliquot of 2. Acetate and oxamate were detected by ion chromatography, suggesting deep oxidation of imidacloprid. Explored at concentrations [2]≥[IMI], the reaction kinetics revealed unusually low kinetic order in 2 (0.164±0.006), which is observed alongside the first order in imidacloprid and an ascending hyperbolic dependence in [H2 O2 ]. Actual independence of the reaction rate on the catalyst concentration is accounted for in terms of a reversible noncovalent binding between a substrate and a catalyst, which usually results in substrate inhibition when [catalyst]≪[substrate] but explains the zero order in the catalyst when [2]>[IMI]. A plausible mechanism of the TAML-catalyzed oxidations of imidacloprid is briefly discussed. Similar zero-order catalysis is presented for the oxidation of 3-methyl-4-nitrophenol by H2 O2 , catalyzed by the TAML analogue of 1 without a NO2 -group in the aromatic ring.

Sulfidized nanoscale zerovalent iron (SNZVI) has desirable properties for in situ groundwater remediation. However, there is limited understanding of how the sulfidation type and particle properties affect the reactivity and selectivity of SNZVI toward groundwater contaminants, or how reactivity changes as the particles age. Here, SNZVI synthesized by either a one-step (SNZVI-1) or two-step (SNZVI-2) process were characterized, and the reactivity of both fresh and aged (1d to 60 d) nanoparticles was assessed. The measured S/Fe ratio was 5.4 ± 0.5 mol % for SNZVI-1 and 0.8 ± 0.1 mol % for SNZVI-2. XPS analysis indicates S2-, S22-, and S n2- species on the surface of both SNZVI-1 and SNZVI-2, while S22- is the dominant species inside of the SNZVI nanoparticles. SNZVI-1 particles were hydrophobic (contact angle = 103 ± 3°), while the other materials were hydrophilic (contact angles were 18 ± 2° and 36 ± 3° for NZVI and SNZVI-2, respectively). SNZVI-1, with greater S content and hydrophobicity, was less reactive with water than either NZVI or SNZVI-2 over a 60 d period, resulting in less H2 evolution. It also had the highest reactivity with TCE and the lowest reactivity with nitrate, consistent with its higher hydrophobicity. In contrast, both NZVI and SNZVI-2 were reactive with both TCE and nitrate. Both types of SNZVI remained more reactive after aging in water over 60 d than NZVI. These data suggest that the properties of the SNZVI made from a one-step synthesis procedure may provide better reactivity, selectivity, and longevity than that made from a two-step process.

Purpose To characterize the current scope and practices of centers performing extracorporeal cardiopulmonary resuscitation (eCPR) on the undifferentiated patient with cardiac arrest in the emergency department. Methods We contacted all US centers in January 2016 that had submitted adult eCPR cases to the Extracorporeal Life Support Organization (ELSO) registry and surveyed them, querying for programs that had performed eCPR in the Emergency Department (ED ECMO). Our objective was to characterize the following domains of ED ECMO practice: program characteristics, patient selection, devices and techniques, and personnel. Results Among 99 centers queried, 70 responded. Among these, 36 centers performed ED ECMO. Nearly 93% of programs are based at academic/teaching hospitals. 65% of programs are less than 5 years old, and 60% of programs perform ≤3 cases per year. Most programs (90%) had inpatient eCPR or salvage ECMO programs prior to starting ED ECMO programs. The majority of programs do not have formal inclusion and exclusion criteria. Most programs preferentially obtain vascular access via the percutaneous route (70%) and many (40%) use mechanical CPR during cannulation. The most commonly used console is the Maquet Rotaflow ® . Cannulation is most often performed by cardiothoracic (CT) surgery, and nearly all programs (>85%) involve CT surgeons, perfusionists, and pharmacists. Conclusions Over a third of centers that submitted adult eCPR cases to ELSO have performed ED ECMO. These programs are largely based at academic hospitals, new, and have low volumes. They do not have many formal inclusion or exclusion criteria, and devices and techniques are variable.

Purpose To evaluate the interstudy repeatability of multislice quantitative cardiovascular magnetic resonance myocardial blood flow (MBF), myocardial perfusion reserve (MPR), and extracellular volume (ECV). A unique saturation recovery self-gated acquisition was used for the perfusion scans. Materials and Methods An ungated golden angle radial turboFLASH pulse sequence was used to scan 10 subjects on two separate days on a 3T scanner. A single saturation pulse was followed by a set of four slices. Rest and hyperemia scans were acquired during free breathing. The images were reconstructed using an iterative algorithm with spatiotemporal constraints. The ungated images were retrospectively binned (self-gated) into near-systole and near-diastole. Deformable registration was performed to adjust for respiratory and residual cardiac motion, and the data were fit with a Fermi model to estimate the interstudy repeatability of quantitative self-gated MBF and MPR. Results The coefficient of variation (CoV) of the territorial MPR using the self-gated near-systole data was 18.6%. The self-gated near-diastole data gave less good CoV of MPR, equal to 46.2%. For MBFs, and using smaller (segmental) regions, the CoVs were 20.1% and 22.7% for the estimation of myocardial blood flow at stress and rest, respectively, using the self-gated near-systole data. The self-gated near-diastole data gave CoV = 48.6% and 44.9% for stress and rest. Conclusion The self-gated free-breathing technique for quantification of myocardial blood flow showed good repeatability for near-systole, with results comparable to published studies on interstudy repeatability of quantitative myocardial perfusion MRI using ECG-gating and breath-holds. Self-gated near-diastole data results were less repeatable. J. Magn. Reson. Imaging 2015.

Introduction Preliminary evidence in adults with spinal muscular atrophy (SMA) and in SMA animal models suggests exercise has potential benefits in improving or stabilizing muscle strength and motor function.

To compare the Disabilities of the Arm, Shoulder, and Hand (DASH) patient-reported outcome measure as administered by tablet computer to the traditional paper format.In a prospective, randomized study design, 223 consecutive adult patients who presented to the clinic of a single hand surgeon at a tertiary medical center were randomized by visit time to receive the DASH by either paper or tablet computer. Test completeness, time to completion, DASH score, and diagnostic and demographic data were collected and compared between the two cohorts. In total, 120 participants took the DASH using the tablet and 103 using paper.43% of the paper surveys had at least one question that was omitted, compared with 13% in the tablet group; 14% of the paper surveys were not scoreable ( 27 questions answered) compared with 4% of the tablet surveys. The mean time to complete was 3.1 minutes for the paper version of the DASH and 4.3 minutes for the tablet version. Among our study population, there was no influence of age, sex, or diagnosis category on the time required to complete either version of the test. The mean DASH score was 45 for the paper version and 32 for the tablet version.The use of digital data entry methods in the arena of health care outcomes research is increasing. Administration of the DASH via a tablet computer resulted in more complete data, slightly increased responder burden, and a lower DASH score. This finding may have important implications for the use of this metric in an electronic format in future research endeavors.Diagnostic II.

BACKROUND: The structure and purpose of inpatient rounding is widely variable. Patient care teams struggle to balance trainee education with timeliness, efficiency and communication. OBJECTIVE: To evaluate timeliness of morning rounds after implementing a family centered, multi-disciplinary rounding model and effect on intern didactic attendance. DESIGN/METHODS: We changed the rounding structure to a family centered, multi-disciplinary model on one resident medical team in a tertiary care children’s hospital. During our intervention, daily rounds occurred in patient rooms, including the family in discussion and formulation of the care plan. …

Background Erythropoietic protoporphyria (EPP) and X-linked protoporphyria (XLP) are inherited cutaneous porphyrias resulting from decreased activity of ferrochelatase and gain-of-function mutations of δ-aminolevulinic acid synthase-2, respectively. Both of these protoporphyrias cause increased protoporphyrin levels that cause photosensitivity and may lead to hepatopathy and further increases in erythrocyte and plasma porphyrin levels. Methods We evaluated erythrocyte protoporphyrin and plasma porphyrin levels in all subjects with EPP (83 subjects) or XLP (9 subjects) without evidence of liver disease tested repeatedly at a single laboratory over 25 years. Results Intersubject variation contributed more than intrasubject variation (78.86% vs 21.14%) to overall variability, and longitudinal variability, estimated by CV, averaged 26%. Erythrocyte total protoporphyrin levels were similar in males and females with EPP (ratio, 0.99; 95% CI, 0.82–1.21; P = 0.96) but were higher in males than females with XLP, although this difference was not statistically significant (ratio, 0.76; 95% CI, 0.43–1.36; P = 0.35). Analysis of 20 subjects from 9 separate families showed significant effects of family compared with effects of individual variation on total variance (50% vs 25%; P < 0.0001). Conclusion Variation of erythrocyte total protoporphyrin up to 25% is expected in patients with protoporphyria, whereas greater increases might raise concern for protoporphyric hepatopathy.

Purpose The relationship between psychosocial factors and self-reported physical function among hand and upper extremity patients is complex. The Patient-Reported Outcomes Measurement Information System (PROMIS) platform has attempted to create a variety of specifically targeted metrics that can be administered using computer adaptive testing (CAT). Three metrics measuring self-reported physical function (herein referred to in combination as "functional" metrics) include the PROMIS Physical Function (PF) CAT, PROMIS Upper Extremity (UE) CAT, and the Quick Disabilities of the Arm, Shoulder, and Hand ( Quick DASH). Two metrics assessing psychosocial factors include the PROMIS Anxiety and Pain Interference (PI) CATs ("nonfunctional" metrics). This study evaluates whether the functional metrics were correlated with nonfunctional metrics. Methods The 5 questionnaires were administered prospectively on a tablet computer to all consecutive adult patients presenting to an outpatient hand and upper extremity (nonshoulder) clinic at a tertiary academic medical center from January 1 to November 1, 2014. For patients with multiple visits during the study period, only the first was included. Data were evaluated retrospectively to assess the relationship between functional and nonfunctional measures, with Pearson correlation coefficients to understand the relationship between continuous variables, and 1-way analysis of variance to examine for differences in outcome measures across demographic groups. Multivariable linear regression analyses were performed to determine factors predicting functional disability. Results We included 1,299 patients: mean age was 46.8 years, 53% were female, and 23% were unemployed or on disability. The PROMIS PF CAT, PROMIS UE CAT, and Quick DASH scores were all significantly correlated with PROMIS Anxiety CAT (Pearson correlation coefficients, –0.46, –0.48, and 0.53, respectively) and PROMIS PI CAT (–0.60, –0.65, and 0.76, respectively) scores. Multivariable regression analyses demonstrated that increased PROMIS Anxiety and PI CAT scores each independently and adversely influenced PROMIS PF CAT, PROMIS UE CAT, and Quick DASH scores. Conclusions Increasing levels of patient anxiety and pain interference are independently associated with decreased patient-reported upper extremity function. Clinical relevance This study provides further support of the biopsychosocial model by highlighting that increased anxiety is associated with decreased self-reported function using the PROMIS platform.

Pediatric heart transplantation (HT) is resource intensive. Event-driven pediatric databases do not capture data on resource use. The objective of this study was to evaluate resource utilization and identify associated factors during initial hospitalization for pediatric HT. This multicenter retrospective cohort study utilized the Pediatric Health Information Systems database (43 children's hospitals in the United States) of children ≤19 years of age who underwent transplant between January 2007 and July 2013. Demographic variables including site, payer, distance and time to center, clinical pre- and post-transplant variables, mortality, cost, and charge were the data collected. Total length of stay (LOS) and charge for the initial hospitalization were used as surrogates for resource use. Charges were inflation adjusted to 2013 dollars. Of 1,629 subjects, 54% were male, and the median age at HT was 5 years (IQR [interquartile range] 0 to 13). The median total and intensive care unit LOS were 51 (IQR 23 to 98) and 23 (IQR 9 to 58) days, respectively. Total charge and cost for hospitalization were $852,713 ($464,900 to $1,609,300) and $383,600 ($214,900 to $681,000) respectively. Younger age, lower volume center, southern region, and co-morbidities before transplant were associated with higher resource use. In later years, charges increased despite shorter LOS. In conclusion, this large multicenter study provides novel insight into factors associated with resource use in pediatric patients having HT. Peritransplant morbidities are associated with increased cost and LOS. Reducing costs in line with LOS will improve health-care value. Regional and center volume differences need further investigation for optimizing value-based care and efficient use of scarce resources.

Objective. Home O2 has been shown to reduce hospitalizations for bronchiolitis but data on outpatient management of home O2 are lacking. We aim to describe outpatient management and challenges to home O2 for bronchiolitis. Methods. We surveyed Colorado and Utah (where home O2 use is prevalent) chapter members of the American Academy of Pediatrics regarding bronchiolitis home O2 management. Results. A total of 1030 providers were surveyed. The response rate was 21% (n = 214). Ninety percent of practicing primary care providers reported experience with home O2. Of those, 46% see patients on postdischarge day 1. Most providers see patients 1 to 3 times before stopping O2. Eighty percent continue O2 for 3 to 7 days. Weaning procedures vary and 56% practice more than 1 method. Most (41%) do not use continuous pulse oximetry. Challenges include parental noncompliance (51%) and difficulty knowing when to stop the O2 (57%). Conclusions. Management of home O2 in patients with bronchiolitis is a common in UT and CO. Weaning practices vary. Further research is needed.

Objective Despite standardization in care, heterogeneity in outcomes persists for infants with hypoplastic left heart syndrome (HLHS). One potential factor is in utero stressors. Uteroplacental insufficiency (UPI) induces systemic vascular and myocardial adaptations in the absence of structural heart disease. The effect of UPI in HLHS is unknown. Methods We retrospectively analyzed infants undergoing Norwood palliation for HLHS from 2007 to 2012. We compared the umbilical artery systolic to diastolic (SD) ratio to growth outcomes and postoperative right ventricular function. Results Forty three infants met our inclusion criteria. Fetuses without a declining SD ratio with advancing gestational age had asymmetric birth biometry, defined as birth weight minus head circumference z scores (−0.9 vs −0.05, p

Background While there is increasing evidence supporting the choice of subcutaneous ports (SPs) over external venous catheters (EVCs) in pediatric oncology patients, prior conflicting studies exist and little data have been gathered as to which type of central line is preferred from the patient/family perspective. Procedure We performed a single institution, 10 years, retrospective analysis of central lines in pediatric oncology patients (n = 878) to evaluate unplanned early removal and cause of removal while simultaneously obtaining a cross sectional survey of 143 of the primary caretakers/parents of these patients to evaluate their overall satisfaction with the line. Results EVCs have significantly higher odds of unplanned early removal in comparison to SPs (6.7% of SPs vs. 27.3% of EVCs, odds ratio (OR) = 6.3, P

Rationale: Genetic testing for Long QT Syndrome is now a standard and integral component of clinical cardiology. A major obstacle to the interpretation of genetic findings is the lack of robust functional assays to determine the pathogenicity of identified gene variants in a high-throughput manner. Objective: The goal of this study was to design and test a high-throughput in vivo cardiac assay to distinguish between disease-causing and benign KCNH2 ( hERG1) variants, using the zebrafish as a model organism. Methods and Results: We tested the ability of previously characterized Long QT Syndrome hERG1 mutations and polymorphisms to restore normal repolarization in the kcnh2 -knockdown embryonic zebrafish. The cardiac assay correctly identified a benign variant in 9 of 10 cases (negative predictive value 90%), whereas correctly identifying a disease-causing variant in 39/39 cases (positive predictive value 100%). Conclusions: The in vivo zebrafish cardiac assay approaches the accuracy of the current benchmark in vitro assay for the detection of disease-causing mutations, and is far superior in terms of throughput rate. Together with emerging algorithms for interpreting a positive long QT syndrome genetic test, the zebrafish cardiac assay provides an additional tool for the final determination of pathogenicity of gene variants identified in long QT syndrome genetic screening.

Prenatal diagnosis of congenital heart disease (CHD) reportedly increases parental stress compared with postnatal diagnosis. We investigated the association of timing of diagnosis with parental stress and modifiers of this relationship.We enrolled parents with a fetus/infant diagnosed prenatally (Group 1) or postnatally (Group 2) with CHD requiring intervention prior to newborn discharge. Parents completed a Basic Symptom Inventory (BSI) - at diagnosis, birth, and follow-up. Adjusted mixed effects regression models compared scores.The BSI was completed by 105 families (Group 1, n = 60 and Group 2, n = 45). On regression modeling, anxiety and global stress were lower in Group 1 (effect size -0.19 to -0.62) at diagnosis and birth though not at follow-up. When stratified by gender, Group 1 scores for anxiety and stress were primarily lower in fathers. Within Group 1, mothers scored higher in all domains and later gestational age at diagnosis was associated with higher anxiety and stress.Contrary to prior reports, parents of prenatally diagnosed infants with CHD had lower anxiety and stress than those diagnosed postnatally after adjusting for severity. Identifying those most vulnerable and modifiable risk factors will allow us to appropriately target psychosocial services for families with a CHD diagnosis.

Objective Quantification of fetal right ventricular (RV) function by 2D-echocardiography is challenging. Velocity vector imaging (VVI) is an angle independent speckle tracking technique that assesses regional myocardial mechanics. Alteration in the deformation of the fetal RV in hypoplastic left heart syndrome (HLHS) is unknown. This study aimed to evaluate the regional mechanics of the fetal RV in HLHS. Methods We retrospectively analyzed HLHS fetuses imaged at our center. Velocity, strain and strain rate were obtained using VVI. Global and regional parameters of RV deformation were compared between HLHS fetuses and gestational age matched controls. Results A total of 30 HLHS fetuses and 30 gestational age matched controls were analyzed. The mean gestational age was 30.5 ±3.5 weeks. Global myocardial strain was significantly decreased in HLHS RVs compared to controls (−1.9% vs. -4.1%, p = 0.003). The volume of the hypoplastic left ventricle did not affect RV deformation in HLHS. Conclusions Compared to controls, the RV in HLHS fetuses has decreased systolic strain. Prenatal abnormalities in RV myocardial deformation may be responsible for inefficient cardiac performance and output. RV myocardial performance in HLHS can be measured prenatally and may be predictive of post-natal RV dysfunction. © 2012 John Wiley & Sons, Ltd.

Background: In 2000, a 7-valent pneumococcal conjugate vaccine (PCV7) was licensed for use among US children. Many sites have since reported changes in invasive pneumococcal disease (IPD). We recognized an opportunity to describe the changes in epidemiology, clinical syndromes, and serotype distribution during a 14-year period including 4 years before vaccine introduction and spanning the entire PCV7 era. Methods: Cases were defined as children 18 years of age who were cared for at Primary Children’s Medical Center for culture-confirmed IPD. We defined the prevaccine period as the time frame spanning from 1997 to 2000 and the postvaccine period from 2001 to 2010. Demographics, clinical data, and outcomes were collected through electronic query and chart review. Streptococcus pneumoniae serotyping was performed using the capsular swelling method. Results: The median age of children with IPD increased from 19 months during the prevaccine period to 27 months during postvaccine period (P 0.02), with a larger proportion of IPD among children older than 5 years. The proportion of IPD associated with pneumonia increased substantially from 29% to 50% (P 0.001). This increase was primarily attributable to an increase in complicated pneumonia (17% to 33%, P 0.001). Nonvaccine serotypes 7F, 19A, 22F, and 3 emerged as the dominant serotypes in the postvaccine period. In children with IPD who were younger than 5 years, for whom vaccine is recommended, 67% of the cases were caused by serotypes in 13-valent PCV during 2005 to 2010. Conclusions: After PCV7 was introduced, significant changes in IPD were noted. One-third of IPD occurred in children older than 5 years, who were outside the age-group for which PCV is recommended. Continued surveillance is warranted to identify further evolution of the epidemiology, clinical syndromes, and serotype distribution of S. pneumoniae after 13-valent PCV licensure.

Disorders of the Ras/mitogen-activated protein kinase (MAPK) pathway have an overlapping skeletal phenotype (e.g. scoliosis, osteopenia). The Ras proteins regulate cell proliferation and differentiation and neurofibromatosis type 1 (NF1) individuals have osteoclast hyperactivity and increased bone resorption as measured by urine pyridinium crosslinks [pyridinoline (Pyd) and deoxypyridinoline (Dpd)]. Pyd and Dpd are hydroxylysine-derived crosslinks of collagen found in bone and cartilage and excreted in the urine. Dpd is most abundant in bone. The aim of this study was to evaluate if other syndromes of the Ras/MAPK pathway have increased bone resorption, which may impact the skeletal phenotype. Participants were individuals with Noonan syndrome (n = 14), Costello syndrome (n = 21), and cardiofaciocutaneous (CFC) syndrome (n = 14). Pyridinium crosslinks from two consecutive first morning urines were extracted after acid hydrolysis and analyzed by high performance liquid chromatography. Three separate analyses of covariance were performed to compare Pyd, Dpd, and Dpd/Pyd ratio of each group to controls after controlling for age. Data were compared to 99 healthy controls. The Dpd and the Dpd/Pyd ratio were elevated (p < 0.0001) in all three conditions compared to controls suggesting that collagen degradation was predominantly from bone. The data suggest that the Ras/MAPK signal transduction pathway is important in bone homeostasis.

TOC Summary: The infection disproportionately affected patients >65 years of age., Since the introduction of the Haemophilus influenzae type b vaccine, the incidence of invasive H. influenzae type b disease among children has fallen dramatically, but the effect on invasive H. influenzae disease among adults may be more complex. In this population-based study we examined the epidemiology and outcomes of invasive disease caused by typeable and nontypeable H. influenzae among Utah adults during 1998–2008. The overall incidence increased over the study period from 0.14/100,000 person-years in 1998 to 1.61/100,000 person-years in 2008. The average incidence in persons >65 years old was 2.74/100,000 person-years, accounting for 51% of cases and 67% of deaths. The incidence was highest for nontypeable H. influenzae (0.23/100,000 person-years), followed by H. influenzae type f (0.14/100,000 person-years). The case-fatality rate was 22%. The incidence of invasive H. influenzae in Utah adults appears to be increasing. Invasive H. influenzae infection disproportionately affected the elderly and was associated with a high mortality rate.

Identify parental beliefs and barriers related to influenza immunization of school-aged children and acceptance of school-based influenza immunization.We conducted a cross-sectional survey of parents of elementary school-aged children in November 2008. Outcomes were receipt of influenza vaccine, acceptance of school-based immunization, and barriers to immunization.Response rate was 65% (259/397). Parents reported that 26% of children had received the vaccine and 24% intended receipt. A total of 50% did not plan to immunize. Factors associated with receipt were belief that immunization is a social norm (adjusted odds ratios [AOR], 10.8; 95% CI, 2.8-41.8), belief in benefit (AOR, 7.8; CI, 1.8-33.8), discussion with a doctor (AOR, 7.0; CI, 2.9-16.8), and belief that vaccine is safe (AOR, 4.0; CI, 1.0-15.8). A total of 75% of parents would immunize their children at school if the vaccine were free, including 59% (76/129) who did not plan to immunize. Factors associated with acceptance of school-based immunization were belief in benefit (AOR, 6.1; 95% CI, 2.7-14.0), endorsement of medical setting barriers (AOR, 3.7; 95% CI, 1.3-10.3), and beliefs that immunization is a social norm (AOR, 3.3; 95% CI, 1.4-7.6) and that the child is susceptible to influenza (AOR, 2.6; 95% CI, 1.2-5.7). Medical setting barriers were competing time demands, inconvenience, and cost; school barriers were parents' desire to be with children and competence of person delivering the vaccine.School-based immunization programs can increase immunization coverage by targeting parents for whom time demands and inconvenience are barriers, demonstrating that immunization is a social norm, and addressing concerns about influenza vaccine benefit and safety.

Background: Pediatric heart transplantation (HT) is resource intensive. Event driven pediatric HT databases do not capture data on resource use. The objective of this study was to evaluate resource utilization and identify associated factors during initial hospitalization for pediatric HT in a large multi-institutional cohort. Methods: This is a multicenter retrospective cohort study using the PHIS database (43 US children’s hospitals) of children ≤ 19 years of age who underwent HT between 1/07 and 7/13. Data collected: Demographic variables including site, payer, distance and time to center, clinical pre and post-transplant variables, mortality, cost and charge. Total length of stay (LOS) and charge for initial hospitalization were used as surrogates for resource use. Charges were inflation adjusted to 2013 $. Gamma regression analysis was performed to evaluate factors associated with resource use. Results: Of 1629 subjects, 54% were male, and the median age at HT was 5 years (IQR 0-13). The median total and ICU LOS were 51 (IQR: 23-98) and 23 (IQR: 9-58) days respectively, and mortality occurred in 82 (5%). Total charge and cost for hospitalization were $852,713 ($464,900-$1,609,300) and $383,600 ($214,900-$681,000) respectively. Factors associated with resource use on multivariate analysis are shown in Table 1. Younger age, lower center volume, southern region, and comorbidities prior to HT were associated with higher resource use. In later years, costs increased despite shorter LOS. Conclusions: This large multicenter study provides novel insight into factors associated with resource use in pediatric HT that cannot be assessed in alternative event driven transplant databases. Peri-transplant morbidities are associated with increased cost and LOS. Reducing costs in line with LOS will improve health care value. Regional and center volume differences need further investigation for optimizing value-based care and efficient use of scarce resources.

To evaluate the interstudy repeatability of multislice quantitative cardiovascular magnetic resonance myocardial blood flow (MBF), myocardial perfusion reserve (MPR), and extracellular volume (ECV). A unique saturation recovery self-gated acquisition was used for the perfusion scans.An ungated golden angle radial turboFLASH pulse sequence was used to scan 10 subjects on two separate days on a 3T scanner. A single saturation pulse was followed by a set of four slices. Rest and hyperemia scans were acquired during free breathing. The images were reconstructed using an iterative algorithm with spatiotemporal constraints. The ungated images were retrospectively binned (self-gated) into near-systole and near-diastole. Deformable registration was performed to adjust for respiratory and residual cardiac motion, and the data were fit with a Fermi model to estimate the interstudy repeatability of quantitative self-gated MBF and MPR.The coefficient of variation (CoV) of the territorial MPR using the self-gated near-systole data was 18.6%. The self-gated near-diastole data gave less good CoV of MPR, equal to 46.2%. For MBFs, and using smaller (segmental) regions, the CoVs were 20.1% and 22.7% for the estimation of myocardial blood flow at stress and rest, respectively, using the self-gated near-systole data. The self-gated near-diastole data gave CoV = 48.6% and 44.9% for stress and rest.The self-gated free-breathing technique for quantification of myocardial blood flow showed good repeatability for near-systole, with results comparable to published studies on interstudy repeatability of quantitative myocardial perfusion MRI using ECG-gating and breath-holds. Self-gated near-diastole data results were less repeatable. J. Magn. Reson. Imaging 2016;43:1369-1378.

Studies report conflicting associations between preeclampsia and retinopathy of prematurity (ROP). This study provides explanations for the discrepancies to clarify the relationship between preeclampsia and ROP.To evaluate the association of maternal preeclampsia and risk of ROP among infants in an unrestricted birth cohort and a restricted subcohort of preterm, very low birth weight (P-VLBW) infants.A retrospective review of 290 992 live births within the Intermountain Healthcare System in Utah from January 1, 2001, through December 31, 2010, was performed. Generalized estimating equations for logistic regressions with covariate adjustment were applied to relate ROP to preeclampsia among the full cohort and in a subcohort of P-VLBW infants born at younger than 31 weeks' gestation and weighing less than 1500 g.The occurrence of ROP was related to maternal preeclampsia in the full cohort and in a subcohort of P-VLBW infants.In the full cohort, 51% of the infants were male and the mean (SD) gestational age was 38.38 (1.87) weeks. In the P-VLBW cohort, 55% were male and the mean (SD) gestational age was 26.87 (2.40) weeks. In the full cohort, preeclampsia was associated with an increased risk of all ROP (adjusted odds ratio [aOR], 2.46; 95% CI, 2.17-2.79; P .001), severe ROP (aOR, 5.21; 95% CI, 3.44-7.91; P .001), infant death (aOR, 1.66; 95% CI, 1.16-2.38; P = .006), and giving birth to a P-VLBW infant (aOR, 7.74; 95% CI, 6.92-8.67; P .001). In the P-VLBW subcohort, preeclampsia was inversely associated with the development of all ROP (aOR, 0.79; 95% CI, 0.68-0.92; P = .003), severe ROP (aOR, 0.62; 95% CI, 0.36-1.06; P = .08), and infant death (aOR, 0.19; 95% CI, 0.11-0.32; P .001).Preeclampsia was associated with an increased risk of developing ROP among an unrestricted cohort but with a reduced risk of ROP among a restricted subcohort of P-VLBW infants. Although the conflicting associations in the full and P-VLBW cohorts may reflect true differences, the association of a reduced risk of ROP among the P-VLBW subcohort also may reflect biases from restricting the cohort to prematurity, because prematurity is an outcome of preeclampsia.

Social media has two essential building blocks: content and people. These two components form a heterogeneous network with various types of relations: people produce and share content, various content items are related to each other, and people have social relations such as collaboration and discussion. Identifying people’s expertise and topics is the first step in evaluating the quality of information in a network. Text semantic analysis and social network analysis address this issue from different perspectives. Text analysis aims at constructing concept similarity networks based on documents and keywords within the documents. Linguistics or keyword co-occurrence is used to detect word associations. Social network analysis utilizes network structures to find prestigious individuals who connect with other experts in a relational network. This study proposes a three-layer framework to integrate the semantic and social networks in order to reveal people’s expertise based on the words they use and their relations. Using social tagging activities on CiteUlike as an example, we illustrate how social relations help identify similar concepts in semantic networks.

Evaluate the effect of duty hour regulation on graduating otolaryngology resident surgical case volume and analyze trends in surgical case volume for Accreditation Council for Graduate Medical Education (ACGME) key indicator cases from 1996 to 2011.Time-trend analysis of surgical case volume.Nationwide sample of otolaryngology residency programs.Operative logs from the American Board of Otolaryngology and ACGME for otolaryngology residents graduating in the years 1996 to 2011.Key indicator volumes and grouped domain volumes before and after resident duty hour regulations (2003) were calculated and compared. Independent t test was performed to evaluate overall difference in operative volume. Wilcoxon rank sum test evaluated differences between procedures per time period. Linear regression evaluated trend.The average total number of key indicator cases per graduating resident was 440.8 in 1996-2003 compared to 500.4 cases in 2004-2011, and overall average per number of key indicators was 31.5 and 36.2, respectively (P = .067). Four key indicator cases showed statistically significant (P.05) increases in volume after duty hour implementation. General/pediatrics was the only grouped domain to show a significant increase. In contrast, the rate of change in operative volume decreased post duty hour for only 2 key indicators (P.05). The year-by-year trend in average operative volume showed significant increases for 5 key indicator cases (P.05).Implementation of the 2003 duty hour regulations has not reduced total volume of key indicator cases for graduating otolaryngology residents. The overall trend in operative volume is increasing for several specific key indicators.

While there is increasing evidence supporting the choice of subcutaneous ports (SPs) over external venous catheters (EVCs) in pediatric oncology patients, prior conflicting studies exist and little data have been gathered as to which type of central line is preferred from the patient/family perspective.We performed a single institution, 10 years, retrospective analysis of central lines in pediatric oncology patients (n = 878) to evaluate unplanned early removal and cause of removal while simultaneously obtaining a cross sectional survey of 143 of the primary caretakers/parents of these patients to evaluate their overall satisfaction with the line.EVCs have significantly higher odds of unplanned early removal in comparison to SPs (6.7% of SPs vs. 27.3% of EVCs, odds ratio (OR) = 6.3, P 0.0001 when controlling for age and diagnosis) secondary to increased infection, malfunction and patient preference. Patients with SPs felt like their central line was easier to care for, had less daily impact in their life, and were overall more satisfied with their central line compared to patients with EVCs, even when controlling for early removal (P 0.0001 for all). SP patients were much more likely to state that they would choose the same type of line again (OR = 15, P 0.0001) than EVC patients.SPs demonstrated lower removal rates and greater patient satisfaction than EVCs. These data should be considered when choosing a central line for pediatric cancer patients.