Normal Mean Research Articles

Serial changes in left atrial strain in symptomatic obstructive hypertrophic cardiomyopathy treated with mavacamten: insights from VALOR-HCM trial

Abstract Background In severely symptomatic patients with obstructive hypertrophic cardiomyopathy (HCM), the VALOR-HCM phase 3 randomized, double-blind, placebo-controlled clinical trial demonstrated that mavacamten, a cardiac myosin inhibitor, reduces the eligibility for septal reduction therapy with sustained improvement in left ventricular (LV) outflow tract gradients and symptoms. Mavacamten also resulted in favorable cardiac remodeling, including improvement in left atrial volume index (LAVI), LV diastolic function, and biomarkers (NT-proBNP and troponin T). However, the impact of mavacamten on LA function is not known. Objective We sought to assess serial changes in LA strain measures in patients enrolled in the VALOR-HCM trial. Methods VALOR-HCM included 112 symptomatic obstructive HCM patients (mean age 60 years, 51% male). Patients assigned to mavacamten at baseline (n=56) continued therapy for 56 weeks and those assigned to placebo crossed over to mavacamten (n=52) from week 16-56 (40-week exposure). Echocardiographic LA strain assessment was performed according to the current American and European guideline recommendations, using a vendor-neutral post processing software. For assessment of LA strain components (reservoir, conduit, and contraction), the non-foreshortened apical 4-chamber view was utilized. The zero reference was set at end-diastole. Results Serial changes in LAVI and all components of mean LA strain from baseline to week 56 are shown in the Figure. At baseline, LAVI and all mean LA strain values were worse than reported normal mean thresholds. There was a continued and significant improvement in all 3 LA strain values, from baseline to week 56, along with a significant reduction in LAVI. Improvement in LAVI was seen earlier than improvements in LA strain. There was a significant inverse correlation between change in mean LAVI and LA reservoir strain from baseline to week 56 (r= -0.42, p<0.001). Conclusions In the VALOR-HCM trial, average LAVI and baseline LA strain values were significantly worse than reported normal thresholds. Treatment with mavacamten resulted in an improvement in LAVI and LA strain values from baseline through week 56 suggesting a sustained favorable impact on cardiac remodeling. The advantageous cardiac remodeling associated with long-term treatment with mavacamten may in turn have a favorable impact on the observed high burden of atrial tachyarrhythmias in HCM.Serial changes in LA strain in VALOR-HCM

Impact of switching from EFV/F/TDF to B/F/TAF on psychiatric symptoms and neurocognition.

The aim was to investigate whether switching from EFV/F/TDF to B/F/TAF may improve neuropsychiatrc symptoms and neurocognition. Pilot, single-arm, prospective study of persons with HIV (PWH) on the efficacy and safety of switching from EFV/F/TDF to B/F/TAF. Participants underwent neuropsychological assessment (NPA) at switch (T0) and after 48 weeks (T1). NPA was carried out through a standardized battery of 12 tests. Neurocognitive impairment (NCI) was defined by a score ≥1 standard deviation (SD) below the normal mean on at least 2 tests or ≥2 SD below on 1 test. Individual z-scores were determined, NPZ-12 was calculated as the average of 12 test z-scores and change of NPZ-12 was the outcome. HIV-associated Neurocognitive Disorder (HAND) was classified by Frascati's criteria. Beck Anxiety Inventory (BAI), Beck Depression Inventory (BDI-II), and Pittsburgh Sleep Quality Index (PSQI) were administered. Paired-Wilcoxon and McNemar tests were used for comparisons, and logistic regression for associations with NCI changes. Out of 126 participants, BAI, BDI-II, and PSQI questionnaires revealed an improvement at T1. NPA revealed NCI in 40.5% of persons at T0 and 42.1% at T1 (p = 0.746). Specifically, at T0, among participants with NCI, 35% improved; among those without, 26% worsened at T1; NPZ-12 score worsened at T1. 5.6% of ANI was observed at T0 and 7.9% at T1. No factor associated with these changes was found. Our results suggest switching from EFV/F/TDF to B/F/TAF significantly improves psychiatric symptoms and sleep quality. Neurocognitive performance remained stable, although a decline in NPZ-12 and in specific domains was observed.

TREATMENT OF PULMONARY ARTERIAL HYPERTENSION ASSOCIATED WITH CONGENITAL HEART DEFECTS

Patients with pulmonary arterial hypertension, associated with congenital heart disease (PAH-CHD) are a heterogeneous population with a varied course of PH. Improvements in pediatric cardiac surgery have changed the epidemiology and survival rate of patients with CHD, of which 90% reach adulthood. Progress in terms of prognosis has also been observed among patients with PAH-CHD. Better survival was observed in ES compared with PAH after defect correction. Advances in surgical treatment of CHD and an increase in life expectancy have led to the study of PAH-CHD and the need to create recommendations for drug treatment of this category of patients. In most of studies, the evaluation of drug treatment of group 1 PAH was carried out without identifying its subgroups. And thus, according to existing recommendations, treatment algorhithms for patients with PAH-CHD are similar to approaches to other forms of PAH. However, various clinical, functional, physical and hemodynamic characteristics of patients with PAH-CHD call into question of correct risk stratification approaches development. Multicenter randomized clinical trials included predominantly a small number of patients with corrected defects, which does not allow the results to be interpreted for the entire population of patients with PAH-CHD. Data from single-center observational studies, expert opinion, and several randomized trials primarily involving patients with Eisenmenger syndrome (ES) indicate the effectiveness and safety of PAH-specific therapy in patients with PAH-CHD. In this literature review, we examined and showed the results of studies involving patients with PAH-CHD and their response to specific therapy. The results obtained significantly expanded the possibilities of using bosentan, sildenafil, epoprostenol, riociguat, ralinepag, sotatercept as they lead to improvement of functional capacity and hemodynamic parameters in patients with PAH-CHD, and only epoprostenol demonstrated an effect on prognosis. Combination PAH-specific therapy, initial or sequential administration of two or more drugs with different mechanisms of action, is an important treatment strategy for patients with PAH. The role of such therapy has increased in recent years. Based on the results of the AMBITION, SERAPHIN, GRIPHON, COMPASS-2 studies, initial or sequential oral combination PAH-specific therapy is recommended for patients with WHO FC II or III. At the same time, there is little evidence to support the effectiveness of this approach in ES patients. The use of anticoagulants in PAH-CHD remains controversial. Low-flow oxygen therapy should be considered individually and continued when there is a significant predominance of subjective or objective benefit. Iron deficiency is associated with poor survival in ES. It is important to note that microcytosis is rare in patients with iron deficiency cyanosis and a normal mean red cell volume does not indicate the absence of anemia. In cases of intolerance to oral iron, intravenous drugs should be used. Currently, based on existing guidelines, most centers follow a consistent symptom-based approach in the treatment of patients with PAH-CHD. Therapy begins with oral ERAs or PDE-5 inhibitors and is escalated if symptoms persist or clinical worsening occurs. If there is no effect of oral PAH-specific therapy, it is recommended to consider parenteral drugs.

Case of Bone Morphogenetic Protein Receptor Type 2 (BMPR2) Mutation Complicated by Pulmonary Hypertension Encountered at Autopsy

Abstract Introduction/Objective Bone morphogenetic protein receptor type 2 is a protein encoded by the BMPR2 gene which regulates cell division and apoptosis. BMPR2 mutations result in monoclonal plexiform lesions of proliferating endothelial cells in pulmonary arterioles, which leads to elevated pulmonary artery pressures, right ventricular failure, and death. Here, we present the case of a 12-year-old male with a known history of pulmonary hypertension secondary to BMPR2 mutation referred for medical autopsy after suffering cardiac arrest and passing away. Methods/Case Report This 12-year-old male was diagnosed with pulmonary hypertension in December 2022 after suffering a syncopal episode while running at recess. Genetic testing was obtained, and he was found to have a BMPR2 mutation. His father was tested and also harbored this mutation, but did not clinically have pulmonary hypertension. The decedent was initiated on ambrisentan, tadalafil, and spironolactone, but continued to present to the Emergency Department periodically for syncopal episodes. In April 2024, he presented to the Medical University of South Carolina in cardiac arrest which began while playing outside. A complete autopsy was performed, including gross and microscopic examination of tissues and postmortem vitreous electrolyte analysis. Gross examination revealed the decedent’s heart was enlarged to 350 grams (normal mean for age = 181 grams). Both cardiac ventricles were dilated and the right ventricle was markedly hypertrophic with a thickness of 1.2 cm. Microscopic sections are pending but will presumptively show significant cardiac myocyte hypertrophy and pulmonary arteriole thickening. Cause of death is pending certification but is presumed to be sudden cardiac death resulting from BMPR2 mutation- related pulmonary hypertension. Results (if a Case Study enter NA) NA Conclusion This case highlights a common genetic presentation of pulmonary arterial hypertension, with approximately 80% of familial cases of pulmonary arterial hypertension harboring BMPR2 mutations. Further research should be done to investigate treatment options and management of this genetic presentation.

Fast and simultaneous prediction of inner quality parameters on intact mangos by near infrared spectroscopy: Impact of spectra pre-processing on prediction accuracy

Near infrared spectroscopy or known as NIRS has been widely employed in many fields including agriculture, especially for sorting and grading of agricultural products. Spectra pre-processing is one of the main factors affecting model accuracy and prediction capabilities of NIRS. The objective of the present study was to study the impact of different spectra corrections namely mean centering (MC), mean normalization (MN), de-trending (DT), multiplicative scatter correction (MSC), standard normal variate (SNV) and orthogonal signal correction (OSC), to the prediction accuracy of quality parameters: titratable acidity (TA) and soluble solids content (SSC) in intact mango. A total of 91 mango samples (cv. Kent) were used as dataset for calibration and external prediction which was separated by means of systematic sampling based on a property (SSBP) approach. Diffuse reflectance spectra (log1/R) were acquired and recorded in wavelength range of 1000 – 2500 nm by Antaris Fourier transform NIR instrument. Judging from calibration and prediction performance, MSC found to be the best spectra pre-processing method prior to prediction model development with R2 prediction are 0.72 for TA and 0.76 for SSC. Although MSC increase the prediction performances based on R2, RMSE, RPD and RER metrics compared to the baseline, the achieved RPD, 1.9 for TA and 1.8 for SSC of this findings are still poor and need improvements to achieve even higher levels of accuracy and reliability necessitates for real-time applications.

The L1 spino-pelvic (L1SP) angle: a simplified approach for the assessment of the PI-LL mismatch in hip surgery

Introduction: Pelvic incidence - lumbar lordosis (PI-LL) mismatch is often considered when assessing spinopelvic alignment in the sagittal plane. The mismatch is conventionally obtained by measuring 2 separate angles on lateral spinopelvic radiographs. This study describes a simplified approach for assessing spinopelvic mobility and measuring the PI-LL mismatch through the evaluation of the L1-spinopelvis angle (L1SP). Methods: 96 standing lateral radiographs were obtained from consecutive patients presenting for total hip arthroplasty between November 2020 and July 2021. 3 operators were recruited to annotate landmarks on digital radiographs. Correlation analysis and error analysis were applied. Measurement reproducibility was assessed using intraclass correlation coefficient (ICC). Results: The correlation coefficients of the 3 variables were respectively 0.87 for PI, 0.94 for LL, and 0.96 for L1SP. The normalised root mean square error between the 2 measurement sets was 9.96% for PI, 5.97% for LL, and 4.41% for L1SP. The absolute error was 3.49° ± 4.63° for PI, 3.23° ± 3.78° for LL, 2.68° ± 3.19° for PI-LL conventional, and 2.35° ± 2.88° for PI-LL via L1SP, respectively. In terms of reproducibility, measurement of L1SP outperformed that of PI and LL (ICC = 0.97 versus 0.83 and 0.93, respectively). Conclusion: The simplified L1SP method, through the measurement of a single angle, produced similar measurements to the conventional PI-LL method. The measurement repeatability between operators was improved using the L1SP method. From a clinical practice perspective, both methods are equivalent. The new method is readily reproducible using commercially available PACS software during preoperative templating.

Hypertension and its correlation with autonomic nervous system dysfunction, heart rate variability and chronic inflammation

Objective This study investigates the relationship between hypertension, dysregulation of the autonomic nervous system, heart rate variability (HRV), and chronic inflammation. Methods We analysed a cohort of 50 hypertensive patients treated at the affiliated Hospital of Jianghan University. The average systolic and diastolic blood pressures (BPs) in this group were 155.26 and 95.32 mmHg, respectively. A control group of 50 healthy volunteers, undergoing routine physical examinations at the same hospital, was also analysed. Results The average systolic BP of the control group was 115.64 ± 10.27 mmHg, and the average diastolic BP was 75.33 ± 8.25 mmHg. In contrast, the experimental group exhibited an average systolic BP of 155.26 ± 20.13 mmHg and an average diastolic BP of 95.32 ± 12.16 mmHg. Both systolic and diastolic BPs were significantly higher in the hypertensive group (p < 0.05). The experimental group also demonstrated reduced HRV and skin conductance response, alongside increased BP variability (BPV), urinary epinephrine levels and prolonged pupillary light reaction time compared to controls (p < 0.05). Notably, Standard Deviation of Normal to Normal Intervals (SDNN) and Root Mean Square of Successive Differences (RMSSD) values were significantly lower in the experimental group (p < 0.05). Furthermore, levels of inflammatory markers such as CRP, TNF-α, IL-6 and IL-1β were markedly elevated in hypertensive patients (p < 0.05). Negative correlations were observed between systolic and diastolic BP with HRV metrics, while positive correlations were found between BP and BPV as well as urinary adrenaline levels. Conclusions The findings indicate that hypertension is closely associated with autonomic nervous system dysfunction, reduced HRV and increased chronic inflammation. A comprehensive approach to hypertension management should integrate these interrelated physiological and pathological mechanisms, with potential therapeutic interventions targeting autonomic function and inflammatory states.

On Properties of the Hyperbolic Distribution

This paper is set to analytically describe properties of the hyperbolic distribution. This law, along with the variance-gamma distribution, is one of the most popular normal mean–variance mixtures from the point of view of various applications. We have found closed form expressions for the cumulative distribution and partial-moment-generating functions of the hyperbolic distribution. The obtained formulas use the values of the Humbert confluent hypergeometric and Whittaker special functions. The results are applied to the problem of European option pricing in the related Lévy model of financial market. The research demonstrates that the discussed normal mean–variance mixture is analytically tractable.

Estimating Ground-Level NO2 Concentrations Using Machine Learning Exclusively with Remote Sensing and ERA5 Data: The Mexico City Case Study

This study explores the estimation of ground-level NO2 concentrations in Mexico City using an integrated approach of machine learning (ML) and remote sensing data. We used the NO2 measurements from the Sentinel-5P satellite, along with ERA5 meteorological data, to evaluate a pre-trained machine learing model. Our findings indicate that the model captures the spatial and temporal variability of NO2 concentrations across the urban landscape. Key meteorological parameters, such as temperature and wind speed, were identified as significant factors influencing NO2 levels. The model’s adaptability was further tested by incorporating additional variables, such as atmospheric boundary layer height. In order to compare the model’s performance to alternative ML models, we estimated the ground-level NO2 using the state-of-the-art TimeGPT. The results demonstrate that our baseline model has the best performance with a mean normalised root mean square error of 84.47%. This research underscores the potential of combining satellite observations with ML for scalable air quality monitoring, particularly in low- and middle-income countries with limited ground-based infrastructure. The study provides critical insights for air quality management and policy-making, aiming to mitigate the adverse health and environmental impacts of NO2 pollution.

Group Inverse-Gamma Gamma Shrinkage for Sparse Linear Models with Block-Correlated Regressors

Heavy-tailed continuous shrinkage priors, such as the horseshoe prior, are widely used for sparse estimation problems. However, there is limited work extending these priors to predictors with grouping structures. Of particular interest in this article, is regression coefficient estimation where pockets of high collinearity in the covariate space are contained within known covariate groupings. To assuage variance inflation due to multicollinearity we propose the group inverse-gamma gamma (GIGG) prior, a heavy-tailed prior that can trade-off between local and group shrinkage in a data adaptive fashion. A special case of the GIGG prior is the group horseshoe prior, whose shrinkage profile is correlated within-group such that the regression coefficients marginally have exact horseshoe regularization. We show posterior consistency for regression coefficients in linear regression models and posterior concentration results for mean parameters in sparse normal means models. The full conditional distributions corresponding to GIGG regression can be derived in closed form, leading to straightforward posterior computation. We show that GIGG regression results in low mean-squared error across a wide range of correlation structures and within-group signal densities via simulation. We apply GIGG regression to data from the National Health and Nutrition Examination Survey for associating environmental exposures with liver functionality.

Serial Changes in Ventricular Strain in Symptomatic Obstructive Hypertrophic Cardiomyopathy Treated With Mavacamten: Insights From the VALOR-HCM Trial.

In severely symptomatic patients with obstructive hypertrophic cardiomyopathy, VALOR-HCM (A Study to Evaluate Mavacamten in Adults With Symptomatic Obstructive Hypertrophic Cardiomyopathy Who Are Eligible for Septal Reduction Therapy) demonstrated that mavacamten reduces the need for septal reduction therapy with sustained improvement in left ventricular (LV) outflow tract gradients and symptoms. Global longitudinal strain (GLS), a measure of regional myocardial function, is a more sensitive marker of systolic function. In VALOR-HCM, we assessed serial changes in LV and right ventricular (RV) strain. VALOR-HCM included 112 patients with symptomatic obstructive hypertrophic cardiomyopathy (mean, 60 years; 51% male; LV ejection fraction, 68%). Patients assigned to mavacamten at baseline continued the drug for 56 weeks (n=56) and those assigned to placebo (n=52) transitioned to mavacamten from weeks 16 to 56 (40-week exposure). LV-GLS and RV-GLS assessment was performed using a vendor-neutral software. Non-foreshortened apical (4-, 3-, and 2-chamber) views were used to obtain peak LV-GLS. RV focused 4-chamber view was used to calculate RV 4-chamber and free wall strain. A more negative strain value is favorable. At baseline, the mean LV-GLS, RV 4-chamber, and free wall strain values were -14.7%, -22.2%, and -16.8%, respectively (all worse than reported normal means). In the total study sample, LV-GLS significantly improved from baseline to week 56 (P=0.02). Twelve patients had transient reduction in LV ejection fraction (<50%) requiring temporary drug interruption (including 3 permanent discontinuations). The LV-GLS in this subgroup was worse at baseline versus total study population (-11.4%), with no significant worsening from baseline through week 56 (P=0.64). Both free wall and 4-chamber RV-GLS remained unchanged from baseline to week 56 (P=0.62 and P=0.56, respectively). In VALOR-HCM, treatment with mavacamten improved LV-GLS from baseline through week 56 (with no significant worsening of LV-GLS in patients with a reduction in LV ejection fraction ≤50%), suggesting a favorable long-term impact on regional LV systolic function. Additionally, there was no detrimental impact on RV systolic function. URL: https://www.clinicaltrials.gov; Unique identifier: NCT04349072.

Bayes oracle property of multiple tests of multivariate normal means under sparsity

The paper considers a multiple testing problem of multivariate normal means under sparsity. First, the Bayes risk of the multivariate Bayes oracle is derived. Then, a hierarchical Bayesian approach is taken with global–local shrinkage priors, where the global parameter is either treated as a tuning parameter or is given a specific prior. The method is shown to attain an asymptotic Bayes optimal under sparsity (ABOS) property. Finally, an empirical Bayes procedure is proposed which involves estimation of the global shrinkage parameter. The approach is also shown to lead to the ABOS property.

Enhancing battery electrochemical-thermal model accuracy through a hybrid parameter estimation framework

Electrochemical-thermal models offer profound insight into the internal state of batteries, demonstrating significant potential in energy storage. However, model complexity makes accurate parameter acquisition challenging. This study proposes a novel hybrid parameter estimation framework for effective parameterisation of battery electrochemical-thermal models. A high-fidelity reduced-order electrochemical-thermal model was established to accelerate the simulation of the entire time-domain process. A global sensitivity analysis of the model parameters was conducted, comprehensively evaluating their impact on simulated voltage and temperature. Highly sensitive parameters were categorised into static and time-dependent dynamic estimation groups for hierarchical estimation. A deep learning method was applied to generate preliminary parameter estimates to accelerate the convergence of estimation process. Subsequently, a genetic algorithm was employed to optimise the preliminary estimates of static parameters. A full temporal domain estimation was conducted using Markov Chain Monte Carlo methods for time-dependent dynamic parameters. The experimental data covers six dynamic and nine constant current conditions to validate the proposed method's feasibility. After parameter estimation, the proposed method reduces the comprehensive normalised root mean square error of voltage and temperature by up to 80.36% compared to four conventional methods. The minimum average absolute errors of voltage and temperature under constant and dynamic current conditions are 10.63 mV and 0.10 °C, respectively. The proposed method provides new insights for the parameterisation of battery models.

Barriers to Early Progress in Adult Cochlear Implant Outcomes.

Adult cochlear implant (CI) recipients obtain varying levels of speech perception from their device. Adult CI users adapt quickly to their CI if they have no peripheral "bottom-up" or neurocognitive "top-down" limiting factors. Our objective here was to understand the influence of limiting factors on the progression of sentence understanding in quiet and in noise, initially and over time. We hypothesized that the presence of limiting factors, detected using a short test battery, would predictably influence sentence recognition with practical consequences. We aimed to validate the test battery by comparing the presence of limiting factors and the success criteria of >90% sentence understanding in quiet 1 month after activation. The study was a single-clinic, cross-sectional, retrospective design incorporating 32 adult unilateral Nucleus CI users aged 27 to 90 years (mean = 70, SD = 13.5). Postoperative outcome was assessed through sentence recognition scores in quiet and in varying signal to noise ratios at 1 day, 1 to 2 months, and up to 2 years. Our clinic's standard test battery comprises physiological and neurocognitive measures. Physiological measures included electrically evoked compound action potentials for recovery function, spread of excitation, and polarity effect. To evaluate general cognitive function, inhibition, and phonological awareness, the Montreal Cognitive Assessment screening test, the Stroop Color-Word Test, and tests 3 and 4 of the French Assessment of Reading Skills in Adults over 16 years of age, respectively were performed. Physiological scores were considered abnormal, and therefore limiting, when total neural recovery periods and polarity effects, for both apical and basal electrode positions, were >1.65 SDs from the population mean. A spread of excitation of >6 electrode units was also considered limiting. For the neurocognitive tests, scores poorer than 1.65 SDs from published normal population means were considered limiting. At 1 month, 13 out of 32 CI users scored ≥90% sentence recognition in quiet with no significant dependence on age. Subjects with no limiting peripheral or neurocognitive factors were 8.5 times more likely to achieve ≥90% score in quiet at 1 month after CI switch-on (p = 0.010). In our sample, we detected 4 out of 32 cases with peripheral limiting factors that related to neural health or poor electrode-neural interface at both apical and basal positions. In contrast, neurocognitive limiting factors were identified in 14 out of 32 subjects. Early sentence recognition scores were predictive of long-term sentence recognition thresholds in noise such that limiting factors appeared to be of continuous influence. Both peripheral and neurocognitive processing factors affect early sentence recognition after CI activation. Peripheral limiting factors may have been detected less often than neurocognitive limiting factors because they were defined using sample-based criteria versus normal population-based criteria. Early performance was generally predictive of long-term performance. Understanding the measurable covariables that limit CI performance may inform follow-up and improve counseling. A score of ≥90% for sentence recognition in quiet at 1 month may be used to define successful progress; whereas, lower scores indicate the need for diagnostic testing and ongoing rehabilitation. Our findings suggest that sentence test scores as early as 1 day after activation can provide vital information for the new CI user and indicate the need for rehabilitation follow-up.

Rapid prediction of wall shear stress in stenosed coronary arteries based on deep learning.

There is increasing evidence that coronary artery wall shear stress (WSS) measurement provides useful prognostic information that allows prediction of adverse cardiovascular events. Computational Fluid Dynamics (CFD) has been extensively used in research to measure vessel physiology and examine the role of the local haemodynamic forces on the evolution of atherosclerosis. Nonetheless, CFD modelling remains computationally expensive and time-consuming, making its direct use in clinical practice inconvenient. A number of studies have investigated the use of deep learning (DL) approaches for fast WSS prediction. However, in these reports, patient data were limited and most of them used synthetic data generation methods for developing the training set. In this paper, we implement 2 approaches for synthetic data generation and combine their output with real patient data in order to train a DL model with a U-net architecture for prediction of WSS in the coronary arteries. The model achieved 6.03% Normalised Mean Absolute Error (NMAE) with inference taking only 0.35s; making this solution time-efficient and clinically relevant.

Body composition of adults with chronic intestinal failure receiving home parenteral nutrition: A descriptive cohort study.

Chronic intestinal failure (CIF) refers to the long-lasting reduction of gut function below the minimum necessary to absorb macronutrients, water, and/or electrolytes. Patients with CIF likely develop various forms of malnutrition and dehydration, yet studies that focus primarily on body composition are lacking. Therefore, this study aimed to evaluate the body composition of adult patients with CIF. This retrospective descriptive cohort study was performed at the Radboud University Medical Center, a tertiary referral center for CIF treatment in the form of home parenteral nutrition. We collected available bioelectrical impedance analysis (BIA) data from routine care between 2019 and 2023. The primary outcome was body composition, which was evaluated by assessing body mass index (BMI), fat-free mass index (FFMI), and fat percentage (fat%). Overall, 147 adult patients with CIF were included with a median (interquartile range) age of 58 (25-68) years; 69% were female. The mean (SD) BMI was 22.1 (4.3) kg/m2, FFMI was 14.2 (1.9) kg/m2 in females and 17.0 (2.0) kg/m2 in males, and fat% was 33.7% (6.8%) in females and 24.6% (6.4%) in males. 63% had an FFMI below references, and 48% had a high fat%. This study found that most adult patients with CIF have an unfavorable body composition characterized by a high fat% and low FFMI despite having a normal mean BMI. These results highlight the necessity for in-depth nutrition assessment, including BIA measurement. Moreover, future studies should focus on exercise interventions to increase FFMI and improve body composition and function.

The Auditory Steady-State Response and the Relationship between Electrophysiological and Behavioural Thresholds.

This study examined the relationship between behavioural thresholds as measured by pure tone audiometry and electrophysiological thresholds measured by the Auditory Steady-State Response (ASSR) in children with normal hearing and sensorineural hearing loss. After being assessed, 45 children of both sexes, ranging in age from 5 to 15, were split into four groups: 10 with moderate to moderately severe sensorineural hearing loss (G2M); 10 with steeply sloping sensorineural hearing loss (G2D); 10 with profound and severe sensorineural hearing loss (G2S); and 15 with normal hearing (G1). ASSR, tympanometry, acoustic reflex testing, pure tone audiometry, and speech audiometry (SRT and SDT) were performed. The electrophysiological maximum in the group with normal hearing thresholds varied from 19 to 27 dB NA. The correlation in the group with moderate to moderately severe hearing loss was 0.42-0.74. The correlation in the steeply sloping hearing loss group was 0.68-0.94. The correlation in the group of people with profound and severe hearing loss was 0.59-0.86. The normal hearing group's mean differences in ASSR threshold and audiometric threshold ranged from -0.3 to 12 dB, in the moderate and moderately severe hearing loss group from -9 to 2 dB, in the steeply sloping hearing loss group from 1.4 to 7.5 dB, and in the severe and profound hearing loss group from -0.40 to 8.5 dB. As expected, there was no strong relationship between behavioural and electrophysiological thresholds in the group with normal hearing. But in children with hearing loss, there was a strong correlation between electrophysiological and behavioural thresholds; this relationship was especially evident in children with severe and profound hearing loss and those with steeply sloping hearing loss.

Anemia and its association with glycemia and transaminitis in patients with type 2 diabetes mellitus: A cross-sectional pilot study.

Anemia impairs glucose homeostasis, affects glycemic control, and predisposes to complications in diabetics. It correlates with oxidative stress and increases the risk of developing microvascular and macrovascular complications. However, it is an underrecognized comorbidity in diabetics. This study was conducted to assess the prevalence of anemia in diabetic patients and compare the metabolic profiles of anemic and non-anemic diabetics. This is a cross-sectional study, conducted among type 2 diabetes (T2DM) patients, at the outpatient clinic. Patients with chronic kidney disease (CKD), known hematological disorders, and chronic inflammatory disorders were excluded. Of the 97 patients, 37 (38.14%) were found to be anemic (hemoglobin (Hb): male <13 g/dl, female <12 g/dl). The mean values of fasting blood sugar (FBS) in low and normal mean corpuscular volume (MCV) patients were 265.9 ± 43.7 mg/dl and 157.2 ± 7.2 mg/dl, respectively (P = 0.0026), and those of postprandial blood sugar (PPBS) were 370.3 ± 58.4 mg/dl and 226.3 ± 10.1 mg/dl, respectively (P = 0.0015). It was found that 6 (22.2%) of 27 patients with raised alanine aminotransferase (ALT) had anemia against 27 (45.8%) of 59 patients with normal ALT (P = 0.03). The mean Hb levels in patients with raised and normal ALT were 13.31 ± 2.3 gm% and 12.2 ± 2.0 gm% (P = 0.03), respectively. Blood sugar may have a direct relationship with MCV in T2DM patients. Hb tends to relate to hepatic enzymes likely due to altered dietary patterns in anemics. Further larger studies on the effect of iron supplementation and dietary habits on glycemic control and hepatic steatosis are warranted.

"Normal Means Blessing!" Awareness and Acceptance in Mothers of Children with Special Care Needs: A Qualitative Study

The objective of this study was to examine the acceptance and awareness of mothers with children with special needs regarding their children’s special situation. A qualitative exploratory approach was employed in this study. Between November 2023 and January 2024, in-depth individual interviews were conducted with 22 mothers of children with special needsFour primary themes emerged from the mothers included in the study regarding their acceptance and awareness of their children’s special situation: Challenges, Transformation, Adaptation, and Mistakes. The Challenges theme encompasses the child who deviates from expectations, lack of information, stigma, lack of support, burden of care, inadequacy, and guilt. The Transformation theme encompasses the concept of the special child, increased responsibilities, attitude toward the social environment, and normal means of blessing. The Adaptation theme encompasses the sub-themes of treatment and care, family dynamics, spirituality, and social. The Mistakes theme encompasses the sub-themes of dependency on the child, dependency of the child, and allowing secondary gain. The parents demonstrated a willingness to accept and be aware of their children’s special condition, which facilitated the process of transformation and adaptation. In order for parents to undergo the transformation process more expeditiously, it is necessary to enhance their acceptance and awareness.

Global-scale evaluation of precipitation datasets for hydrological modelling

Abstract. Precipitation is the most important driver of the hydrological cycle, but it is challenging to estimate it over large scales from satellites and models. Here, we assessed the performance of six global and quasi-global high-resolution precipitation datasets (European Centre for Medium-Range Weather Forecasts (ECMWF) Reanalysis version 5 (ERA5), Climate Hazards group Infrared Precipitation with Stations version 2.0 (CHIRPS), Multi-Source Weighted-Ensemble Precipitation version 2.80 (MSWEP), TerraClimate (TERRA), Climate Prediction Centre Unified version 1.0 (CPCU), and Precipitation Estimation from Remotely Sensed Information using Artificial Neural Networks-Cloud Classification System-Climate Data Record (PERSIANN-CCS-CDR, hereafter PERCCDR) for hydrological modelling globally and quasi-globally. We forced the WBMsed global hydrological model with the precipitation datasets to simulate river discharge from 1983 to 2019 and evaluated the predicted discharge against 1825 hydrological stations worldwide, using a range of statistical methods. The results show large differences in the accuracy of discharge predictions when using different precipitation input datasets. Based on evaluation at annual, monthly, and daily timescales, MSWEP followed by ERA5 demonstrated a higher correlation (CC) and Kling–Gupta efficiency (KGE) than other datasets for more than 50 % of the stations, whilst ERA5 was the second-highest-performing dataset, and it showed the highest error and bias for about 20 % of the stations. PERCCDR is the least-well-performing dataset, with a bias of up to 99 % and a normalised root mean square error of up to 247 %. PERCCDR only show a higher KGE and CC than the other products for less than 10 % of the stations. Even though MSWEP provided the highest performance overall, our analysis reveals high spatial variability, meaning that it is important to consider other datasets in areas where MSWEP showed a lower performance. The results of this study provide guidance on the selection of precipitation datasets for modelling river discharge for a basin, region, or climatic zone as there is no single best precipitation dataset globally. Finally, the large discrepancy in the performance of the datasets in different parts of the world highlights the need to improve global precipitation data products.