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https://www.readbyqxmd.com/read/29665967/structural-and-functional-changes-in-the-heart-of-adult-patients-with-osteogenesis-imperfecta-case-control-study
#1
Verónica Hernández Jiménez, Jesús Saavedra Falero, María Teresa Alberca Vela, Rebeca Mata Caballero, José Antonio Rosado Sierra, Isabel Pavón de Paz
INTRODUCTION AND OBJECTIVES: Osteogenesis imperfecta (OI) is a connective tissue disease characterised by an anomalous synthesis of type i collagen. Little is known about the cardiovascular affectation suffered by these patients. The most frequently described are valvular dysfunction and dilatation of the aorta. We wanted to analyse the structural and functional changes of the heart in adult patients with OI. METHODS: We prospectively studied 82 patients with OI and compared them with 60 healthy subjects matched for age and sex...
April 14, 2018: Medicina Clínica
https://www.readbyqxmd.com/read/29619932/mortality-and-morbidity-in-patients-with-osteogenesis-imperfecta-in-denmark
#2
Lars Folkestad
Ostegenesis imperfecta (OI) is a hereditary disease of the connective tissue caused by mutations to, mainly, the genes that are involved in the biosynthesis of collagen type 1. Patients are grouped according to clinical severity and mode of inheritance according to Sillence's classification (originally 1979, updated 2014). According to our data, the population prevalence of OI in Denmark was 10.3 per 100,000, with 575 patients registered with an OI diagnosis in the National Patient Register and alive at the end of 2012 out of a total population of 5,602,628 persons...
April 2018: Danish Medical Journal
https://www.readbyqxmd.com/read/29593562/toll-like-receptors-inflammation-and-calcific-aortic-valve-disease
#3
REVIEW
Carmen García-Rodríguez, Iván Parra-Izquierdo, Irene Castaños-Mollor, Javier López, J Alberto San Román, Mariano Sánchez Crespo
Inflammation, the primary response of innate immunity, is essential to initiate the calcification process underlying calcific aortic valve disease (CAVD), the most prevalent valvulopathy in Western countries. The pathogenesis of CAVD is multifactorial and includes inflammation, hemodynamic factors, fibrosis, and active calcification. In the development of CAVD, both innate and adaptive immune responses are activated, and accumulating evidences show the central role of inflammation in the initiation and propagation phases of the disease, being the function of Toll-like receptors (TLR) particularly relevant...
2018: Frontiers in Physiology
https://www.readbyqxmd.com/read/29569266/a-late-presentation-of-congenital-cardiac-anomaly-accessory-chordae-from-the-left-atrium-causing-severe-mitral-regurgitation
#4
Giovanni D'Onghia, Marta Martin, Maria Teresa Mancini, Silvia Quintarelli, Adele Cozza, Fabrizio Guarracini, Roberto Bonmassari
Mitral regurgitation secondary to accessory mitral valve (MV) chordae of the left atrium is an extremely rare congenital disease. A 85-year-old female (NYHA I-II) was hospitalized for investigations. An echocardiogram showed calcification of the MV with mild stenosis and moderate regurgitation. Transesophageal three-dimensional echocardiogram revealed a band-like structure extending from the distal third of the anterior wall of the left atrium to the MV. This accessory chordae determined severe systolic regurgitation and mild mitral stenosis...
March 22, 2018: Echocardiography
https://www.readbyqxmd.com/read/29523490/thrombotic-microangiopathy-in-a-very-young-infant-with-mitral-valvuloplasty
#5
Yuka Matsunaga, Masataka Ishimura, Hazumu Nagata, Kiyoshi Uike, Tadamune Kinjo, Masayuki Ochiai, Kenichiro Yamamura, Hidetoshi Takada, Yoshihisa Tanoue, Masaki Hayakawa, Masanori Matsumoto, Toshiro Hara, Shouichi Ohga
BACKGROUND: Thrombotic microangiopathies (TMA) are microvascular occlusive disorders characterized by systemic or intrarenal platelet aggregation, thrombocytopenia, and red cell fragmentation. Post-operative TMA mostly occurs in adult patients with cardiovascular surgery, with the distinct pathophysiology from classical thrombotic thrombocytopenic purpura (TTP) although the exact pathophysiology remains unclear. CASE PRESENTATION: A one-month-old infant developed TMA after the initial surgery of double outlet right ventricle...
February 7, 2018: Pediatrics and Neonatology
https://www.readbyqxmd.com/read/29471496/should-acute-q-fever-patients-be-screened-for-valvulopathy-to-prevent-endocarditis
#6
Marit M A de Lange, Laura E V Gijsen, Cornelia C H Wielders, Wim van der Hoek, Arko Scheepmaker, Peter M Schneeberger
Background: Echocardiographic screening of acute Q-fever patients and antibiotic prophylaxis for patients with cardiac valvulopathy are considered an important approach to prevent chronic Q-fever-related endocarditis. During a large Q-fever epidemic in the Netherlands, routine screening echocardiography was discontinued, raising controversy in the international literature. We followed a cohort of acute Q-fever patients to estimate the risk for developing chronic Q-fever, and we evaluated the impact of screening in patients who were not yet known to have a valvulopathy...
February 20, 2018: Clinical Infectious Diseases: An Official Publication of the Infectious Diseases Society of America
https://www.readbyqxmd.com/read/29397446/marseille-scoring-system-for-empiric-treatment-of-infective-endocarditis
#7
Frédérique Gouriet, Hervé Tissot-Dupont, Jean-Paul Casalta, Sandrine Hubert, Pierre-Edouard Fournier, Sophie Edouard, Alexis Theron, Hubert Lepidi, Dominique Grisoli, Gilbert Habib, Didier Raoult
Despite advances in medical, surgical, and critical care, infective endocarditis (IE) remains associated with considerable morbidity and mortality. We evaluated the performance of the Marseille score, including clinical data and biological tests obtained within 2 h, to identify patients at high risk of IE in order to initiate early antimicrobial treatment. This was secondarily confirmed using modified ESC criteria combined with molecular testing and (18) fluorodeoxyglucose-positron emission tomography/computed tomography as diagnostic tools...
May 2018: European Journal of Clinical Microbiology & Infectious Diseases
https://www.readbyqxmd.com/read/29395258/extra-criteria-manifestations-of-antiphospholipid-syndrome-risk-assessment-and-management
#8
Massimo Radin, Michelle Remião Ugolini-Lopes, Savino Sciascia, Danieli Andrade
OBJECTIVES: Extra-criteria manifestations of antiphospholipid syndrome (APS) might impact on prognosis and morbidity of the disease. In this study, we aimed to evaluate a population of patients with primary APS (PAPS) whether the extra-criteria manifestations were more frequently found in subjects with higher adjusted Global APS Score (aGAPSS) values when compared to patients with thrombotic and/or obstetric APS ("criteria" manifestations) only. METHODS: Clinical records were analyzed to retrieve extra-criteria manifestation of APS, cardiovascular risk factors and antiphospholipid antibodies profile...
January 5, 2018: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/29374829/in-vitro-safety-pharmacology-profiling-of-topical-%C3%AE-adrenergic-agonist-treatments-for-erythema-of-rosacea
#9
David Piwnica, Atul Pathak, Gregor Schäfer, James R Docherty
BACKGROUND: Topical α-adrenergic agonist therapy has been developed to treat the persistent erythema of rosacea patients. Brimonidine and oxymetazoline are both topical α-adrenergic agonists. OBJECTIVES: The objective of this in vitro safety pharmacology study was to compare the potential safety profiles of brimonidine and oxymetazoline. METHODS: Brimonidine and oxymetazoline underwent pharmacological profiling with a standard panel of 151 assays, including α-adrenergic receptors and 5-hydroxytryptamine (5-HT) receptors...
March 2018: Drugs in R&D
https://www.readbyqxmd.com/read/29335891/serum-bilirubin-concentration-is-associated-with-left-ventricular-remodeling-in-patients-with-type-2-diabetes-mellitus-a-cohort-study
#10
Tomoaki Inoue, Noriyuki Sonoda, Shinsuke Hiramatsu, Shinichiro Kimura, Yoshihiro Ogawa, Toyoshi Inoguchi
INTRODUCTION: Previous studies have shown that serum bilirubin concentration is inversely associated with the risk of cardiovascular disease. The relationship between serum bilirubin concentration and left ventricular geometry, however, has not been investigated in patients with diabetes mellitus. METHODS: In this cohort study, 158 asymptomatic patients with type 2 diabetes mellitus without overt heart disease were enrolled. Left ventricular structure and function were assessed using echocardiography...
February 2018: Diabetes Therapy: Research, Treatment and Education of Diabetes and related Disorders
https://www.readbyqxmd.com/read/29295699/aortic-stenosis-aortic-regurgitation-and-arterial-hypertension
#11
V Katsi, G Georgiopoulos, D Oikonomou, C Aggeli, C Grassos, D P Papadopoulos, C Thomopoulos, M Marketou, K Dimitriadis, K Toutouzas, P Nihoyannopoulos, C Tsioufis, D Tousoulis
BACKGROUND: Hypertension (HT) is an important risk factor for cardiovascular disease and might precipitate pathology of the aortic valve. OBJECTIVE: To investigate the association of HT with aortic dysfunction (including both aortic regurgitation and stenosis) and the impact of antihypertensive treatment on the natural course of underlying aortic disease. METHODS: We performed a systematic review of the literature for all relevant articles assessing the correlation between HT and phenotype of aortic disease...
January 1, 2018: Current Vascular Pharmacology
https://www.readbyqxmd.com/read/29291394/serotonin-receptor-2b-signaling-with-interstitial-cell-activation-and-leaflet-remodeling-in-degenerative-mitral-regurgitation
#12
Kathryn H Driesbaugh, Emanuela Branchetti, Juan B Grau, Samuel J Keeney, Kimberly Glass, Mark A Oyama, Nancy Rioux, Salma Ayoub, Michael S Sacks, John Quackenbush, Robert J Levy, Giovanni Ferrari
AIMS: Mitral valve interstitial cells (MVIC) play an important role in the pathogenesis of degenerative mitral regurgitation (MR) due to mitral valve prolapse (MVP). Numerous clinical studies have observed serotonin (5HT) dysregulation in cardiac valvulopathies; however, the impact of 5HT-mediated signaling on MVIC activation and leaflet remodeling in MVP have been investigated to a limited extent. Here we test the hypothesis that 5HT receptors (5HTRs) signaling contributes to MVP pathophysiology...
February 2018: Journal of Molecular and Cellular Cardiology
https://www.readbyqxmd.com/read/29258677/sixteen-year-experience-of-david-and-bentall-procedures-in-acute-type-a-aortic-dissection
#13
Bo Yang, Himanshu J Patel, Claire Sorek, Whitney E Hornsby, Xiaoting Wu, Sarah Ward, Marc Thomas, Anisa Driscoll, Victoria A Waidley, Elizabeth L Norton, Donald S Likosky, G Michael Deeb
BACKGROUND: To examine short-term and midterm outcomes after the David and Bentall procedures in patients with an acute type A aortic dissection. METHODS: Between 2001 and 2017, patients (n = 135) with acute type A aortic dissection underwent an aortic root replacement with either the David (n = 40) or Bentall (n = 95) procedure. Perioperative outcome, reoperation rate, aortic valve function, and long-term survival were evaluated. RESULTS: The median age of the entire cohort was 56 years...
March 2018: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/29201548/risk-factors-of-carotid-plaque-and-carotid-common-artery-intima-media-thickening-in-a-high-stroke-risk-population
#14
ChunFang Wang, GaoPeng Lv, DaWei Zang
Introduction: To analyze the risk factors of carotid plaque (CP) and carotid common artery intima-media thickening (CCAIMT) and the association between the risk factors and CP numbers and the side of the CCAIMT in a high-stroke-risk population. Methods: Carotid ultrasonography was conducted in 2025 participants with high stroke risk. Participants were divided into different groups according to the results of the ultrasound. The risk factors and blood biochemical indices were recorded...
November 2017: Brain and Behavior
https://www.readbyqxmd.com/read/29169750/bicuspid-aortic-valve-a-complex-aortopathy-rather-than-a-simple-valvulopathy
#15
EDITORIAL
Guido Gelpi, Claudia Romagnoni
No abstract text is available yet for this article.
January 1, 2018: International Journal of Cardiology
https://www.readbyqxmd.com/read/29140515/surveillance-for-q-fever-endocarditis-in-the-united-states-1999-2015
#16
Anne Straily, F Scott Dahlgren, Amy Peterson, Christopher D Paddock
Background: Q fever is a worldwide zoonosis caused by Coxiella burnetii. In some persons, particularly those with cardiac valve disease, infection with C. burnetii can cause a life-threatening infective endocarditis. There are few descriptive analyses of Q fever endocarditis in the United States. Methods: Q fever case report forms submitted during 1999-2015 were reviewed to identify reports describing endocarditis. Cases were categorized as confirmed or probable using criteria defined by the Council for State and Territorial Epidemiologists (CSTE)...
November 13, 2017: Clinical Infectious Diseases: An Official Publication of the Infectious Diseases Society of America
https://www.readbyqxmd.com/read/29135403/a-case-of-relapsing-polychondritis-mimicking-lung-cancer
#17
Fatma Canbay, Aydın Yılmaz, Ayperi Öztürk, Zafer Aktaş, Ebru Tatcı
Relapsing polychondritis (RP) is a rare autoimmune disease, characterized by episodic inflammatory attacks on cartilaginous tissue. Elastic cartilage of the ears and nose, hyaline cartilage of the peripheral joints, vertebral fibrocartilage, tracheobronchial cartilage, and tissues rich in proteoglycan such as those in the eyes, heart, blood vessels, and inner ear are more likely to be affected. A 35-year-old male presented with complaints of hoarseness, tinnitus and dyspnea for 19 years, with a history of several diagnostic and therapeutic interventions for laryngeal and respiratory tract...
September 2017: Tüberküloz Ve Toraks
https://www.readbyqxmd.com/read/29122631/mitral-valve-prolapse-multimodality-imaging-and-genetic-insights
#18
REVIEW
Purvi Parwani, Jean-Francois Avierinos, Robert A Levine, Francesca N Delling
Mitral valve prolapse (MVP) is a common heritable valvulopathy affecting approximately 2.4% of the population. It is the most important cause of primary mitral regurgitation (MR) requiring surgery. MVP is characterized by fibromyxomatous changes and displacement of one or both mitral leaflets into the left atrium. Echocardiography represents the primary diagnostic modality for assessment of MVP. Accurate quantitation of ventricular volumes and function for surgical planning in asymptomatic severe MR can be provided with both echocardiography and cardiac magnetic resonance...
November 2017: Progress in Cardiovascular Diseases
https://www.readbyqxmd.com/read/29092907/atrial-fibrillation-burden-in-young-patients-with-congenital-heart-disease
#19
Zacharias Mandalenakis, Annika Rosengren, Georg Lappas, Peter Eriksson, Thomas Gilljam, Per-Olof Hansson, Kristofer Skoglund, Maria Fedchenko, Mikael Dellborg
BACKGROUND: Patients with congenital heart disease (CHD) are assumed to be vulnerable to atrial fibrillation (AF) as a result of residual shunts, anomalous vessel anatomy, progressive valvulopathy, hypertension, and atrial scars from previous heart surgery. However, the risk of developing AF and the complications associated with AF in children and young adults with CHD have not been compared with those in control subjects. METHODS: Data from the Swedish Patient and Cause of Death registers were used to identify all patients with a diagnosis of CHD who were born from 1970 to 1993...
February 27, 2018: Circulation
https://www.readbyqxmd.com/read/29066082/performance-of-a-simplified-dichotomous-phenotypic-classification-of-bicuspid-aortic-valve-to-predict-type-of-valvulopathy-and-combined-aortopathy
#20
Byung Joo Sun, Sahmin Lee, Jeong Yoon Jang, Osung Kwon, Jae Seok Bae, Ji Hye Lee, Dae-Hee Kim, Sung-Ho Jung, Jong-Min Song, Duk-Hyun Kang, Cheol Hyun Chung, Jae-Kwan Song
BACKGROUND: A simplified classification of bicuspid aortic valve (BAV) morphology using only the orientation of fused cusps was recently proposed. The aim of this study was to test whether it is useful for showing an association with the type of valvulopathy or aortopathy. METHODS: BAV phenotype was retrospectively classified in 681 patients (mean age, 59 ± 12 years; 424 men) who underwent aortic valve surgery. Each BAV was classified using both dichotomous (right and left coronary cusp fusion [CCF] vs mixed cusp fusion [MCF]) and conventional methods, and its association with the dominant valvulopathy (aortic stenosis [AS] vs regurgitation) and concomitant aortic surgery was analyzed...
December 2017: Journal of the American Society of Echocardiography
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