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https://www.readbyqxmd.com/read/28334757/perturbations-in-cell-signaling-elicit-early-cardiac-defects-in-mucopolysaccharidosis-type-ii
#1
Roberto Costa, Andrea Urbani, Marika Salvalaio, Stefania Bellesso, Domenico Cieri, Ilaria Zancan, Mirella Filocamo, Paolo Bonaldo, Ildiko Szabò, Rosella Tomanin, Enrico Moro
Morphogens release and activity can be negatively affected by an impaired glycosaminoglycans (GAGs) turnover and proteoglycans assembly in the extracellular matrix, leading to altered tissue morphogenesis. In this work, we show that loss of Iduronate-2-sulfatase (IDS) activity, affecting GAGs catabolism and responsible for a life-threatening valvulopathy in mucopolysaccharidosis type II (MPSII), triggers early Sonic Hedgehog (Shh) and Wnt/β-catenin signaling defects, leading to aberrant heart development and atrioventricular valve formation in a zebrafish model...
February 21, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28290006/exercise-testing-and-stress-imaging-in-mitral-valve-disease
#2
REVIEW
Damien Voilliot, Patrizio Lancellotti
Mitral valve disease represented by mitral stenosis and mitral regurgitation is the second most frequent valvulopathy. Mitral stenosis leads to an increased left atrial pressure whereas mitral regurgitation leads to an increased left atrial pressure associated with a volume overload. Secondary to an upstream transmission of this overpressure, both mitral stenosis and regurgitation lead to pulmonary hypertension and right heart failure. In addition, mitral regurgitation also leads to left ventricular dilatation and dysfunction with left heart failure...
March 2017: Current Treatment Options in Cardiovascular Medicine
https://www.readbyqxmd.com/read/28283766/autoantibodies-to-types-i-and-iv-collagen-and-heart-valve-disease-in-systemic-lupus-erythematosus-antiphospholipid-syndrome
#3
José Pardos-Gea, Josefina Cortés-Hernández, Jesus Castro-Marrero, Eva Balada, José Ordi-Ros
Introduction/objectives autoantibodies to types I and IV collagen have been described in rheumatic fever and infective endocarditis. We tried to elucidate if an autoimmune response against collagens I and IV exists, associated with heart valve disease in systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS). A cohort of 172 patients with SLE (n = 79), primary APS (PAPS, n = 83), and secondary APS (n = 10) were assessed for valvulopathy by transthoracic echocardiograms. Autoantibodies to types I and IV collagen were assessed in patients and 50 controls, setting autoantibody positivity at two standard deviations above the mean antibody level of controls...
March 10, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28281241/decellularized-cryopreserved-allografts-as-off-the-shelf-allogeneic-alternative-for-heart-valve-replacement-in-vitro-assessment-before-clinical-translation
#4
Laura Iop, Adolfo Paolin, Paola Aguiari, Diletta Trojan, Elisa Cogliati, Gino Gerosa
Cryopreserved allogeneic conduits are the elective biocompatible choice among currently available substitutes for surgical replacement in end-stage valvulopathy. However, degeneration occurs in 15 years in adults or faster in children, due to recipient's immunological reactions to donor's antigens. Here, human aortic valves were decellularized by TRICOL, based on Triton X-100 and sodium cholate, and submitted to standard cryopreservation (TRICOL-human aortic valves (hAVs)). Tissue samples were analyzed to study the effects of the combined procedure on original valve architecture and donor's cell removal...
March 9, 2017: Journal of Cardiovascular Translational Research
https://www.readbyqxmd.com/read/28229615/bacteremia-with-raoultella-planticola-in-the-setting-of-acute-pancreatitis-complicated-with-acute-cholangitis
#5
Esther Merino Rodríguez, Susana Rebolledo Olmedo, Joaquín Miquel Plaza
The bacterium Raoultella planticola (R planticola) is a rare pathogen in humans. We report a case of mild acute pancreatitis (MAP) of biliary origin with cholangitis and bacteremia with R planticola in association with pancreatic panniculitis (PP). A 55-year-old woman with rheumatic multiple valvulopathy presented with epigastric pain radiating to the back in a belt-like fashion for 48 hours, jaundice, choluria, acholia, and fever (38.2oC). Lab tests showed: amylase 2126 U/L, ALT 124 U/L, AST 169 U/L, GGT 3548 U/L, AP 1516 U/L, TBil 12...
February 23, 2017: Revista Española de Enfermedades Digestivas
https://www.readbyqxmd.com/read/28217516/long-term-cardiac-valvulopathy-safety-of-cabergoline-in-prolactinoma
#6
Shruti Khare, Anurag R Lila, Rishikesh Patil, Milind Phadke, Prafulla Kerkar, Tushar Bandgar, Nalini S Shah
BACKGROUND: Clinical relevance of association of cabergoline use for hyperprolactinemia and cardiac valvulopathy remains unclear. OBJECTIVE: The aim of the study was to determine the prevalence of valvular heart abnormalities in patients taking cabergoline for the treatment of prolactinoma and to explore any associations with the cumulative dose of drug used. DESIGN: A cross-sectional echocardiographic study was performed in patients who were receiving cabergoline therapy for prolactinoma...
January 2017: Indian Journal of Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28051025/retrospective-examination-of-q-fever-endocarditis-an-underdiagnosed-disease-in-the-mainland-of-china
#7
Xiao Han, Jeffrey Hsu, Qi Miao, Bao-Tong Zhou, Hong-Wei Fan, Xiao-Lu Xiong, Bo-Hai Wen, Lian Wu, Xiao-Wei Yan, Quan Fang, Wei Chen
BACKGROUND: Q fever endocarditis, a chronic illness caused by Coxiella burnetii, can be fatal if misdiagnosed or left untreated. Despite a relatively high positive rate of Q fever serology in healthy individuals in the mainland of China, very few cases of Q fever endocarditis have been reported. This study summarized cases of Q fever endocarditis among blood culture negative endocarditis (BCNE) patients and discussed factors attributing to the low diagnostic rate. METHODS: We identified confirmed cases of Q fever endocarditis among 637 consecutive patients with infective endocarditis (IE) in the Peking Union Medical College Hospital between 2006 and 2016...
2017: Chinese Medical Journal
https://www.readbyqxmd.com/read/27889859/chronic-high-dose-glucocorticoid-therapy-triggers-the-development-of-chronic-organ-damage-and-worsens-disease-outcome-in-systemic-lupus-erythematosus
#8
Tünde Tarr, Gábor Papp, Nikolett Nagy, Edina Cserép, Margit Zeher
Long-term survival of patients with systemic lupus erythematosus (SLE) improved worldwide; thus, prevention of cumulative organ damage became a major goal in disease management. The aim of our study was to investigate the chronic organ damages and their influence on disease outcome in SLE. We evaluated clinical conditions, laboratory findings and medications of 357 consecutive SLE patients and assessed their impact on Systemic Lupus Collaborating Clinics (SLICC)/American College of Rheumatology (ACR) Damage Index (SDI) and disease outcome...
February 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/27888609/effects-of-lorcaserin-on-pre-existing-valvulopathy-a-pooled-analysis-of-phase-3-trials
#9
Neil J Weissman, Steven R Smith, Randi Fain, Nancy Hall, William R Shanahan
OBJECTIVE: To evaluate the effects of lorcaserin in patients with pre-existing Food and Drug Administration (FDA)-defined valvulopathy. METHODS: This is a pooled, post hoc analysis of three Phase 3 studies. BLOOM and BLOSSOM patients were 18 to 65 years of age without diabetes and with a body mass index (BMI) of 27 to 29.9 kg/m(2) and ≥1 weight-related comorbidity or a BMI of 30 to 45 kg/m(2) . BLOOM-DM patients had a BMI of 27 to 45 kg/m(2) and type 2 diabetes...
January 2017: Obesity
https://www.readbyqxmd.com/read/27861189/oral-anticoagulation-for-stroke-prevention-amongst-atrial-fibrillation-patients-with-valvular-heart-disease-an-update
#10
Andrew C T Ha, Atul Verma, Subodh Verma
PURPOSE OF REVIEW: The majority of evidence on the safety and efficacy of oral anticoagulation for stroke prevention amongst patients with atrial fibrillation is derived from those without significant valvular heart disease. This article will review current knowledge, areas of uncertainty and controversy, and ongoing research on oral anticoagulation for stroke prevention amongst patients with valvular heart disease. RECENT FINDINGS: The rates of stroke, systemic embolism, and major bleeding were similar for patients with and without significant native valvular disease when treated with direct oral anticoagulants (DOACs) or vitamin K antagonists...
March 2017: Current Opinion in Cardiology
https://www.readbyqxmd.com/read/27856776/practice-guideline-summary-treatment-of-restless-legs-syndrome-in-adults-report-of-the-guideline-development-dissemination-and-implementation-subcommittee-of-the-american-academy-of-neurology
#11
John W Winkelman, Melissa J Armstrong, Richard P Allen, K Ray Chaudhuri, William Ondo, Claudia Trenkwalder, Phyllis C Zee, Gary S Gronseth, David Gloss, Theresa Zesiewicz
OBJECTIVE: To make evidence-based recommendations regarding restless legs syndrome (RLS) management in adults. METHODS: Articles were classified per the 2004 American Academy of Neurology evidence rating scheme. Recommendations were tied to evidence strength. RESULTS AND RECOMMENDATIONS: In moderate to severe primary RLS, clinicians should consider prescribing medication to reduce RLS symptoms. Strong evidence supports pramipexole, rotigotine, cabergoline, and gabapentin enacarbil use (Level A); moderate evidence supports ropinirole, pregabalin, and IV ferric carboxymaltose use (Level B)...
December 13, 2016: Neurology
https://www.readbyqxmd.com/read/27804074/mechanical-dyssynchrony-according-to-validated-cut-off-values-using-gated-spect-myocardial-perfusion-imaging
#12
Santiago Aguadé-Bruix, Guillermo Romero-Farina, Jaume Candell-Riera, María N Pizzi, David García-Dorado
BACKGROUND: The aim of this study was to establish different degrees of mechanical dyssynchrony according to validated cut-off (CO) values of myocardial perfusion gated SPECT phase analysis parameters (SD, standard deviation; B, bandwidth; S, skewness; K, kurtosis). METHODS: Using Emory Cardiac Toolbox™, we prospectively analyzed 408 patients (mean age 64.1 years, 26.7% female), divided into a control group of 150 normal subjects and a validation group of 258 patients (left bundle branch block: 17...
November 1, 2016: Journal of Nuclear Cardiology: Official Publication of the American Society of Nuclear Cardiology
https://www.readbyqxmd.com/read/27790834/low-dose-fenfluramine-significantly-reduces-seizure-frequency-in-dravet-syndrome-a-prospective-study-of-a-new-cohort-of-patients
#13
A Schoonjans, B P Paelinck, F Marchau, B Gunning, A Gammaitoni, B S Galer, L Lagae, B Ceulemans
BACKGROUND AND PURPOSE: Dravet syndrome (DS) is a severe, drug-resistant epilepsy. Fenfluramine has been reported to have a long-term clinically meaningful anticonvulsive effect in patients with DS. METHODS: This prospective, open-label study assessed the safety and effectiveness of low-dose fenfluramine in a new cohort of patients with DS. Following a 3-month baseline period, fenfluramine was added to each patient's current antiepileptic drug regimen at a dose of 0...
February 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/27709470/prospective-long-term-study-of-the-effect-of-cabergoline-on-valvular-status-in-patients-with-prolactinoma-and-idiopathic-hyperprolactinemia
#14
Laurent Vroonen, Patrizio Lancellotti, Monica Tomé Garcia, Raluca Dulgheru, Matilde Almanza, Ibrahima Maiga, Julien Magne, Patrick Petrossians, Renata Auriemma, Adrian F Daly, Albert Beckers
Since the 1990's cabergoline has been the treatment of choice in prolactinoma, as it permits rapid and effective hormonal and tumor control in most cases. Evidence of cardiac valvulopathy was demonstrated in Parkinson's disease patients treated with dopamine agonists. Retrospective studies in prolactinoma patients treated with cabergoline at lower doses did not show such an effect. However, few prospective data with long-term follow-up are available. The aim of this study was to assess the safety of cabergoline regarding cardiac valvular status during prospective follow-up in patients treated for prolactinoma or idiopathic hyperprolactinemia...
January 2017: Endocrine
https://www.readbyqxmd.com/read/27648285/hyperinsulinemia-and-sulfonylurea-use-are-independently-associated-with-left-ventricular-diastolic-dysfunction-in-patients-with-type-2-diabetes-mellitus-with-suboptimal-blood-glucose-control
#15
Tomoaki Inoue, Yasutaka Maeda, Noriyuki Sonoda, Shuji Sasaki, Teppei Kabemura, Kunihisa Kobayashi, Toyoshi Inoguchi
OBJECTIVE: Although diabetes mellitus is associated with an increased risk of heart failure with preserved ejection fraction, the underlying mechanisms leading to left ventricular diastolic dysfunction (LVDD) remain poorly understood. The study was designed to assess the risk factors for LVDD in patients with type 2 diabetes mellitus. RESEARCH DESIGN AND METHODS: The study cohort included 101 asymptomatic patients with type 2 diabetes mellitus without overt heart disease...
2016: BMJ Open Diabetes Research & Care
https://www.readbyqxmd.com/read/27627785/safety-and-tolerability-review-of-lorcaserin-in-clinical-trials
#16
REVIEW
F L Greenway, W Shanahan, R Fain, T Ma, D Rubino
Lorcaserin is a novel selective serotonin 2C receptor agonist indicated by the US Food and Drug Administration for chronic weight management in adults with obesity or overweight with ≥1 comorbidity. The safety and efficacy of lorcaserin were established during two Phase III clinical trials in patients without diabetes (BLOOM and BLOSSOM) and one Phase III clinical trial in patients with type 2 diabetes (BLOOM-DM). Headache was the most common adverse event experienced by patients during all Phase III trials...
October 2016: Clinical Obesity
https://www.readbyqxmd.com/read/27599727/flow-measurement-at-the-aortic-root-impact-of-location-of-through-plane-phase-contrast-velocity-mapping
#17
Litten Bertelsen, Jesper Hastrup Svendsen, Lars Køber, Ketil Haugan, Søren Højberg, Carsten Thomsen, Niels Vejlstrup
BACKGROUND: Cardiovascular magnetic resonance (CMR) is considered the gold standard of cardiac volumetric measurements. Flow in the aortic root is often measured at the sinotubular junction, even though placing the slice just above valve level may be more precise. It is unknown how much flow measurements vary at different levels in the aortic root and which level corresponds best to left ventricle volumetry. METHODS: All patients were older than 70 years presenting with at least one of the following diagnoses: diabetes, hypertension, prior stroke and/or heart failure...
September 7, 2016: Journal of Cardiovascular Magnetic Resonance
https://www.readbyqxmd.com/read/27571182/a-follow-up-study-of-the-prevalence-of-valvular-heart-abnormalities-in-hyperprolactinemic-patients-treated-with-cabergoline
#18
William M Drake, Craig E Stiles, John S Bevan, Niki Karavitaki, Peter J Trainer, D Aled Rees, Tristan I Richardson, Stephanie E Baldeweg, Nemanja Stojanovic, Robert D Murray, Andrew A Toogood, Niamh M Martin, Bijay Vaidya, Than S Han, Rick P Steeds, F C Baldeweg, U E Sheikh, N Kyriakakis, S K Parasuraman, L Taylor, N Butt, S Anyiam
CONTEXT: Uncertainty exists whether the long-term use of ergot-derived dopamine agonist (DA) drugs for the treatment of hyperprolactinemia may be associated with clinically significant valvular heart disease and whether current regulatory authority guidelines for echocardiographic screening are clinically appropriate. OBJECTIVE: Our objective was to provide follow-up echocardiographic data on a previously described cohort of patients treated with DA for lactotrope pituitary tumors and to explore possible associations between structural and functional valve abnormalities with the cumulative dose of drug used...
November 2016: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/27547548/computational-fluid-dynamics-modelling-of-left-valvular-heart-diseases-during-atrial-fibrillation
#19
Stefania Scarsoglio, Andrea Saglietto, Fiorenzo Gaita, Luca Ridolfi, Matteo Anselmino
BACKGROUND: Although atrial fibrillation (AF), a common arrhythmia, frequently presents in patients with underlying valvular disease, its hemodynamic contributions are not fully understood. The present work aimed to computationally study how physical conditions imposed by pathologic valvular anatomy act on AF hemodynamics. METHODS: We simulated AF with different severity grades of left-sided valvular diseases and compared the cardiovascular effects that they exert during AF, compared to lone AF...
2016: PeerJ
https://www.readbyqxmd.com/read/27534757/children-with-post-rheumatic-valvulopathies-in-natural-history-five-years-follow-up-in-the-cardiac-centre-st-elizabeth-catholic-general-hospital-shisong-cameroon
#20
J C Tantchou Tchoumi, J C Ambassa, G Butera
The aim of the study was to investigate the pattern of valvular lesions, the mortality and the challenges in five years follow-up in children with post-rheumatic valvulopathies in natural history in St. Elizabeth Catholic General hospital Shisong, cardiac centre. This retrospective analysis included 270 patients aged between 5 and 16 years old suffering from post-rheumatic valvulopathies who consulted in the cardiac centre from July 2008 through July 2013. Postrheumatic valvulopathies were diagnosed according to the World heart federation criteria...
December 2016: Bulletin de la Société de Pathologie Exotique
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