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10篇 您的检索式:作者名="Braunlin"
    题名 作者 年代 出处 被引量
1Enhancing efficacy of stem cell transplantation to the heart with a PEGylated fibrin biomatrix显示文摘Zhang Ge Hu Qing-song Braunlin EA 2008Tissue Eng Part A2008,14,6:1
2Interruption of the aortic arch with aorticopulmonary septal defect显示文摘Elizabeth Braunlin William M. Peoples Robert M. Freedom Donald C. Fyler Allan Goldblatt Jesse E. Edwards 1982Pediatric Cardiology1982,,4:1
3Cardiac disease in patients with nmcopolysaccharidosis: presentation, diagnosis and management 显示文摘Braunlin EA Harmatz PR Scarpa M 2011J Inherit Metab Dis2011,34,:1
4Usefulness of bone marrow transplantation in the Hurler syndrome 显示文摘Braunlin EA Stauffer NR Peters CH 2003Am J Cardiol2003,92,7:1
5Interruption of the aortic arch with aorucoulmon-ary septal defect; an anatomic review 显示文摘Braunlin E peoples WM Freedom RM 1982Pediatr Cardiol1982,3,:1
6Cardiac disease in patients with mucopolysacchari-dosis :presentation, diagnosis and management显示文摘BRAUNLIN E A HARMATZ P R SCARPA M etal 2011JInherit Metab Dis2011,34,:1
7显示文摘 Bailey M J Braunlin W H 2001Macrormoleclues2001,34,6:1
8Unexpected coronary artery findings in mucopolysaccharidosis. Report of four cases and literature review显示文摘Elizabeth Braunlin Paul J. Orchard Chester B. Whitley Luke Schroeder Robyn C. Reed J. Carlos Manivel 2014Cardiovascular Pathology2014,,:1
9Usefulness of bone marrow transplantation in the Hurler syndrome显示文摘Braunlin EA Stauffer NR Peters CH 2003Am J Cantiol2003,92,7:1
10黏多糖贮积症I型酶替代治疗后的心脏改变显示文摘Mucopolysaccharidosis type I is a lethal autosomal recessive storage disease caused by a deficiency of lysosomal α-l-iduronidase and the consequent systemic accumulation of glycosaminoglycan. Cardiomyopathy and valvar insufficiency occur as glycosaminoglycan accumulates in the myocardium, expands the spongiosa of cardiac valves, and proliferates within the myointima of the epicardial coronary arteries. Congestive heart failure and death occur within the first decade of life in the most severe cases. Allogeneic hematopoietic stem cell transplantation, used in severe forms of the disease, markedly prolongs survival, alleviates ventricular hypertrophy, and preserves cardiac function, but cardiac valves continue to thicken and valvular insufficiency progresses. Enzyme replacement therapy with human recombinant α-l-iduronidase has been proposed as an alternative therapy for patients with mucopolysaccharidosis type I in whom the risk/benefit ratio of hematopoietic stem cell transplantation seems unfavorable. The investigators report the cardiac findings in a small series of 5 children with mucopolysaccharidosis type I who received enzyme replacement therapy for as long as 7 years. No deaths occurred during treatment. Left ventricular hypertrophy,which was present before therapy, resolved in all cases, and myocardial function remained normal. In contrast, the mitral and aortic valves remained thickened and, in some instances, developed progressive thickening and regurgitation. In conclusion, long-term enzyme replacement therapy has some clear benefits for the myocardium, but the cardiac valves appear unresponsive, and the ultimate effect on the coronary vasculature is unknown.Braunlin E.A. Berry J.M. Whitley C.B. 雷聪 2006世界核心医学期刊文摘(心脏病学分册)2006,,12:0
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