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Cardiomyopathies

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https://www.readbyqxmd.com/read/28822817/rosiglitazone-promotes-cardiac-hypertrophy-and-alters-chromatin-remodeling-in-isolated-cardiomyocytes
#1
Lama Fawaz Pharaon, Naglaa Fathi El-Orabi, Muhammad Kunhi, Nadya Al Yacoub, Salma Mahmoud Awad, Coralie Poizat
Rosiglitazone is an anti-diabetic agent that raised a major controversy over its cardiovascular adverse effects. There is in vivo evidence that Rosiglitazone promotes cardiac hypertrophy by PPAR -γ-independent mechanisms. However, whether Rosiglitazone directly alters hypertrophic growth in cardiac cells is unknown. Chromatin remodeling by histone post-translational modifications has emerged as critical for many cardiomyopathies. Based on these observations, this study was initiated to investigate the cardiac hypertrophic effect of Rosiglitazone in a cellular model of primary neonatal rat cardiomyocytes (NRCM)...
August 16, 2017: Toxicology Letters
https://www.readbyqxmd.com/read/28822689/cholesterol-lowering-gene-therapy-counteracts-the-development-of-non-ischemic-cardiomyopathy-in-mice
#2
Ilayaraja Muthuramu, Ruhul Amin, Andrey Postnov, Mudit Mishra, Joseph Pierre Aboumsallem, Tom Dresselaers, Uwe Himmelreich, Paul P Van Veldhoven, Olivier Gheysens, Frank Jacobs, Bart De Geest
A causal role of hypercholesterolemia in non-ischemic heart failure has never been demonstrated. Adeno-associated viral serotype 8 (AAV8)-low-density lipoprotein receptor (AAV8-LDLr) gene transfer was performed in LDLr-deficient mice without and with pressure overload induced by transverse aortic constriction (TAC). AAV8-LDLr gene therapy resulted in an 82.8% (p < 0.0001) reduction of plasma cholesterol compared with controls. Mortality rate was lower (p < 0.05) in AAV8-LDLr TAC mice compared with control TAC mice (hazard ratio for mortality 0...
August 1, 2017: Molecular Therapy: the Journal of the American Society of Gene Therapy
https://www.readbyqxmd.com/read/28822653/titin-truncating-variants-increase-the-risk-of-cardiovascular-death-in-patients-with-hypertrophic-cardiomyopathy
#3
Ce Zhang, Hongju Zhang, Guixin Wu, Xiaoliang Luo, Channa Zhang, Yubao Zou, Hu Wang, Rutai Hui, Jizheng Wang, Lei Song
BACKGROUND: Titin-truncating variants (TTNtv) have been detected in a variety of cardiomyopathies and represent the most common cause of dilated cardiomyopathy. However, their significance in hypertrophic cardiomyopathy (HCM) is still unclear. METHODS: The titin gene (TTN) was sequenced for truncating variants in a cohort of 529 Chinese patients with HCM and 307 healthy controls. Baseline and follow-up clinical data (for 4.7 ± 3.2 years) from these patients were obtained...
June 8, 2017: Canadian Journal of Cardiology
https://www.readbyqxmd.com/read/28822614/heterogeneity-in-a-large-pedigree-with-danon-disease-implications-for-pathogenesis-and-management
#4
Jonathan C P Roos, Matthew J Daniels, Elizabeth Morris, Hanna I Hyry, Timothy M Cox
BACKGROUND: Danon disease is an X-linked disturbance of autophagy manifesting with cognitive impairment and disordered heart and skeletal muscle. After a period of relative stability, patients deteriorate rapidly and may quickly become ineligible for elective heart transplantation - the only life-saving therapy. METHODS: We report a large pedigree with diverse manifestations of Danon disease in hemizygotes and female heterozygotes. RESULTS: Malignant cardiac arrhythmias requiring amiodarone treatment induced thyroid disease in two patients; intractable thyrotoxicosis, which enhances autophagy, caused the death of a 21year-old man...
June 21, 2017: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/28822388/outcomes-following-noncardiac-surgery-in-patients-with-ventricular-assist-devices-a-single-center-experience
#5
Shawna M Kettyle, Nikhil L Chervu, Appajosula Sarada Rao, Salaam Sadi, David Majure, Jack A Sava, Laura S Johnson
the prevalence of ventricular assist devices (VADs) is increasing as advanced cardiac therapies progress. These patients commonly require non-cardiac surgical procedures (NCS), although data are scant regarding the safety, timing, and operations that may safely be performed. We aim to describe our experience with VAD patients undergoing NCS. We retrospectively reviewed records on patients who underwent NCS after VAD implantation between 2013 and 2015 at a single Joint Commission-accredited VAD institution. Data collection included demographics, ischemic cardiomyopathy or nonischemic cardiomyopathy, operative details, and perioperative anticoagulation management and outcomes...
August 1, 2017: American Surgeon
https://www.readbyqxmd.com/read/28821969/interleukin-1-receptor-like-1-protein-st2-is-a-potential-biomarker-for-cardiomyopathy-in-duchenne-muscular-dystrophy
#6
Julia Anderson, Haeri Seol, Heather Gordish-Dressman, Yetrib Hathout, Christopher F Spurney
Duchenne muscular dystrophy (DMD) is a rare, fatal X-linked disorder characterized by the lack of dystrophin, a key sarcolemma muscle protein. Cardiac failure is a significant cause of death in DMD subjects. The purpose of our research was to identify potential cardiac serum biomarkers associated with DMD cardiomyopathy. This is an observational, case-controlled study using subjects from the CINRG DMD natural history study with cardiomyopathy (ejection fraction (EF) <55%; shortening fraction (SF) <28%), subjects without cardiomyopathy (EF ≥ 55%; SF ≥ 28%) compared to normal healthy volunteer subjects...
August 18, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28821963/myocardial-regenerative-therapy-using-a-scaffold-free-skeletal-muscle-derived-cell-sheet-in-patients-with-dilated-cardiomyopathy-even-under-a-left-ventricular-assist-device-a-safety-and-feasibility-study
#7
Yasushi Yoshikawa, Shigeru Miyagawa, Koichi Toda, Atsuhiro Saito, Yasushi Sakata, Yoshiki Sawa
BACKGROUND AND PURPOSE: Despite promising experimental results, clinically, intramyocardial myoblast injection failed to reverse remodeling and it induced arrhythmogenicity. In contrast, scaffold-free skeletal muscle-derived cell (SC) sheets attenuated cardiac dysfunction and arrhythmogenicity via paracrine effects. We report the first clinical trial of SC sheet implantation (SCSI) conducted in four patients with dilated cardiomyopathy (DCM) supported by a left ventricular assist device (LVAD)...
August 18, 2017: Surgery Today
https://www.readbyqxmd.com/read/28821761/enhanced-therapeutic-effects-of-human-ips-cell-derived-cardiomyocyte-by-combined-cell-sheets-with-omental-flap-technique-in-porcine-ischemic-cardiomyopathy-model
#8
Masashi Kawamura, Shigeru Miyagawa, Satsuki Fukushima, Atsuhiro Saito, Kenji Miki, Shunsuke Funakoshi, Yoshinori Yoshida, Shinya Yamanaka, Tatsuya Shimizu, Teruo Okano, Takashi Daimon, Koichi Toda, Yoshiki Sawa
Transplant of human induced pluripotent stem cell derived cardiomyocytes (hiPS-CMs) cell-sheet is a promising approach for treating ischemic cardiomyopathy (ICM). However, poor blood supply to the transplanted cell-sheet is a concern related to the effectiveness and durability of the treatment. Herein, we hypothesized that the combined the omentum flap might enhance survival and the therapeutic effects of hiPS-CM cell-sheet transplant for ICM treatment. Treatment by Wnt signaling molecules in hiPS cells produced hiPS-CMs, which were magnetically labeled by superparamagnetic iron oxide (SPIO), followed by culture in the thermoresponsive dishes to generate hiPS-CMs cell-sheets...
August 18, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28821499/primary-ventricular-tachycardia-in-paediatric-population-in-a-tertiary-centre
#9
Shuenn-Nan Chiu, Wei-Lun Wu, Chun-Wei Lu, Wei-Chieh Tseng, Kun-Lang Wu, Jou-Kou Wang, Mei-Hwan Wu
OBJECTIVE: To delineate the outcome of ventricular tachycardia (VT) in the paediatric population. METHODS: Patients who developed sustained VT between the ages of 0 and 18 years in a referral centre from 1991 to 2015 were enrolled. RESULTS: A total of 116 patients (67 male/49 female) had documented VT, and 53 (46%) had associated heart disease, namely cardiomyopathy in 20 (17%), structural heart disease in 19 (16%) and channelopathy in 14 (12%), and some of them presented with two types of associated heart disease...
August 18, 2017: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/28820736/long-qt-syndrome-diagnosed-in-two-sisters-with-propionic-acidemia-a-case-report
#10
Ensar Duras, Ahmet İrdem, Ozan Özkaya
Propionic acidemia (PA) is a rare autosomal recessive metabolic disorder caused by deficiency of the mitochondrial enzyme propionyl-CoA carboxylase (PCC). This disorder mostly progresses with episodes of metabolic acidosis. Cardiomyopathy is among the cardiac complications known to occur during metabolic decompensation episodes. However, several recent papers emphasized the association of PA and long QT syndrome (LQTS) which may lead to extremely serious and fatal consequences. In this report, we describe two sisters with PA who have prolonged QT duration that were incidentally detected in an outpatient setting...
August 18, 2017: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/28820624/retinopathy-and-optic-atrophy-expanding-the-phenotypic-spectrum-of-pathogenic-variants-in-the-aars2-gene
#11
Jason H Peragallo, Stephanie Keller, Marjo S van der Knaap, Bruno P Soares, Suma P Shankar
BACKGROUND: Optic atrophy may be the sequela of optic nerve injury due to any insult, including isolated and syndromic genetic diseases. Alanyl-tRNA synthetase 2 (AARS2) pathogenic variants have been reported to cause leukodystrophy with ovarian failure, and cardiomyopathy (#615889) as well as combined oxidative phosphorylation deficiency-8 (#614096). We report a young child who presented with decreased vision due to optic atrophy and was found to harbor missense variants in the AARS2 gene expanding the phenotypic expression of the AARS2 gene...
August 18, 2017: Ophthalmic Genetics
https://www.readbyqxmd.com/read/28819957/four-dimensional-flow-magnetic-resonance-imaging-in-hypertrophic-obstructive-cardiomyopathy
#12
Mateusz Śpiewak, Agata Kubik, Mariusz Kłopotowski, Konrad Werys, Magdalena Marczak
No abstract text is available yet for this article.
2017: Kardiologia Polska
https://www.readbyqxmd.com/read/28819918/network-analysis-of-se-and-zn-related-proteins-in-the-serum-proteomics-expression-profile-of-the-endemic-dilated-cardiomyopathy-keshan-disease
#13
Sen Wang, Yanyan Lv, Yingting Wang, Peiru Du, Wuhong Tan, Mikko J Lammi, Xiong Guo
Keshan disease (KD) is an endemic cardiomyopathy with high mortality. Selenium (Se) and zinc (Zn) deficiencies are closely related to KD. The molecular mechanism of KD pathogenesis is still unclear. There are only few studies on the interaction of trace elements and proteins associated with the pathogenesis of KD. In this study, isobaric tags for relative and absolute quantitation (iTRAQ)-coupled two-dimensional liquid chromatography tandem mass spectrometry (2DLC-MS/MS) technique analysis was used to analyze the differential expression of proteins from serum samples...
August 18, 2017: Biological Trace Element Research
https://www.readbyqxmd.com/read/28819571/incidental-finding-of-an-undiagnosed-coarctation-of-the-aorta-causing-dilated-cardiomyopathy-in-an-adult
#14
Abdalla Ibrahim, Zahir Satti, Ronan Curtin
31-year-old male with no past medical history apart from high blood pressure noted by GP one week prior to admission presented with a three-week history of a flu-like illness and symptoms of heart failure with severe global left ventricular dilation and dysfunction on Transthoracic Echocardiography (TTE). Two weeks following admission he complained of left arm pain and CT upper limb confirmed embolic occlusion of the left brachial artery and incidental severe coarctation of the proximal descending aorta after the origin of the left subclavian artery...
2017: Case Reports in Cardiology
https://www.readbyqxmd.com/read/28819491/-psychiatric-and-psychological-evaluation-of-tako-tsubo-syndrome-about-a-case
#15
Ghizlane Slimani, Hicham Lakbiri, Fatima Zahra Sekkat
Stress cardiomyopathy or Tako Tsubo cardiomyopathy is a cardiac pathology evoking acute coronary syndrome characterized by electrocardiographic signs, cardiac enzyme elevation and no obstructive coronary lesions. It generally affects postmenopausal women and it usually occurs after periods of intense stress. Disease onset is widely variable, ranging from anginal pain (most common) to cardiogenic shock. Exact pathophysiological mechanism continues to be debated. Various hypotheses have been posited. Abrupt elevation of adrenaline levels appears to be the most credible...
2017: Pan African Medical Journal
https://www.readbyqxmd.com/read/28819361/unilateral-headache-status-after-intra-aortic-balloon-pump-placement
#16
Garret M Weber, Alan L Gass, Shalvi B Parikh
INTRODUCTION: Intra-aortic balloon pump (IABP) counterpulsation is a catheter-based treatment for coronary artery disease and decompensated heart failure to increase coronary blood flow and improve cardiac output. IABP is generally well tolerated, and complications are usually related to peripheral vasculature or red blood cell and platelet consumption. The usual insertion site via femoral artery renders the patient bedbound. Recently, axillary artery has been used in patients with atherosclerotic peripheral vascular disease and documented small arteries or in those awaiting transplant to ensure ambulation and prevent deconditioning...
2017: Case Reports in Medicine
https://www.readbyqxmd.com/read/28819303/automated-segmentation-of-light-sheet-fluorescent-imaging-to-characterize-experimental-doxorubicin-induced-cardiac-injury-and-repair
#17
René R Sevag Packard, Kyung In Baek, Tyler Beebe, Nelson Jen, Yichen Ding, Feng Shi, Peng Fei, Bong Jin Kang, Po-Heng Chen, Jonathan Gau, Michael Chen, Jonathan Y Tang, Yu-Huan Shih, Yonghe Ding, Debiao Li, Xiaolei Xu, Tzung K Hsiai
This study sought to develop an automated segmentation approach based on histogram analysis of raw axial images acquired by light-sheet fluorescent imaging (LSFI) to establish rapid reconstruction of the 3-D zebrafish cardiac architecture in response to doxorubicin-induced injury and repair. Input images underwent a 4-step automated image segmentation process consisting of stationary noise removal, histogram equalization, adaptive thresholding, and image fusion followed by 3-D reconstruction. We applied this method to 3-month old zebrafish injected intraperitoneally with doxorubicin followed by LSFI at 3, 30, and 60 days post-injection...
August 17, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28819214/hiv-1-nef-induced-cardiotoxicity-through-dysregulation-of-autophagy
#18
Manish K Gupta, Rafal Kaminski, Brian Mullen, Jennifer Gordon, Tricia H Burdo, Joseph Y Cheung, Arthur M Feldman, Muniswamy Madesh, Kamel Khalili
Cardiovascular disease is a leading cause of co-morbidity in HIV-1 positive patients, even those in whom plasma virus levels are well-controlled. The pathogenic mechanism of HIV-1-associated cardiomyopathy is unknown, but has been presumed to be mediated indirectly, owing to the absence of productive HIV-1 replication in cardiomyocytes. We sought to investigate the effect of the HIV-1 auxiliary protein, Nef, which is suspected of extracellular release by infected CD4+ T cells on protein quality control and autophagy in cardiomyocytes...
August 17, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28818478/mutations-in-ddhd1-encoding-a-phospholipase-a1-is-a-novel-cause-of-retinopathy-and-neurodegeneration-with-brain-iron-accumulation
#19
Rodolphe Dard, Claire Meyniel, Valérie Touitou, Giovanni Stevanin, Foudil Lamari, Alexandra Durr, Claire Ewenczyk, Fanny Mochel
Defects of phospholipids remodelling and synthesis are inborn errors of metabolism responsible for various clinical presentations including spastic paraplegia, retinopathy, optic atrophy, myo- and cardiomyopathies, and osteo-cutaneous manifestations. DDHD1 encodes a phospholipase A1, which is involved in the remodelling of phospholipids. We previously described a relatively pure hereditary spastic paraplegia (HSP) phenotype associated with mutations in DDHD1. Here we report a complex form of HSP associated with retinal dystrophy and a pattern of neurodegeneration with brain iron accumulation (NBIA) on brain MRI, due to a novel homozygous mutation in DDHD1...
August 14, 2017: European Journal of Medical Genetics
https://www.readbyqxmd.com/read/28818336/association-between-premature-ventricular-complexes-during-exercise-long-term-occurrence-of-life-threatening-arrhythmia-and-mortality
#20
Jorge Aviles-Rosales, Hermes Ilarraza-Lomeli, Marianna Garcia-Saldivia, Jessica Rojano-Castillo, Maria-Dolores Rius-Suarez, Juan-Pablo Nunez-Urquiza, Pedro Iturralde
OBJECTIVE: Exercise-induced premature ventricular complexes (EiPVCs) are often considered as benign arrhythmias, nevertheless they are associated with a high risk of all-cause death in general healthy population. However, an intermediate pathophysiological process remains unclear, particularly in patients with known cardiovascular disease. The aim of this study was to find an association between EiPVCs, the occurrence of life-threatening ventricular arrhythmias (LACO) and all-cause mortality in patients with cardiovascular disease...
August 14, 2017: Archivos de Cardiología de México
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