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https://www.readbyqxmd.com/read/28318817/clinical-and-mutational-characteristics-of-duchenne-muscular-dystrophy-patients-based-on-a-comprehensive-database-in-south-china
#1
Dan-Ni Wang, Zhi-Qiang Wang, Lei Yan, Jin He, Min-Ting Lin, Wan-Jin Chen, Ning Wang
The development of clinical trials for Duchenne muscular dystrophy (DMD) in China faces many challenges due to limited information about epidemiological data, natural history and clinical management. To provide these detailed data, we developed a comprehensive database based on registered DMD patients from South China and analysed their clinical and mutational characteristics. The database included DMD registrants confirmed by clinical presentation, family history, genetic detection, prognostic outcome, and/or muscle biopsy...
February 21, 2017: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/28314659/diagnostic-and-prognostic-value-of-cmr-t1-mapping-in-patients-with-heart-failure-and-preserved-ejection-fraction
#2
Karl-Philipp Rommel, Christian Lücke, Philipp Lurz
Heart failure with preserved ejection fraction (HFpEF) presents a major challenge in modern cardiology. Although this syndrome is of increasing prevalence and is associated with unfavorable outcomes, treatment trials have failed to establish effective therapies. Currently, solutions to this dilemma are being investigated, including categorizing and characterizing patients more diversely to individualize treatment. In this regard, new imaging techniques might provide important information. Diastolic dysfunction is a diagnostic and pathophysiological cornerstone in HFpEF and is believed to be caused by systemic inflammation with the development of interstitial myocardial fibrosis and myocardial stiffening...
March 14, 2017: Revista Española de Cardiología
https://www.readbyqxmd.com/read/28303771/epidemiology-and-outcome-of-acute-kidney-injury-in-children-a-single-center-study
#3
Werner Keenswijk, Jill Vanmassenhove, Ann Raes, Evelyn Dhont, Johan VandeWalle
BACKGROUND: Information on the epidemiology of Acute Kidney Injury (AKI) in children is scarce. We performed a single center retrospective cohort study to analyze the incidence of AKI, the male/female ratio, the underlying etiology, and age at presentation. We also aimed to assess outcome measured by mortality, duration of PICU stay, and development of Chronic Kidney Disease (CKD). METHODS: Records were searched for children presenting with or developing AKI between 1st January 2008 and 1st January 2015...
March 17, 2017: Acta Clinica Belgica
https://www.readbyqxmd.com/read/28302739/changing-landscape-of-congenital-heart-disease
#4
Berto J Bouma, Barbara J M Mulder
Congenital heart disease is the most frequently occurring congenital disorder affecting ≈0.8% of live births. Thanks to great efforts and technical improvements, including the development of cardiopulmonary bypass in the 1950s, large-scale repair in these patients became possible, with subsequent dramatic reduction in morbidity and mortality. The ongoing search for progress and the growing understanding of the cardiovascular system and its pathophysiology refined all aspects of care for these patients. As a consequence, survival further increased over the past decades, and a new group of patients, those who survived congenital heart disease into adulthood, emerged...
March 17, 2017: Circulation Research
https://www.readbyqxmd.com/read/28302557/prevalence-and-prognostic-features-of-ecg-abnormalities-in-acute-stroke-findings-from-the-siren-study-among-africans
#5
Abiodun M Adeoye, Okechukwu S Ogah, Bruce Ovbiagele, Rufus Akinyemi, Vincent Shidali, Francis Agyekum, Akinyemi Aje, Oladimeji Adebayo, Joshua O Akinyemi, Philip Kolo, Lambert Tetteh Appiah, Henry Iheonye, Uwanuruochi Kelechukwu, Amusa Ganiyu, Taiwo O Olunuga, Onoja Akpa, Ojo Olakanmi Olagoke, Fred Stephen Sarfo, Kolawole Wahab, Samuel Olowookere, Adekunle Fakunle, Albert Akpalu, Philip B Adebayo, Kwadwo Nkromah, Joseph Yaria, Philip Ibinaiye, Godwin Ogbole, Aridegbe Olumayowa, Sulaiman Lakoh, Benedict Calys-Tagoe, Paul Olowoyo, Chukwuonye Innocent, Hemant K Tiwari, Donna Arnett, Osaigbovo Godwin, Bisi Ayotunde, Josephine Akpalu, Okeke Obiora, Odo Joseph, Adeleye Omisore, Carolyn Jenkins, Daniel Lackland, Lukman Owolabi, Suleiman Isah, Abdu H Dambatta, Morenikeji Komolafe, Andrew Bock-Oruma, Ezinne Sylvia Melikam, Lucius Chidiebere Imoh, Taofiki Sunmonu, Mulugeta Gebregziabher, Oluyemisi Olabisi, Kevin Armstrong, Ugochukwu U Onyeonoro, Emmanuel Sanya, Atinuke M Agunloye, Luqman Ogunjimi, Oyedunni Arulogun, Temitope H Farombi, Olugbo Obiabo, Reginald Obiako, Mayowa Owolabi
BACKGROUND: Africa has a growing burden of stroke with associated high morbidity and a 3-year fatality rate of 84%. Cardiac disease contributes to stroke occurrence and outcomes, but the precise relationship of abnormalities as noted on a cheap and widely available test, the electrocardiogram (ECG), and acute stroke outcomes have not been previously characterized in Africans. OBJECTIVES: The study assessed the prevalence and prognoses of various ECG abnormalities among African acute stroke patients encountered in a multisite, cross-national epidemiologic study...
March 13, 2017: Global Heart
https://www.readbyqxmd.com/read/28288937/incidence-of-fatal-snake-bite-in-australia-a-coronial-based-retrospective-study-2000-2016
#6
Ronelle Welton, Danny Liew, George Braitberg
INTRODUCTION: It has been over 20 years since a national review of recorded deaths from snake envenoming. The present study aimed to provide an updated review of the epidemiology of deaths from snake bites in Australia. METHODS: Deaths were identified from January 2000 to December 2016 from the National Coronial Information System. Cases identified due to snakes were extracted with data on coronial findings, autopsy and police records. RESULTS: Thirty five deaths (2...
March 10, 2017: Toxicon: Official Journal of the International Society on Toxinology
https://www.readbyqxmd.com/read/28287212/-the-pacemaker-and-implantable-cardioverter-defibrillator-registry-of-the-italian-association-of-arrhythmology-and-cardiac-pacing-annual-report-2015
#7
Alessandro Proclemer, Massimo Zecchin, Antonio D'Onofrio, Giuseppe Boriani, Giovanni Luca Botto, Domenico Facchin, Luca Rebellato, Marco Ghidina, Giulia Bianco, Emanuela Bernardelli, Elsa Pucher, Dario Gregori
BACKGROUND: The pacemaker (PM) and implantable cardioverter-defibrillator (ICD) Registry of the Italian Association of Arrhythmology and Cardiac Pacing (AIAC) monitors the main epidemiological data in real-world practice. The survey for the 2015 activity collects information about demographics, clinical characteristics, main indications for PM/ICD therapy and device types from the Italian collaborating centers. METHODS: The Registry collects prospectively national PM and ICD implantation activity on the basis of European cards...
January 2017: Giornale Italiano di Cardiologia
https://www.readbyqxmd.com/read/28279414/management-of-heart-failure-in-cancer-patients-and-cancer-survivors
#8
REVIEW
Jose Emanuel Finet
The number of cancer survivors increases annually, because of advances in detection and treatment, and the aging and growth of the population. This increase has brought a concomitant increase in morbidity and mortality from other conditions related to the adverse effects of cancer treatments. Cardiovascular diseases, and in particular left ventricular dysfunction and heart failure, are among the most significant of these. There are no unified and universally accepted evidence-based practice guidelines on the management of heartfailure in this population...
April 2017: Heart Failure Clinics
https://www.readbyqxmd.com/read/28277756/in-hospital-cardiac-arrest-epidemiology-in-a-mature-rapid-response-system
#9
Daryl Jones, Inga Mercer, Melodie Heland, Karen Detering, Sam Radford, Graeme Hart, David O'Donnell, Rinaldo Bellomo
An audit examined the epidemiology of in-hospital cardiac arrests 5 years after a rapid response system was introduced, exploring the frequency of arrests in monitored and unmonitored areas. Details of the initial cardiac rhythm and what proportion of events were preceded by a medical emergency team call were also assessed.
March 2, 2017: British Journal of Hospital Medicine
https://www.readbyqxmd.com/read/28274521/cavopulmonary-anastomosis-during-same-hospitalization-as-stage-1-norwood-hybrid-palliative-surgery
#10
Sachin D Tadphale, Xinyu Tang, Nahed O ElHassan, Brandon Beam, Parthak Prodhan
BACKGROUND: Limited literature has examined characteristics of infants with hypoplastic left heart syndrome (HLHS) who remain hospitalized during the interstage period. We described their epidemiologic characteristics, in-hospital outcomes, and identified risk factors that predict the need for superior cavopulmonary anastomosis (SCPA) during the same hospitalization. METHODS: This retrospective multicenter database analysis included infants with HLHS who underwent stage 1 palliation from 2004 through 2013...
March 5, 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28259455/variation-in-extubation-failure-rates-after-neonatal-congenital-heart-surgery-across-pediatric-cardiac-critical-care-consortium-hospitals
#11
Brian D Benneyworth, Christopher W Mastropietro, Eric M Graham, Darren Klugman, John M Costello, Wenying Zhang, Michael Gaies
OBJECTIVE: In a multicenter cohort of neonates recovering from cardiac surgery, we sought to describe the epidemiology of extubation failure and its variability across centers, identify risk factors, and determine its impact on outcomes. METHODS: We analyzed prospectively collected clinical registry data on all neonates undergoing cardiac surgery in the Pediatric Cardiac Critical Care Consortium database from October 2013 to July 2015. Extubation failure was defined as reintubation less than 72 hours after the first planned extubation...
February 4, 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28259228/cha2ds2-vasc-score-a-predictor-of-thromboembolic-events-and-mortality-in-patients-with-an-implantable-monitoring-device-without-atrial-fibrillation
#12
Christine Parsons, Salma I Patel, Stephen Cha, Win-Kuang Shen, Santosh Desai, Alanna M Chamberlain, Sushil Allen Luis, Maria I Aguilar, Bart M Demaerschalk, Farouk Mookadam, Fadi Shamoun
OBJECTIVE: To determine if the CHA2DS2-VASc score (congestive heart failure, hypertension, age ≥75 years, diabetes mellitus, stroke or transient ischemic attack, vascular disease, age 65-74 years, sex category) predicts thromboembolism and death in patients without atrial fibrillation in a population with implantable cardiac monitoring devices. PATIENTS AND METHODS: A retrospective review utilizing the Rochester Epidemiology Project research infrastructure was conducted to evaluate the CHA2DS2-VASc tool as a predictor of mortality and ischemic stroke, transient ischemic attack, or systemic embolism in patients without atrial fibrillation...
March 2017: Mayo Clinic Proceedings
https://www.readbyqxmd.com/read/28256370/neonatal-dilated-cardiomyopathy
#13
Paulo Soares, Gustavo Rocha, Susana Pissarra, Henrique Soares, Filipa Flôr-de-Lima, Sandra Costa, Cláudia Moura, Sofia Dória, Hercília Guimarães
Cardiomyopathies are rare diseases of the heart muscle, of multiple causes, that manifest with various structural and functional phenotypes but are invariably associated with cardiac dysfunction. Dilated cardiomyopathy is the commonest cardiomyopathy in children, and the majority present before one year of age. Its etiology may be acquired or genetic. Myocarditis is an important cause and is responsible for the majority of acquired cases. Inherited (familial) forms of dilated cardiomyopathy may occur in 25-50% of patients...
March 2017: Portuguese Journal of Cardiology: An Official Journal of the Portuguese Society of Cardiology
https://www.readbyqxmd.com/read/28254555/epidemiology-and-outcomes-of-arterial-ischemic-stroke-in-children-the-canadian-pediatric-ischemic-stroke-registry
#14
Gabrielle A deVeber, Adam Kirton, Frances A Booth, Jerome Y Yager, Elaine C Wirrell, Ellen Wood, Michael Shevell, Ann-Marie Surmava, Patricia McCusker, M Patricia Massicotte, Daune MacGregor, E Athen MacDonald, Brandon Meaney, Simon Levin, Bernard G Lemieux, Lawrence Jardine, Peter Humphreys, Michèle David, Anthony K C Chan, David J Buckley, Bruce H Bjornson
BACKGROUND: Pediatric arterial ischemic stroke remains incompletely understood. Population-based epidemiological data inform clinical trial design but are scant in this condition. We aimed to determine age-specific epidemiological characteristics of arterial ischemic stroke in neonates (birth to 28 days) and older children (29 days to 18 years). METHODS: We conducted a 16-year, prospective, national population-based study, the Canadian Pediatric Ischemic Stroke Registry, across all 16 Canadian acute care children's hospitals...
January 26, 2017: Pediatric Neurology
https://www.readbyqxmd.com/read/28241746/factors-influencing-mortality-in-abdominal-solid-organ-transplant-recipients-with-multidrug-resistant-gram-negative-bacteremia
#15
Bingbing Qiao, Jianzhen Wu, Qiquan Wan, Sheng Zhang, Qifa Ye
BACKGROUND: Although multidrug-resistant (MDR) gram-negative bacteremia (GNB) has been recognized as an important cause of morbidity and mortality among abdominal solid organ transplant (ASOT) recipients, there are no data on its prognostic factors after an interim standard definition of MDR was proposed in 2012. The objective of this study was to describe the epidemiology, microbiology, and predictors of infection-related 30-day mortality in ASOT recipients with MDR GNB. METHODS: We performed a retrospective, double-center analysis of ASOT patients with MDR GNB over a 13-year study period...
February 27, 2017: BMC Infectious Diseases
https://www.readbyqxmd.com/read/28237535/a-small-cohort-omega-3-pufa-supplement-study-implications-of-stratifying-according-to-lipid-membrane-incorporation-in-cardiac-surgical-patients
#16
Wendy T K Ip, Chanchal Chandramouli, Julian A Smith, Peter L McLennan, Salvatore Pepe, Lea M D Delbridge
BACKGROUND: Epidemiological studies and randomised clinical trials (RCTs) report disparate findings in relation to omega-3 polyunsaturated fatty acids (n-3 PUFA) benefit for cardiac patients. With RCTs interpretation is potentially confounded by background n-3 PUFA intake. The goal of this pilot, small cohort, pre-surgical supplementation study was to evaluate postoperative atrial fibrillation (AF) and cardiac molecular expression profiles employing two data analysis approaches - by treatment randomisation and by stratification using measured n-3 PUFA...
February 6, 2017: Heart, Lung & Circulation
https://www.readbyqxmd.com/read/28229201/-sudden-cardiac-death-epidemiology-pathophysiology-and-risk-stratification
#17
B Rudic, E Tülümen, V Liebe, J Kuschyk, I Akin, M Borggrefe
Sudden cardiac death (SCD) remains a major public health burden despite revolutionary progress in the last three decades in the treatment of ventricular tachyarrhythmia with the use of implantable cardioverter defibrillator (ICD) therapy. Survivors of sudden cardiac arrest are at high risk for recurrent tachyarrhythmia events. Early recognition of low left ventricular ejection fractions (≤35%) as a strong predictor of mortality and the causal association between ventricular tachyarrhythmia and SCD has led to a significant development of not only pharmacological antiarrhythmic therapy but also device-based prevention of SCD...
February 22, 2017: Herz
https://www.readbyqxmd.com/read/28229199/-coronary-artery-disease-interventional-and-operative-therapeutic-options-after-cardiac-arrest
#18
M Behnes, K Mashayekhi, M Borggrefe, I Akin
Coronary artery disease (CAD) represents a common structural cause for developing cardiac arrest in older patients, whereas in young adults cardiac arrest is more often caused by cardiomyopathies and cardiac channelopathies. A structural heart disease is known in almost 50% of patients prior to cardiac arrest. The present review outlines current interventional and operative therapeutic options for patients surviving cardiac arrest. The focus is on associations between epidemiological data on the incidence of malignant arrhythmias causing cardiac arrest depending on the presence or absence of CAD...
February 22, 2017: Herz
https://www.readbyqxmd.com/read/28223004/sudden-cardiac-death-among-hemodialysis-patients
#19
Melissa S Makar, Patrick H Pun
Hemodialysis patients carry a large burden of cardiovascular disease; most onerous is the high risk for sudden cardiac death. Defining sudden cardiac death among hemodialysis patients and understanding its pathogenesis are challenging, but inferences from the existing literature reveal differences between sudden cardiac death among hemodialysis patients and the general population. Vascular calcifications and left ventricular hypertrophy may play a role in the pathophysiology of sudden cardiac death, whereas traditional cardiovascular risk factors seem to have a more muted effect...
February 17, 2017: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/28219933/akt-signalling-as-a-mediator-of-cardiac-adaptation-to-low-birth-weight
#20
Kimberley Cw Wang, Kimberley J Botting, Song Zhang, Caroline McMillen, Doug A Brooks, Janna L Morrison
Intrauterine insults, such as poor nutrition and placental insufficiency, can alter cardiomyocyte development and this can have significant long-term implications for heart health. Consequently, epidemiological studies have shown that low birth weight babies have an increased risk of death from cardiovascular disease in adult life. In addition, intrauterine growth restriction can result in increased left ventricular hypertrophy, which is the strongest predictor for poor health outcomes in cardiac patients. The mechanisms responsible for these associations are not clear, but a suboptimal intrauterine environment can program alternative expression of genes such as cardiac IGF-2/H19, IGF-2R and AT1R through either an increase or decrease in DNA methylation or histone acetylation at specific loci...
February 20, 2017: Journal of Endocrinology
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