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Restrictive cardiomyopathy (RCM) is a rare cardiomyopathy in children, and its prognosis until now, has been poor. Recently some sarcomeric mutations have been reported as disease-causing genes of RCM. However, the genotype-phenotype correlation is not fully understood. Additionally, prognostic factors including sudden death in patients with RCM have not been elucidated. We report our experience in treating twin sisters with RCM or hypertrophic cardiomyopathy with RCM phenotype, both carriers of the same mutation in TNNI3, which encodes one of the major sarcomeric proteins in myofibrils. They were both diagnosed with RCM by cardiac catheterization at the age of 11 years. Despite appropriate follow-up and medical treatment, one died suddenly at the age of 11 years and the other also died at the age of 15 years due to heart failure while awaiting heart transplantation. In addition to our cases, other reports of younger fatal cases with RCM carrying TNNI3 mutations may suggest it as one of the prognostic factors. Genetic diagnosis is important in the clinical diagnosis, management, and treatment of cardiomyopathy. .The titration of cardioprotective agents is essential for successful treatment of heart failure (HF) patients with reduced left ventricular ejection fraction. However, hypotension is one of the limiting factors for titration. Ivabradine reduces heart rate without compromising systolic function by prolonging diastolic filling time. Herein two cases of dilated cardiomyopathy (DCM) are presented in which ivabradine improved blood pressure (BP)-limited tolerability and allowed for further titration of cardioprotective agents. In both cases, the introduction of ivabradine raised the BP, which permitted further increase of the dose of renin-angiotensin system inhibitors or beta-blockers. One major hypothesized mechanism of ivabradine-induced BP elevation has been postulated to be an increase in stroke volume due to prolonged ventricular diastolic filling time. However, ivabradine is not expected to increase BP for all HF patients. In those with small and poorly compliant ventricles with severe diastolic or restricted dysfunction, decreased heart rate and prolonged diastole may excessively suppress compensatory mechanisms, and thus may not lead to increased cardiac output and BP. In contrast, ivabradine potentially increases BP and improves BP-limited tolerability of cardioprotective agents in DCM patients with a large and compliant heart. In addition, subsequent titration of cardioprotective agents may provide additional cardiac reverse remodeling. Learning objective Ivabradine is usually used for heart failure patients with reduced ejection fraction when the tolerability of cardioprotective agents is maximized. This agent has no direct cardiac contractility-suppressing action. It potentially increases blood pressure and improves tolerability of cardioprotective agents in patients with a large and compliant heart such as dilated cardiomyopathy. Furthermore, subsequent titration of cardioprotective agents may provide additional cardiac reverse remodeling.We report an unusual case of heart failure due to massive myocardial calcification related to a rare combination of idiopathic mitral annular calcification, myocardial calcification of the left ventricular septum and the inferior wall without other predisposing factors, such as previous myocardial infarction, ventricular aneurysms, myocarditis, rheumatic heart disease, tuberculosis, chronic renal failure, or systemic metabolic disease (sarcoidosis or primary hyperoxaluria). The related restrictive pattern of diastolic filling of the left ventricle could explain this unusual case of heart failure with preserved ejection fraction. .Is it true that parents always prioritize educational effectiveness when selecting childcare services? The current study identified the potential requirements of dual-income parents toward social robots' diverse childcare functions (e.g., socialization, education, entertainment, and consultation). The results revealed that parental attitudes toward robots were made more positive by all the childcare functions of robots except for their educational features. Furthermore, parents' expectations of childcare functions varied based on their parenting characteristics. Stem Cells inhibitor Spectral clustering analysis identified distinctive parenting styles (e.g., family-oriented, work-oriented, noninterventional, and dominant), and multigroup structural equation modeling suggested that the impact of robots' socialization function was significant in all parent groups, while other childcare functions exerted limited influence according to specific parenting styles. In addition, children's characteristics were found to alter parents' preferences for each childcare function. These results offer practical implications for the early adoption of childcare robots through predetermining parents' acceptability based on their specific parenting characteristics.The preventive and cautionary measures taken by the UAE and Abu Dhabi governments to reduce the spread of the coronavirus disease (COVID-19) and promote social distancing have led to a reduction of mobility and a modification of economic and social activities. This paper provides statistical analysis of the air quality data monitored by the Environment Agency - Abu Dhabi (EAD) during the first 10 months of 2020, comparing the different stages of the preventive measures. Ground monitoring data is compared with satellite images and mobility indicators. The study shows a drastic decrease during lockdown in the concentration of the gaseous pollutants analysed (NO2, SO2, CO, and C6H6) that aligns with the results reported in other international cities and metropolitan areas. However, particulate matter (PM10 and PM2.5) averaged concentrations followed a markedly different trend from the gaseous pollutants, indicating a larger influence from natural events (sand and dust storms) and other anthropogenic sources. The ozone (O3) levels increased during the lockdown, showing the complexity of O3 formation. The end of lockdown led to an increase of the mobility and the air pollution; however, air pollutant concentrations remained in lower levels than during the same period of 2019. The results in this study show the large impact of human activities on the quality of air and present an opportunity for policymakers and decision-makers to design stimulus packages to overcome the economic slow-down, with strategies to accelerate the transition to resilient, low-emission economies and societies more connected to the nature that protect human health and the environment.
The online version contains supplementary material available at 10.1007/s11869-021-01000-2.
The online version contains supplementary material available at 10.1007/s11869-021-01000-2.
Homepage: https://www.selleckchem.com/products/GDC-0449.html
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