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coronary collagen congenital

Michele Abate, Vincenzo Salini, Isabel Andia
Congenital metabolic disorders are consequence of defects involving single genes that code for enzymes. Blocking metabolic pathways, the defect leads to the shortage of essential compounds, and/or to the accumulation of huge quantities of precursors, which interfere with normal functions. Only few of these diseases are characterized by a clinically significant tendon involvement.Heterozygous Familial Hypercholesterolaemia results from the inheritance of a mutant low-density lipoprotein receptor gene; patients show high cholesterol levels, precocious coronary artery disease, and may develop tendon xanthomata (mainly in Achilles tendon)...
2016: Advances in Experimental Medicine and Biology
Maythem Saeed, Steven W Hetts, Robert Jablonowski, Mark W Wilson
Myocardial pathologies are major causes of morbidity and mortality worldwide. Early detection of loss of cellular integrity and expansion in extracellular volume (ECV) in myocardium is critical to initiate effective treatment. The three compartments in healthy myocardium are: intravascular (approximately 10% of tissue volume), interstitium (approximately 15%) and intracellular (approximately 75%). Myocardial cells, fibroblasts and vascular endothelial/smooth muscle cells represent intracellular compartment and the main proteins in the interstitium are types I/III collagens...
November 26, 2014: World Journal of Cardiology
Katrien Vandoorne, Moriel H Vandsburger, Karen Weisinger, Vlad Brumfeld, Brian A Hemmings, Alon Harmelin, Michal Neeman
Even though congenital heart disease is the most prevalent malformation, little is known about how mutations affect cardiovascular function during development. Akt1 is a crucial intracellular signaling molecule, affecting cell survival, proliferation, and metabolism. The aim of this study was to determine the role of Akt1 on prenatal cardiac development. In utero echocardiography was performed in fetal wild-type, heterozygous, and Akt1-deficient mice. The same fetal hearts were imaged using ex vivo micro-computed tomography (μCT) and histology...
November 2013: Physiological Reports
Jin Yang, Miriam Zeini, Chieh-Yu Lin, Chien-Jung Lin, Yiqin Xiong, Ching Shang, Pei Han, Wei Li, Thomas Quertermous, Bin Zhou, Ching-Pin Chang
AIMS: Congenital coronary artery anomalies produce serious events that include syncope, arrhythmias, myocardial infarction, or sudden death. Studying the mechanism of coronary development will contribute to the understanding of the disease and help design new diagnostic or therapeutic strategies. Here, we characterized a new calcineurin-NFAT signalling which specifically functions in the epicardium to regulate the development of smooth muscle wall of the coronary arteries. METHODS AND RESULTS: Using tissue-specific gene deletion, we found that calcineurin-NFAT signals in the embryonic epicardium to direct coronary smooth muscle cell development...
January 1, 2014: Cardiovascular Research
Frank G Scholl, Mark M Boucek, Kak-Chen Chan, Lilliam Valdes-Cruz, Richard Perryman
An ideal material for repair of congenitally malformed hearts would encourage tissue regeneration with growth potential. Decellularized porcine small intestinal submucosa extracellular matrix (SIS-ECM) promotes tissue regeneration in animal models and noncardiac human applications. This retrospective review evaluates SIS-ECM for reconstruction of congenital heart defects. From June 2007 to May 2009, SIS-ECM patches were used in 43 operations on 40 patients aged 2 days to 13 years. In 16 cases, the SIS-ECM was used for pericardial closure...
April 2010: World Journal for Pediatric & Congenital Heart Surgery
Hai-Tao Chen, Xing-Hai Yang
OBJECTIVE: To evaluate the histopathological characteristics and clinical implication of sarcolemma tissue in prepubertal concealed penis. METHODS: After measurement of the penile length, 10 prepubertal children with congenital concealed penis underwent modified Devine's operation (treatment group), and another 10 normal prepubertal children received circumcision (control group). The anatomic features of the penile sarcolemma tissue was observed intraoperatively, and its fibrosis was evaluated by Masson trichrome staining...
March 2013: Zhonghua Nan Ke Xue, National Journal of Andrology
Joseph K Perloff
BACKGROUND: The coronary circulation in cyanotic congenital heart disease (CCHD) includes the extramural coronary arteries, basal coronary blood flow, flow reserve, the coronary microcirculation, and coronary atherogenesis. METHODS: Coronary arteriograms were analyzed in 59 adults with CCHD. Dilated extramural coronaries were examined histologically in six patients. Basal coronary blood flow was determined with N-13 positron emission tomography in 14 patients and in 10 controls...
February 2012: Current Cardiology Reviews
Dharani K Ajithdoss, Angela M Arenas-Gamboa, John F Edwards
A fibrous band connecting the middle of the free edge (nodulus Arantii) of the non-coronary aortic valve cusp to the ascending aorta just above the level of the non-coronary sinus of Valsalva was observed in an asymptomatic, 11-year-old, male Border Collie. The fibrous band was unrelated to the cause of the death in this dog. Such fibrous bands are usually reported in humans with congenital bicuspid aortic valves. To our knowledge, this is the first report of a fibrous band in the aortic valve in a domestic animal...
June 2011: Journal of Veterinary Cardiology: the Official Journal of the European Society of Veterinary Cardiology
Marie Lockhart, Elaine Wirrig, Aimee Phelps, Andy Wessels
The extracellular matrix (ECM) of the developing heart contains numerous molecules that form a dynamic environment that plays an active and crucial role in the regulation of cellular events. ECM molecules found in the heart include hyaluronan, fibronectin, fibrillin, proteoglycans, and collagens. Tight regulation of the spatiotemporal expression, and the proteolytic processing of ECM components by proteases including members of the ADAMTS family, is essential for normal cardiac development. Perturbation of the expression of genes involved in matrix composition and remodeling can interfere with a myriad of events involved in the formation of the four-chambered heart and result in prenatal lethality or cardiac malformations as seen in humans with congenital heart disease...
June 2011: Birth Defects Research. Part A, Clinical and Molecular Teratology
Xuling Huang, Xiaolin Gao, Ramon Diaz-Trelles, Pilar Ruiz-Lozano, Zhong Wang
Dissecting the molecular mechanisms that guide the proper development of epicardial cell lineages is critical for understanding the etiology of both congenital and adult forms of human cardiovascular disease. In this study, we describe the function of BAF180, a polybromo protein in ATP-dependent SWI/SNF chromatin remodeling complexes, in coronary development. Ablation of BAF180 leads to impaired epithelial-to-mesenchymal-transition (EMT) and arrested maturation of epicardium around E11.5. Three-dimensional collagen gel assays revealed that the BAF180 mutant epicardial cells indeed possess significantly compromised migrating and EMT potentials...
July 15, 2008: Developmental Biology
Juan Asbun, Francisco J Villarreal
Diabetes has emerged as a major threat to worldwide health. The increasing incidence of diabetes in young individuals is particularly worrisome given that the disease is likely to evolve over a period of years. In 1972, the existence of a diabetic cardiomyopathy was proposed based on the experience with four adult diabetic patients who suffered from congestive heart failure in the absence of discernible coronary artery disease, valvular or congenital heart disease, hypertension, or alcoholism. The exact mechanisms underlying the disease are unknown; however, an important component of the pathological alterations observed in these hearts includes the accumulation of extracellular matrix (ECM) proteins, in particular collagens...
February 21, 2006: Journal of the American College of Cardiology
Joseph K Perloff
BACKGROUND: Dilated coronary arteries, coronary blood flow, and the paucity of coronary atherosclerosis have not been studied in cyanotic congenital heart disease. METHODS: Coronary arteriograms were interpreted in 59 cyanotic adults, and dilated coronaries were examined histologically in 6. Coronary blood flow was determined with N-13 ammonia positron emission tomography in 14 Eisenmenger syndrome patients and in 10 controls. Total non-fasting cholesterols were retrieved in 279 patients who were divided into: Group A--143 cyanotic unoperated, Group B--47 acyanotic after operation, Group C--41 acyanotic unoperated, Group D--48 acyanotic before and after operation...
December 2004: International Journal of Cardiology
No abstract text is available yet for this article.
July 1964: Progress in Cardiovascular Diseases
Sona Nair, Kanjaksha Ghosh, Bipin Kulkarni, Shrimati Shetty, Dipika Mohanty
Glanzmann's thrombasthenia is an autosomal recessive disorder, rare in a global context, but a relatively more common platelet function defect in communities where consanguineous marriages are more frequent. On clinical grounds alone, it cannot be distinguished from other congenital platelet function defects. Epistaxis, gum bleeding, menorrhagia are the common clinical manifestations, whereas large muscle hematoma or hemarthrosis seldom occur in these patients. Essential diagnostic features are a normal platelet count and morphology, a greatly prolonged bleeding time, absence of platelet aggregation in response to ADP, collagen, epinephrine, thrombin and to all aggregating agents which ultimately depend on fibrinogen binding to platelets for this effect, flow cytometry, studies of GPIIb-IIIa receptors on the platelet membrane surface using monoclonal antibodies...
November 2002: Platelets
Xue Zhao, Xiangru Lu, Qingping Feng
We recently demonstrated that mice deficient in endothelial nitric oxide (NO) synthase (eNOS) have congenital septal defects and postnatal heart failure. However, the mechanisms by which eNOS affects heart development are not clear. We hypothesized that deficiency in eNOS impairs myocardial angiogenesis. Myocardial capillary densities were measured morphometrically in neonatal mouse hearts. In vitro tube formation on Matrigel was investigated in cardiac endothelial cells. In vivo myocardial angiogenesis was performed by implanting Matrigel in the left ventricular myocardium...
December 2002: American Journal of Physiology. Heart and Circulatory Physiology
Sotiris A Korossis, Catherine Booth, Helen E Wilcox, Kevin G Watterson, John N Kearney, John Fisher, Eileen Ingham
BACKGROUND AND AIMS OF THE STUDY: For both young patients with congenital heart disease and young, growing adults there is a need for replacement heart valves that will develop with the patient. Tissue-engineered heart valves coupled with in-vitro recellularization have this potential. One approach is to use acellular tissue matrices, but the decellularization treatment must not affect the biomechanical integrity of the valvular matrix. This study investigated the effect of 0.03% (w/v) and 0...
July 2002: Journal of Heart Valve Disease
I J Benjamin
In this comprehensive review of matrix remodeling, one central theme that bears re-emphasis is the extensivecross-talk and dynamic interactions that exist between terminally differentiated, postmitotic cells, proliferative cells, and the ECM of the cardiovascular system. The activities of MMPs and TIMPs constitute a well-orchestrated contest to maintain tissue integrity and homeostasis. Overexpression of MMPs tilts the balance in favor of irreversible tissue destruction of joints (eg, as in rheumatic disease), and efforts to curtail such errant pathways are ongoing (123)...
September 2001: Journal of Investigative Medicine: the Official Publication of the American Federation for Clinical Research
A Da Costa, K Isaaz, E Faure, S Mourot, A Cerisier, M Lamaud
OBJECTIVES: The purpose of this study was to evaluate the clinical outcome of a large cohort of patients who suffered an acute myocardial infarction with absolutely normal epicardial coronary arteries at the post-myocardial infarction coronary angiogram. The aetiological and prognostic factors in this population were also analysed. BACKGROUND: Few data exist concerning the outcome, and aetiological and prognostic factors, of patients with myocardial infarction and angiographically absolutely normal coronary arteries...
August 2001: European Heart Journal
P Vaideeswar, V Shankar, J R Deshpande, A Sivaraman, N Jain
Supravalvar aortic stenosis is a rare congenital heart anomaly, producing left ventricular outflow tract obstruction. Of the two anatomic variants that have been described, diffuse type is the rarest. We report five such cases in children between two months and nine years of age. None had features of Williams syndrome. The entire aorta was involved in three cases, with abdominal aortic coarctation in two cases. Stenosis was mainly due to involvement of the media, which showed smooth muscle hypertrophy, abnormal elastic fibers, and mild collagenization...
January 2001: Cardiovascular Pathology: the Official Journal of the Society for Cardiovascular Pathology
R P Bolande, E A Leistikow, F S Wartmann, T M Louis
Newborn piglets were subjected to 45 min of sustained norepinephrine-induced hypertension and then monitored for 4 hr at baseline conditions. They were then sacrificed and the anterior descending coronary artery was serially sectioned for study by light and electron microscopy. Other groups were sacrificed after 72 and 168 hr of baseline conditions. Changes were limited to the endothelium and subendothelial intima of the most proximal segment of the anterior descending coronary artery. As similar changes are normally present in perinatal piglets, the experimental animals were compared with sham-operated controls to determine if there was a modification of the naturally occurring congenital lesions...
October 1995: Experimental and Molecular Pathology
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