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Children with perinatal arterial ischemic stroke (PAIS) have increased rates of attention and executive functioning (EF) weaknesses. Research in other pediatric disorders has documented poor consistency between parent report of these skills and performance-based measures. We compared these data sources in children with PAIS. Forty full-term (≥37 weeks) children ages 3–16 (median = 7.2 years; 58% male) with PAIS completed neuropsychological testing and composite scores were created for seven attention and EF domains (Processing Speed; Attention; Working Memory; Verbal Retrieval; Inhibitory Control; Flexibility/Shifting; Planning). Parents completed "real-world" functioning questionnaires (ADHD Rating Scale-IV, BRIEF). Correlational analysis were used to compare parent and performance measures. Correlations between ADHD Rating Scale-IV scores and the performance-based Attention and Inhibition composite scores were nonsignificant. Significant negative correlations were found between the BRIEF GEC and performance-based Verbal Retrieval and Processing Speed composites, but remaining GEC/composite comparisons were nonsignificant. Analyses between parent report BRIEF index scores and the corresponding performance-based domain identified one significant negative correlation between the BRIEF Working Memory Index and the Working Memory composite score. While children with PAIS demonstrate difficulties in attention and EF on both parent report and performance measures, little significance was found in comparisons of these two types of measures. There may be several explanations for this dissociation: measures assessing different aspects of the same underlying construct; performance-based measures lacking ecological validity; and parents underestimating/underreporting their child's deficits. Thus, multiple sources of informant and performance data are necessary to make more accurate conclusions about functioning in these domains. [ABSTRACT FROM AUTHOR]

Perinatal arterial ischemic stroke (PAIS) is a form of childhood stroke; the majority of those affected experience neurologic sequelae, including motor, language and neurocognitive impairments. This study examines the attention and executive functioning (EF) profiles of children following PAIS, as well as the impact of age and sex. In this single-center cross-sectional study, 40 children aged 3 to 16 years (median age 7.2 years; 58% male) who have suffered a PAIS underwent a comprehensive neuropsychological battery to assess attention and EF. Parents completed behavioral questionnaires regarding real-world functioning. Composite scores were calculated for seven attention and EF domains (Attention, Working Memory, Verbal Retrieval, Inhibitory Control, Flexibility/Shifting, Planning/Organization, and Processing Speed). The results for all measured domains of attention and EF are significantly lower in the participants compared to the normative samples (p < .001), with the exception of Working Memory. However, increasing difficulty with Working Memory is associated with developing age. Older age at time of testing is also associated with a higher incidence of clinically-elevated attention deficit hyperactivity disorder (ADHD) symptoms. Sex is not associated with performance measures or parental report of functioning. The participants demonstrate mild-to-moderate attention and EF impairment compared to the normative population. Clinicians, families, and educators should be informed about the neurocognitive sequelae of PAIS and the need for close developmental surveillance in this population to identify vulnerable children and initiate appropriate therapeutic interventions in a timely fashion. [ABSTRACT FROM PUBLISHER]

Background: Children with perinatal arterial ischemic stroke (PAIS) are at risk for later neurocognitive and behavioral deficits, yet the clinical predictors of these outcomes are understudied. We examined the influence of clinical and infarct characteristics on attention and executive functioning in children following PAIS.Methods: Forty children born at term (≥37 weeks' gestation) with PAIS (28 with neonatal arterial ischemic stroke and 12 with presumed PAIS) underwent a comprehensive neuropsychological battery at age three to 16 years (median age 7.2 years; 58% male) to assess attention and executive functioning. Parents also completed questionnaires regarding real-world functioning. Clinical variables including perinatal stroke subtype, infarct characteristics (location, laterality, and volume), and the presence of comorbid epilepsy were ascertained from the medical record.Results: Presumed PAIS, larger infarct volume, and comorbid epilepsy negatively influenced the performance on attention and executive functioning measures. These clinical variables were also associated with greater functional problems on parent reports, including a higher frequency of attention-deficit/hyperactivity disorder symptoms and greater difficulties in some subdomains of executive functioning. Infarct location and laterality were not associated with performance measures or parental report of functioning.Conclusion: Although all children with PAIS are at risk for later deficits in attention and executive functioning, those with presumed PAIS, larger infarct size, and comorbid epilepsy appear to be the most vulnerable. As they approach and reach school age, these children should undergo neuropsychological assessment to ensure timely implementation of therapeutic interventions and behavioral strategies. [ABSTRACT FROM AUTHOR]

The objective of the study was to compare executive functioning (EF) profiles across several pediatric medical conditions and explore the influence of age of diagnosis and evaluation. A retrospective, cross-sectional study of 734 children aged 5 to 18 years was conducted across five medical groups (brain tumor, leukemia [ALL], epilepsy [EPI], neurofibromatosis type 1 [NF1], and ornithine transcarbamylase deficiency [OTC-D]), attention deficit hyperactivity disorder (ADHD) controls, and matched healthy controls. We compared groups across the scales of a parent-completed Behavior Rating Inventory of Executive Functioning (BRIEF) using a repeated measures analysis of variance (ANOVA). Separate ANOVAs were conducted to look at age factors. The results showed that the ADHD group differed from all other groups and had the highest level of reported EF problems. The NF1 and OTC-D groups differed significantly from the healthy comparison group for overall EF problems, while the EPI and cancer groups did not. Working memory was the most elevated scale across medical groups, followed by plan/organize. Children with medical disorders were two to four times more likely than healthy controls to have clinically significant problems in several EF domains. There was a main effect for age at diagnosis and age at evaluation. A subset of children with medical disorders were found to have parent-reported EF difficulties, with particular vulnerability noted in working memory and organizational/planning skills. This has relevance for the development of interventions that may be helpful across disorders. Children with particular diagnoses and earlier age of diagnosis and evaluation had greater reported EF problems. [ABSTRACT FROM AUTHOR]

Atenolol/Nifedipine Combination: Efficacy and Tolerability of Low Dose Synergistic Bitherapy for the Treatment of Arterial Hypertension During recent decades, undeniable progress has been made with regard to the management of arterial hypertension. Larger numbers of patients are aware they have hypertension, receive treatment and benefit from this therapy. Furthermore, significant reductions have been observed in morbidity and mortality resulting from cardiovascular diseases. The objectives of hypertension treatment have been formulated on the basis of results of extensive epidemiological studies. Only a few patients receiving monotherapy actually achieve and maintain acceptable blood pressure levels. The complex pathogenesis of essential hypertension, the implications of nervous and humoral counter-regulatory effects, the heterogeneous character of individual responses to any given class of antihypertensive treatment and the onset of adverse effects all account for these failures. The search for a simple, effective and well-tolerated treatment based on a low dose combination of 2 classes of antihypertensive agents is consequently legitimate. The fixed combination of atenolol 50mg and sustained release nifedipine 20mg enables patients to benefit from the antihypertensive synergy of a beta-blocker and a calcium antagonist (dihydropyridine). Several open-ended or double-blind, controlled studies have shown that this combination produces a more marked antihypertensive effect than the individual components used alone or other reference monotherapies. Furthermore, it has been shown that this effect persists throughout the entire 24-hour period; this has been confirmed by 24-hour blood pressure monitoring. Short and medium term tolerability is significantly improved: the side effects commonly associated with the 2 drugs when used alone are reduced with the combination formulation since the 2 active substances have different and complementary mechanisms of action. In addition, long term studies have shown that therapeutic efficacy and tolerability remain stable and have even been seen to improve over a 12-month period. The fixed combination of atenolol-nifedipine has a role in strategies for the treatment of mild to moderate hypertension, particularly under the following conditions: - when first-line monotherapy has failed to attain specific clearly defined objectives, including stabilised blood pressure levels together with acceptable tolerability when patient compliance is jeopardised as a result of undesirable side effects when the vascular burden is aggravated through lack of attention to individual risk factors in hypertensive patients. In more serious forms of hypertension, the atenolol-nifedipine combination can replace sequential monotherapies or other combination treatments that have failed to comply with the various criteria of therapeutic efficacy. Controlling arterial hypertension commonly requires polytherapy with 3 or even 4 different drugs in conjunction with particularly strict rules governing hygiene and diet. The addition of the fixed combination of atenolol-nifedipine simplifies the treatment of patients with arterial hypertension by limiting the daily doses and reducing laboratory monitoring. Resume Les objectifs du traitement de lhypertension arterielle (HTA) ont ete precises dapres les resultats des grandes enquetes epidemiologiques. Seule une minorite de patients sous monotherapie atteint et se maintient a un niveau souhaitable de pression arterielle. La pathogenie complexe de lHTA essentielle, la mise en jeu de contre-regulations nerveuses et humorales, lheterogeneite de la reponse individuelle a une classe donnee dantihypertenseurs, et la survenue deffets secondaires, expliquent ces echecs et justifient la recherche dune therapeutique simple, efficace et bien toleree par lassociation de deux classes danti-hypertenseurs a dose faible. Lassociation fixe datenolol 50mg a la nifedipine a liberation prolongee 20mg (A50/N20) permet de beneficier de leffet anti-hypertenseur synergique dun beta-bloquant et dun inhibiteur calcique (dihydropyridine). Plusieurs etudes en ouvert ou controlees en double aveugle ont montre que lassociation possedait un effet antihypertenseur plus marque que ses composants separes ou que dautres monotherapies de reference; que leffet se maintenait au long du nycthemere, notamment au cours des mesures ambulatoires sur 24 heures; que les effets indesirables habituellement observes avec chacune des monotherapies etaient diminues avec lassociation de ces deux principes actifs ayant des modes daction differents et complementaires; enfin que lefficacite therapeutique et la tolerance se maintenaient ou samelioraient lors detudes a long terme menees sur 12 mois. Lassociation A50/N20 sinscrit dans la strategie therapeutique de lHTA legere a moderee des lors quune monotherapie de premiere intention ne repond pas aux criteres stricts. [ABSTRACT FROM AUTHOR]

Although the singular value decomposition (SVD) and proper orthogonal decomposition have been widely used in fluid mechanics, Venturi (J Fluid Mech 559:215–254, 2006) and Epps and Techet (Exp Fluids 48:355–367, 2010) were among the first to consider how noise in the data affects the results of these decompositions. Herein, we extend those studies using perturbation theory to derive formulae for the 95% confidence intervals of the singular values and vectors, as well as formulae for the root mean square error (rmse) of each noisy SVD mode. Moreover, we show that the rmse is well approximated by ϵ / s ~ k (where ϵ is the rms noise and s ~ k is the singular value), which provides a useful estimate of the overall uncertainty in each mode. [ABSTRACT FROM AUTHOR]

The singular value decomposition (SVD) and proper orthogonal decomposition are widely used to decompose velocity field data into spatiotemporal modes. For noisy experimental data, the lower SVD modes remain relatively clean, which suggests the possibility for data filtering by retaining only the lower modes. Herein, we provide a method to (1) estimate the noise level in a given noisy dataset, (2) estimate the root mean square error (rmse) of the SVD modes, and (3) filter the noise using only the SVD modes that have low enough rmse. We show through both analytic and PIV examples that this method yields nearly the most accurate possible SVD-based reconstruction of the clean data. Moreover, we provide an analytic estimate of the accuracy of this reconstruction. [ABSTRACT FROM AUTHOR]

Objective: Children with a history of stroke often experience neurocognitive and behavioral deficits across multiple domains, yet the added impact of epilepsy remains largely understudied. We examined the effect of comorbid epilepsy on intellectual and executive functioning in pediatric stroke. Method: Participant variables, IQ data, and parent questionnaires (BRIEF) were retrospectively collected from 20 children (n = 10 stroke with epilepsy, “S + E,” n = 10 stroke without epilepsy, “S–E,” matched for gender, age, and stroke type). Inclusion criteria were: history of overt stroke due to a primary vascular cause/disease. Medical records were reviewed and test data were extracted from clinical reports. Results: Intellectual abilities were significantly lower in the S + E group (mean FSIQ = 71, 40% with Intellectual Disability [ID]) compared to the S–E group (mean FSIQ = 91, 0% with ID). Children with comorbid epilepsy demonstrated greater reported problems in nearly all aspects of executive functioning; however, results were not significant. Behavioral regulation skills were notably more problematic for the S + E group (OR = 8.8 for T >60 on BRIEF BRI). The S + E group also had a slightly higher incidence of ADHD (30% vs. 20%). Conclusion(s): Children with comorbid epilepsy demonstrated greater weaknesses in intelligence and aspects of executive functioning, particularly behavioral regulation problems. Additionally, these children were at higher risk for diagnoses of ADHD and ID than those without comorbid epilepsy. Future research examining other variables that predict poor neurocognitive and behavioral outcomes is crucial for assessing pediatric stroke risk factors and designing interventions. [ABSTRACT FROM AUTHOR]

Children with perinatal stroke are at increased risk for developmental language disorders, learning difficulties, and other mental health conditions. However to date, autism (ASD) prevalence in this group has not been reported. Given that early identification of ASD is essential to promoting optimal outcomes, our goal was to establish prevalence of ASD in children with perinatal stroke. A prospectively enrolled, single-center stroke registry maintained at our institution since 2005 was queried for all potentially eligible patients with a history of perinatal stroke. Information regarding stroke features, ASD diagnosis/concern, intellectual disability/global developmental delay, cerebral palsy/hemiparesis, epilepsy, and language disorder were collected via retrospective chart review from electronic health records. 311 children were identified, of which 201 complete records were analyzed. Twenty-three cases were formally diagnosed with ASD (11.4%). First concerns were noted in toddlerhood (Mage = 2.66 years), yet the average age of diagnosis was 6.26 years. Children with ASD were more likely to have earlier diagnoses of intellectual disability/global developmental delay or a mixed receptive-expressive language disorder (ps <.001) but did not differ on CP/hemiplegia or epilepsy diagnoses. Risk for ASD increased with accumulating diagnoses. Children with perinatal stroke have an increased prevalence of ASD (11.4%) than in the general population. ASD concerns arise at a similar age as the general population, yet ASD is diagnosed almost two years later than the general population and 3.60 years after first concerns present. Co-occurring neurological conditions are common. Clinicians must be aware of increased prevalence and implement screening as part of routine care for all pediatric patients with perinatal stroke. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented concerning cases of vasculitis with cryoglobulinemia following vaccination against hepatitis B.

Background Safety of HPV vaccines is still in question due to reports of autoimmune diseases (ADs) following HPV immunization. Objectives To assess the risk of ADs associated with HPV vaccination of female adolescents/young adults in France. Methods Systematic prospective case-referent study conducted to assess the risks associated with real-life use of HPV vaccines. Cases were female 11–25 years old with incident ADs [central demyelination/multiple sclerosis (CD/MS), connective tissue disease (CTD), Guillain-Barré syndrome (GBS), type-1 diabetes (T1D), autoimmune thyroiditis (AT), and idiopathic thrombocytopenic purpura (ITP)]. Cases were consecutively and prospectively identified at specialized centers across France (2008–2014) and individually matched by age and place of residence to referents recruited in general practice. Risk was computed using multivariate conditional logistic regression models adjusted for family history of ADs, living in France (north/south), co-medications and co-vaccinations. Results With a total of 478 definite cases matched to 1869 referents, all ADs combined were negatively associated to HPV vaccination with an adjusted odds ratio of 0.58 (95% confidence interval: 0.41–0.83). Similar results were obtained for CD/MS, AT, CT, and T1D, the last two not reaching statistical significance. No association was found for ITP and GBS. Sensitivity analyses combining definite and possible cases with secondary time window showed similar results. Conclusion Exposure to HPV vaccines was not associated with an increased risk of ADs within the time period studied. Results were robust to case definitions and time windows of exposure. Continued active surveillance is needed to confirm this finding for individual ADs. [ABSTRACT FROM AUTHOR]

This study examined the effect of traumatic brain injury (TBI) in young children on sleep problems and the relationship of sleep problems to neuropsychological and psychosocial functioning. Participants were drawn from an ongoing longitudinal study of injury in young children recruited from 3 to 6 years of age. They constituted three groups: orthopedic injury (OI; n=92), complicated mild/moderate TBI (mTBI; n=55); and severe TBI (sTBI; n=20). Caregivers completed the Children's Sleep Habits Questionnaire (CSHQ), as well as ratings of behavioral adjustment, adaptive functioning, and everyday executive function at 1, 6, 12, and 18 months postinjury. Retrospective ratings of preinjury sleep and psychosocial functioning were obtained at the initial assessment. Children completed neuropsychological testing at all occasions. Children with complicated mTBI demonstrated more total sleep problems than children with OI at 6 months postinjury, but not at 12 or 18 months. Children with sTBI displayed more bedtime resistance and shorter sleep duration than those with complicated mTBI or OI at several occasions. Across groups, total sleep problems predicted more emotional and behavioral problems and worse everyday executive function as rated by parents across follow-up occasions. In contrast, sleep problems were generally not related to neuropsychological test performance. The results suggest that young children with TBI demonstrate more sleep problems than children with injuries not involving the head. Sleep problems, in turn, significantly increase the risk of poor psychosocial outcomes across time, but are not associated with worse neuropsychological test performance. [ABSTRACT FROM AUTHOR]

Abstract: The Urea Cycle Disorders Consortium (UCDC) was created as part of a larger network established by the National Institutes of Health to study rare diseases. This paper reviews the UCDC’s accomplishments over the first 6years, including how the Consortium was developed and organized, clinical research studies initiated, and the importance of creating partnerships with patient advocacy groups, philanthropic foundations and biotech and pharmaceutical companies. [Copyright &y& Elsevier]

Abstract: The Research Challenges in CNS Manifestations of Inborn Errors of Metabolism workshop was designed to address challenges in translating potential therapies for these rare disorders, and to highlight novel therapeutic strategies and innovative approaches to CNS delivery, assessment of effects and directions for the future in the treatment of these diseases. Therapies for the brain in inborn errors represent some of the greatest challenges to translational research due to the special properties of the brain, and of inborn errors themselves. This review covers the proceedings of this workshop as submitted by participants. Scientific, ethical and regulatory issues are discussed, along with ways to measure outcomes and the conduct of clinical trials. Participants included regulatory and funding agencies, clinicians, scientists, industry and advocacy groups. [Copyright &y& Elsevier]

Abstract: In this paper, turbocharger centrifugal compressors with dual volute design were investigated by using Computational Fluid Dynamics (CFD) method. The numerical simulation focused on the air flow from compressor impeller inlet to volute exit, and the overall performance level and range are predicted. The numerical investigation revealed that the dual volute design could separate the compressor into two operating regions: “high efficiency” and “low efficiency” regions with different air flow characteristics, and treating these two regions separately with dual diffuser design showed extended stable operating range and improved efficiency by comparing with conventional single volute design. The “dual sequential volute” concept also showed the potential to further extend the stable operating range by closing one of the volutes at low air flow rates. Furthermore, by comparing with other alternate designs such as variable diffuser vanes and variable inlet guide vanes, the operation of the dual sequential volute also features relatively simple control and calibration. [Copyright &y& Elsevier]

BACKGROUNDMedullary thyroid carcinoma is a rare tumour derived from the thyroid parafollicular calcitonin-secreting cells. Calcitonin is a very specific marker of this cancer that allows preoperative diagnosis. Serum calcitonin assay is particularly useful to define the postoperative state of patients (cured, apparently cured, not cured) and, because of its great sensitivity, it has a major place in the postoperative follow-up. OBJECTIVETo identify, among patients thyroidectomized for medullary thyroid carcinoma (MTC), the characteristics of authentic recurrent MTC [re-elevation of stimulated serum calcitonin (CT) level measured by a sensitive immunoradiometric assay, after postoperative normalization]. PATIENTS AND METHODSWe first collected, through the national registry of the French Calcitonin Tumour Study Group (GETC), patients who had undergone a total thyroidectomy with or without lymph node surgery and who were not cured at the last follow-up visit. Among 453 such patients included in the database, 15 patients met the criteria for authentic recurrence as defined in previous studies: they had been first considered as cured during the 6 months following the initial surgical procedure (basal and pentagastrin-stimulated serum calcitonin level ≤ 10 ng/l) but had a secondary rerise of their pentagastrin-stimulated calcitonin level, over 10 ng/l. Eight of them even had an undetectable (< 2 ng/l) immediate postoperative CT, confirming the initial postoperative cure. The characteristics (age, nature of disease, stage at surgery and type of node dissection) of these 15 patients were studied. RESULTSAccording to the Tumour Node Metastasis classification, nine patients were T1 and, among them, five patients had had complete lymph node surgery without any evidence of nodal metastases (N0). The recurrence was 3·2 ± 2·2 years (range: 0·7–7·5) after the initial surgery. CONCLUSIONS3·3% of patients not cured at the last... [ABSTRACT FROM AUTHOR]