Genomic and functional analyses unveil the response to hyphal wall stress in Candida albicans cells lacking β(1,3)-glucan remodeling

Class 2: Transcripts that are less abundant in the phr1Δ mutant than in the wild type. (XLSX 39 kb

The relationship between maximal left ventricular wall thickness and sudden cardiac death in childhood onset hypertrophic cardiomyopathy

Background: Maximal left ventricular wall thickness (MLVWT) is a risk factor for sudden cardiac death (SCD) in hypertrophic cardiomyopathy (HCM). In adults, the severity of left ventricular hypertrophy has a nonlinear relationship with SCD, but it is not known whether the same complex relationship is seen in childhood. The aim of this study was to describe the relationship between left ventricular hypertrophy and SCD risk in a large international pediatric HCM cohort. Methods: The study cohort comprised 1075 children (mean age, 10.2 years [±4.4]) diagnosed with HCM (1–16 years) from the International Paediatric Hypertrophic Cardiomyopathy Consortium. Anonymized, noninvasive clinical data were collected from baseline evaluation and follow-up, and 5-year estimated SCD risk was calculated (HCM Risk-Kids). Results: MLVWT Z score was <10 in 598 (58.1%), ≥10 to <20 in 334 (31.1%), and ≥20 in 143 (13.3%). Higher MLVWT Z scores were associated with heart failure symptoms, unexplained syncope, left ventricular outflow tract obstruction, left atrial dilatation, and nonsustained ventricular tachycardia. One hundred twenty-two patients (71.3%) with MLVWT Z score ≥20 had coexisting risk factors for SCD. Over a median follow-up of 4.9 years (interquartile range, 2.3–9.3), 115 (10.7%) had an SCD event. Freedom from SCD event at 5 years for those with MLVWT Z scores <10, ≥10 to <20, and ≥20 was 95.6%, 87.4%, and 86.0, respectively. The estimated SCD risk at 5 years had a nonlinear, inverted U-shaped relationship with MLVWT Z score, peaking at Z score +23. The presence of coexisting risk factors had a summative effect on risk. Conclusions: In children with HCM, an inverted U-shaped relationship exists between left ventricular hypertrophy and estimated SCD risk. The presence of additional risk factors has a summative effect on risk. While MLVWT is important for risk stratification, it should not be used either as a binary variable or in isolation to guide implantable cardioverter defibrillator implantation decisions in children with HCM

Clinical Features and Natural History of Preadolescent Nonsyndromic Hypertrophic Cardiomyopathy

BACKGROUND Up to one-half of childhood sarcomeric hypertrophic cardiomyopathy (HCM) presents before the age of 12 years, but this patient group has not been systematically characterized. OBJECTIVES The aim of this study was to describe the clinical presentation and natural history of patients presenting with nonsyndromic HCM before the age of 12 years. METHODS Data from the International Paediatric Hypertrophic Cardiomyopathy Consortium on 639 children diagnosed with HCM younger than 12 years were collected and compared with those from 568 children diagnosed between 12 and 16 years. RESULTS At baseline, 339 patients (53.6%) had family histories of HCM, 132 (20.9%) had heart failure symptoms, and 250 (39.2%) were prescribed cardiac medications. The median maximal left ventricular wall thickness z-score was 8.7 (IQR: 5.3-14.4), and 145 patients (27.2%) had left ventricular outflow tract obstruction. Over a median follow-up period of 5.6 years (IQR: 2.3-10.0 years), 42 patients (6.6%) died, 21 (3.3%) underwent cardiac transplantation, and 69 (10.8%) had life-threatening arrhythmic events. Compared with those presenting after 12 years, a higher proportion of younger patients underwent myectomy (10.5% vs 7.2%; P = 0.045), but fewer received primary prevention implantable cardioverter-defibrillators (18.9% vs 30.1%; P = 0.041). The incidence of mortality or life-threatening arrhythmic events did not differ, but events occurred at a younger age. CONCLUSIONS Early-onset childhood HCM is associated with a comparable symptom burden and cardiac phenotype as in patients presenting later in childhood. Long-term outcomes including mortality did not differ by age of presentation, but patients presenting at younger than 12 years experienced adverse events at younger ages. (C) 2022 The Authors. Published by Elsevier on behalf of the American College of Cardiology Foundation.Peer reviewe

Relationship Between Maximal Left Ventricular Wall Thickness and Sudden Cardiac Death in Childhood Onset Hypertrophic Cardiomyopathy

Background: Maximal left ventricular wall thickness (MLVWT) is a risk factor for sudden cardiac death (SCD) in hypertrophic cardiomyopathy (HCM). In adults, the severity of left ventricular hypertrophy has a nonlinear relationship with SCD, but it is not known whether the same complex relationship is seen in childhood. The aim of this study was to describe the relationship between left ventricular hypertrophy and SCD risk in a large international pediatric HCM cohort. Methods: The study cohort comprised 1075 children (mean age, 10.2 years [+/- 4.4]) diagnosed with HCM (1-16 years) from the International Paediatric Hypertrophic Cardiomyopathy Consortium. Anonymized, noninvasive clinical data were collected from baseline evaluation and follow-up, and 5-year estimated SCD risk was calculated (HCM Risk-Kids). Results: MLVWT Z score was = 10 to = 20 in 143 (13.3%). Higher MLVWT Z scores were associated with heart failure symptoms, unexplained syncope, left ventricular outflow tract obstruction, left atrial dilatation, and nonsustained ventricular tachycardia. One hundred twenty-two patients (71.3%) with MLVWT Z score >= 20 had coexisting risk factors for SCD. Over a median follow-up of 4.9 years (interquartile range, 2.3-9.3), 115 (10.7%) had an SCD event. Freedom from SCD event at 5 years for those with MLVWT Z scores = 10 to = 20 was 95.6%, 87.4%, and 86.0, respectively. The estimated SCD risk at 5 years had a nonlinear, inverted U-shaped relationship with MLVWT Z score, peaking at Z score +23. The presence of coexisting risk factors had a summative effect on risk. Conclusions: In children with HCM, an inverted U-shaped relationship exists between left ventricular hypertrophy and estimated SCD risk. The presence of additional risk factors has a summative effect on risk. While MLVWT is important for risk stratification, it should not be used either as a binary variable or in isolation to guide implantable cardioverter defibrillator implantation decisions in children with HCM.Peer reviewe

Serrations effect on the aerodynamic performance of wind turbine airfoils

A numerical and experimental analysis of the trailing edge serrations effect on the aerodynamic performance of the NACA643418 airfoil is presented. 3D Reynolds-averaged Navier-Stokes simulations (RANS) of the flow over the airfoil with and without trailing edge serrations have been carried out with the Transitions SST turbulence model. From these calculations an empirical law for the prediction of the aerodynamic behaviour of the airfoil with serrations is derived. The law is validated through wind tunnel experiments. The NACA643418 airfoil has been tested with trailing edge serrations installed with different flap angles to analyze the effect of the parameter on the performance of the airfoil. Results show that the trailing edge serrations cause a significant increment in lift coefficient with low penalties in drag.Wind Energ

Trailing edge serrations effects on the aerodynamic performance of a NACA 643418

A study of the aerodynamic performance of a NACA 643418 airfoil with trailing edge serrations is presented. For the prediction of the changes in lift due to the serration installation, an empirical law is derived that can be extended to typical cambered airfoils for wind turbine applications. The law is deduced from 2D and 3D Reynolds‐averaged Navier–Stokes simulations (RANS) of the flow over the airfoil. Lift and drag together with the changes in the wake flow due to the presence of the serrated edges are investigated. An additional study of the sensitivity of the results at Rec = 3·106 with respect to the turbulence modeling is carried out by using three different RANS models: Spalart–Allmaras, k‐omega SST, and Transition SST. Results show that the changes in lift due to trailing‐edge extensions are approximated by the effect of a split plate with reduced length.Green Open Access added to TU Delft Institutional Repository ‘You share, we take care!’ – Taverne project https://www.openaccess.nl/en/you-share-we-take-care Otherwise as indicated in the copyright section: the publisher is the copyright holder of this work and the author uses the Dutch legislation to make this work public.Wind Energ

Trailing-edge serrations effect on the performance of a wind turbine

An experimental study focusing on the change of the aerodynamic performance of a wind turbine with the employment of trailing-edge serrations is presented. The design procedure which starts from the serration design, together with the experimental wind tunnel testing and the installation in an already operating wind turbine is discussed. A prediction methodology to estimate the aerodynamic performance change of the machine with the blade add-on is validated by in-field measurements. Wind tunnel experiments for the validation of the original serration design have been carried out on a Nordex ADO30 airfoil with a relative thickness of 30% and on a NACA643418 airfoil with 18% thickness. The two airfoils are respectively used in this study due to their peculiar characteristics. While the first has been designed for relatively high Cl/Cd performance versus structural integrity (i.e. relatively high thickness), the second one is a typical reference airfoil used in wind turbines for its interesting Cl/Cd performance in both laminar and rough conditions. Clean and rough conditions have been tested in order to prepare a database for the analysis of the full turbine in several wind conditions. Aerodynamic forces with the serrated trailing-edge extensions are measured for different angles of attack and serration flap angles. The results are further discussed and employed for the analysis of the full rotor. Results already show that an increase in the flap angle is typically associated with an increment in lift, but not necessarily in drag. This has a beneficial effect on the operational regime of the machine when taken into account. The influence of the trailing-edge serrations on an operating wind turbine has been quantified in terms of total loads and energy production. The power curves with and without the trailing-edge installations are further analyzed and compared with the theoretical predictions. • Experimental tests show big impact of serrations on airfoil's aerodynamic properties • Predictive law used for extending the serrations influence to the whole blade • Theoretically, serrations have positive impact on power production of wind turbines • Theoretically, serrations increase wind turbine loads and reduce its life-time • Positive impact of serrations on power production of real machines has been probedGreen Open Access added to TU Delft Institutional Repository ‘You share, we take care!’ – Taverne project https://www.openaccess.nl/en/you-share-we-take-care Otherwise as indicated in the copyright section: the publisher is the copyright holder of this work and the author uses the Dutch legislation to make this work public.Wind Energ