715 research outputs found

    Comparison of calculated and measured pressures on straight and swept-tip model rotor blades

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    Using the quasi-steady, full potential code, ROT22, pressures were calculated on straight and swept tip model helicopter rotor blades at advance ratios of 0.40 and 0.45, and into the transonic tip speed range. The calculated pressures were compared with values measured in the tip regions of the model blades. Good agreement was found over a wide range of azimuth angles when the shocks on the blade were not too strong. However, strong shocks persisted longer than predicted by ROT22 when the blade was in the second quadrant. Since the unsteady flow effects present at high advance ratios primarily affect shock waves, the underprediction of shock strengths is attributed to the simplifying, quasi-steady, assumption made in ROT22

    Risk factors for mortality in adult patients with sickle cell disease: a meta-analysis of studies in North America and Europe

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    Although recent studies show an improved survival of children with sickle cell disease in the US and Europe, for adult patients mortality remains high. This study was conducted to evaluate the factors associated with mortality in adult patients following the approval of hydroxyurea. We first evaluated the association between selected variables and mortality at an academic center (University of North Carolina). Data sources were then searched for publications from 1998 to June 2016, with meta-analysis of eligible studies conducted in North America and Europe to evaluate the associations of selected variables with mortality in adult patients. Nine studies, combined with the UNC cohort (total n=3257 patients) met the eligibility criteria. Mortality was significantly associated with age (per 10-year increase in age) [7 studies, 2306 participants; hazard ratio (HR): 1.28; 95% confidence interval (CI): 1.10–1.50], tricuspid regurgitant jet velocity 2.5 m/s or more (5 studies, 1577 participants; HR: 3.03; 95%CI: 2.0–4.60), reticulocyte count (3 studies, 1050 participants; HR: 1.05; 95%CI: 1.01–1.10), log(N-terminal-pro-brain natriuretic peptide) (3 studies, 800 participants; HR: 1.68; 95%CI: 1.48–1.90), and fetal hemoglobin (7 studies, 2477 participants; HR: 0.97; 95%CI: 0.94–1.0). This study identifies variables associated with mortality in adult patients with sickle cell disease in the hydroxyurea era

    Albuminuria Is Associated with Endothelial Dysfunction and Elevated Plasma Endothelin-1 in Sickle Cell Anemia

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    The pathogenesis of albuminuria in SCD remains incompletely understood. We evaluated the association of albuminuria with measures of endothelial function, and explored associations of both albuminuria and measures of endothelial function with selected biological variables (vascular endothelial growth factor [VEGF], endothelin-1 [ET-1], soluble fms-like tyrosine kinase-1 [sFLT-1], soluble vascular cell adhesion molecule-1 [soluble VCAM-1] and plasma hemoglobin)

    Hemodynamic Characteristics and Predictors of Pulmonary Hypertension in Patients With Sickle Cell Disease

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    Pulmonary hypertension (PH) is a common comorbidity of sickle cell disease (SCD) with an associated increased mortality risk, but its etiology is not well-understood. To evaluate the hemodynamic characteristics, clinical predictors, and cardiovascular manifestations of elevated pulmonary arterial pressure in this population, we performed noninvasive hemodynamic assessments of 135 patients with SCD using Doppler echocardiography. A diagnosis of PH was based on gender-, age-, and body mass index (BMI)-specific normal reference ranges for tricuspid regurgitation jet velocities (TRV). A high TRV was noted in 34 (25%) of patients. Pulmonary vascular resistance (PVR) was elevated in only 2 of the 34 patients (6%) with suspected PH, but was significantly higher than in those with a normal TRV. In univariate regression, TRV correlated with age, BMI, left atrial pressure, and right ventricular stroke volume, and was negatively associated with hemoglobin and glomerular filtration rate. Left atrial pressure, right ventricular stroke volume, and hemoglobin remained independent predictors of TRV in a multivariate model. A higher TRV was also associated with larger right ventricular and right atrial chamber sizes and higher N-terminal pro-brain natriuretic peptide levels. Our results suggest that the mild elevation in TRV often observed in patients with SCD is rarely associated with a high PVR, and that multiple factors – including the compensatory high output state associated with anemia, pulmonary venous hypertension, and a pulmonary vasculopathy – may contribute to an elevated pulmonary arterial pressure in these patients

    Real time quaking-induced conversion analysis of cerebrospinal fluid in sporadic Creutzfeldt-Jakob disease

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    OBJECTIVE: Current cerebrospinal fluid (CSF) tests for sporadic Creutzfeldt-Jakob disease (sCJD) are based on the detection of surrogate markers of neuronal damage such as CSF 14-3-3 which are not specific for sCJD. A number of prion protein conversion assays have been developed, including real-time quaking induced conversion (RT-QuIC). The objective of this study is to investigate whether CSF RT-QuIC analysis could be used as a diagnostic test in sCJD. METHODS: An exploratory study was undertaken which analysed 108 CSF samples from patients with neuropathologically confirmed sCJD or from control patients. Of the 108 CSF samples 56 were from sCJD patients (30 female, 26 male, aged 31–84 years; 62.3 ± 13.5 years) and 52 were from control patients (26 female, 26 male, aged 43–84 years; 67.8 ± 10.4 years). A confirmatory group of 118 patients were subsequently examined which consisted of 67 cases of neuropathologically confirmed sCJD (33 female, 34 male, aged 39–82 years; 67.5 ± 9.0 years) and 51 control cases (26 female, 25 male, aged 36–87 years; 63.5 ± 11.6 years). RESULTS: The exploratory study showed that RT-QuIC analysis had a sensitivity of 91% and a specificity of 98% for the diagnosis of sCJD. These results were confirmed in the confirmatory study which showed that CSF RT-QuIC analysis had a sensitivity and specificity of 87% and 100% respectively. INTERPRETATION: This study shows that CSF RT-QuIC analysis has the potential to be a more specific diagnostic test for sCJD than current CSF tests

    Longitudinal study of echocardiography-derived tricuspid regurgitant jet velocity in sickle cell disease

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    Although echocardiography-derived tricuspid regurgitant jet velocity (TRV) is associated with increased mortality in sickle cell disease (SCD), its rate of increase and predictive markers of its progression are unknown. We evaluated 55 subjects (median age: 38 years, range: 20 – 65 years) with at least 2 measurable TRVs, followed for a median of 4.5 years (range: 1.0 – 10.5 years) in a single-centre, prospective study. Thirty-one subjects (56%) showed an increase in TRV, while 24 subjects (44%) showed no change or a decrease in TRV. A linear mixed effects model indicated an overall rate of increase in the TRV of 0.02 m/s per year (p = 0.023). The model showed that treatment with hydroxycarbamide was associated with an initial TRV that was 0.20 m/s lower than no such treatment (p = 0.033), while treatment with angiotensin converting enzyme inhibitors and inhibitors/ angiotensin receptor blockers was associated with an increase in the TRV (p = 0.006). In summary, although some patients have clinically meaningful increases, the overall rate of TRV increase is slow. Treatment with hydroxycarbamide may decrease the progression of TRV. Additional studies are required to determine the optimal frequency of screening echocardiography and the effect of therapeutic interventions on the progression of TRV in SCD

    Biomarkers and diagnostic guidelines for sporadic Creutzfeldt-Jakob disease

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    Sporadic Creutzfeldt-Jakob disease is a fatal neurodegenerative disease caused by misfolded prion proteins (PrP^{Sc}). Effective therapeutics are currently not available and accurate diagnosis can be challenging. Clinical diagnostic criteria use a combination of characteristic neuropsychiatric symptoms, CSF proteins 14-3-3, MRI, and EEG. Supportive biomarkers, such as high CSF total tau, could aid the diagnostic process. However, discordant studies have led to controversies about the clinical value of some established surrogate biomarkers. Development and clinical application of disease-specific protein aggregation and amplification assays, such as real-time quaking induced conversion (RT-QuIC), have constituted major breakthroughs for the confident pre-mortem diagnosis of sporadic Creutzfeldt-Jakob disease. Updated criteria for the diagnosis of sporadic Creutzfeldt-Jakob disease, including application of RT-QuIC, should improve early clinical confirmation, surveillance, assessment of PrP^{Sc} seeding activity in different tissues, and trial monitoring. Moreover, emerging blood-based, prognostic, and potentially pre-symptomatic biomarker candidates are under investigation

    Intradialytic hyperalimentation as adjuvant support in pregnant hemodialysis patients: case report and review of the literature

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    Pregnancy in chronic dialysis patients is unusual and associated with many complications. Infants are often born both prematurely and small for gestational age. We report a case of a 36-year-old diabetic hemodialysis patient G4P3 who had prolonged hyperemesis gravidarum, for whom intradialytic parenteral nutrition (IDPN) was started at week 14 and continued throughout her pregnancy. She delivered a 3.5-kg baby girl at the 36th week of gestation by cesarean section. We discuss the use of IDPN as adjunct therapy for pregnant dialysis patients

    Late pregnancy vitamin D deficiency is associated with doubled odds of birth asphyxia and emergency caesarean section: A prospective cohort study

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    Objectives: The aim of this prospective cohort study was to investigate the associations between maternal vitamin D status in late pregnancy and emergency caesarean section (EMCS) and birth asphyxia, in a population based sample of women in Sweden. Methods: Pregnant women were recruited at the antenatal care in Sweden and 1832 women were included after exclusion of miscarriages, terminated pregnancies and missing data on vitamin D status. Mode of delivery was retrieved from medical records. EMCS was defined as caesarean section after onset of labour. Birth asphyxia was defined as either 5 min Apgar score < 7 or arterial umbilical cord pH < 7.1. Serum was sampled in the third trimester of pregnancy (T3) and 25-hydroxyvitamin D (25OHD) was analysed by liquid chromatography tandem mass spectrometry. Vitamin D deficiency was defined as 25OHD < 30 nmol/L, and associations were studied using logistic regression analysis and expressed as adjusted odds ratios (AOR). Results: In total, 141 (7.7%) women had an EMCS and 58 (3.2%) children were born with birth asphyxia. Vitamin D deficiency was only associated with higher odds of EMCS in women without epidural anaesthesia (AOR = 2.01, p = 0.044). Vitamin D deficiency was also associated with higher odds of birth asphyxia (AOR = 2.22, p = 0.044). Conclusions for Practice: In this Swedish prospective population-based cohort study, vitamin D deficiency in late pregnancy was associated with doubled odds of birth asphyxia and with EMCS in deliveries not aided by epidural anaesthesia. Prevention of vitamin D deficiency among pregnant women may reduce the incidence of EMCS and birth asphyxia. The mechanism behind the findings require further investigation
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