Prevalence and cardiac phenotype of patients with aphospholamban mutation
NETHERLANDS HEART JOURNAL
Authors: Hof, I. E.; van der Heijden, J. F.; Kranias, E. G.; Sanoudou, D.; de Boer, R. A.; van Tintelen, J. P.; van der Zwaag, P. A.; Doevendans, P. A.
Abstract
Pathogenic mutations in the phospholamban (PLN) gene may give rise to inherited cardiomyopathies due to its role in calcium homeostasis. Several PLN mutations have been identified, with the R14del mutation being the most prevalent cardiomyopathy-related mutation in the Netherlands. It is present in patients diagnosed with arrhythmogenic cardiomyopathy as well as dilated cardiomyopathy. Awareness of the phenotype of this PLN mutation is of great importance, since many carriers remain to be identified. Patients with the R14del mutation are characterised by older age at onset, low-voltage electrocardiograms and ahigh frequency of ventricular arrhythmias. Additionally, these patients have apoor prognosis often with left ventricular dysfunction and early-onset heart failure. Therefore, when there is asuspicion of aPLN mutation, cardiac and genetic screening is strongly recommended.
The impact of probability of electricity price spike and outside temperature to define total expected cost for air conditioning
ENERGY
Authors: Marwan, Marwan
Abstract
The purpose of this paper is to address the problems experienced by customers in the utilisation of electrical energy for air conditioners (ACs). According to the proposed schema in this paper, the customer will be able to evaluate the impacts of a probable electricity price spike and the outside temperature (Tout) to calculate the total expected electricity cost for an AC (TEC). The aim of this paper is to show how consumers can estimate the TEC for any temperature. In this research, a model considering two types of price spikes (PS) is developed, namely: short-duration and long-duration spikes. To evaluate and examine this model, spike durations of half hour, one hour, and one and half hour were simulated to determine TEC. This proposed schema also examines how the control system applies a pre-cooling method if there is a substantial risk of PS. The results present possible savings on the electrical energy consumption when the consumer applies this method to anticipate spike events. This model is tested considering the demand and market price curves of electricity in South Sulawesi, which were published by the Indonesian State Electricity Company (called PLN=Perusahaan Listrik Negara) and the value of Tout in the Makassar area. (C) 2020 Elsevier Ltd. All rights reserved.
COA
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