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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

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.

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

Social question and answer sites (SQAs) are increasingly popular knowledge sharing platforms. In this paper, we outline how an SQA site functions as a social learning community. The success of an SQA site depends not only on effectively organizing and delivering information, but also on whether it can provide the cues needed by community members to successfully learn to be productive contributors. We explore this learning process in four different SQAs that utilize the Stack Exchange platform: Science Fiction & Fantasy, Seasoned Advice (cooking), Database Administrators, and Android Enthusiasts. Using longitudinal fixed effects models, we examine whether users learn to be better question askers over time, and how user interface features and community norms affect the cultivation of this critical skill. The study offers design implications by highlighting factors that help users develop into literate and productive community contributors in social platforms.

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.

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.