Download Diagnosis and Management of Marfan Syndrome by Anne H. Child PDF

By Anne H. Child

This publication has been written in accordance with the various very good questions posed by way of our sufferers and their care groups, questions which deserve the best-informed and recent solutions supplied by means of our specialists in all the many overall healthiness components laid low with Marfan syndrome. the purpose of this article is to supply a precis of the current day realizing of prognosis, administration and top clinical and surgical procedure of babies, youngsters and adults with Marfan syndrome. The authors disguise the lifelong difficulties from start to outdated age, in every one affected process. 40 percentage of this data is the results of new cautious examine in line with a well-defined longitudinally studied united kingdom sufferer population.​

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Similarly, Pees demonstrated that monotherapy with losartan reduced aortic dilatation in a small, unselected group of children and adolescents with Marfan syndrome with the suggestion that efficacy was improved when therapy was started at a younger age and patients were treated for longer periods [27]. 5 years) randomised to atenolol or losartan, there was no difference in reduction of aortic root Z scores, aortic dissection rates, or the need for aortic root surgery over a 3 year period [28]. Thus, at present, the evidence suggests that children with Marfan syndrome may develop less aortic dilatation when treated with an angiotensin receptor blocker but the relative merits of betablockade and angiotensin converting enzyme blockade remain unclear.

1991;18(1):57–63. 17. Wilson DG, et al. Endothelial function in Marfan syndrome: selective impairment of flowmediated vasodilation. Circulation. 1999;99(7):909–15. 18. Keane MG, Pyeritz RE. Medical management of Marfan syndrome. Circulation. 2008;117(21):2802–13. 19. Tinkle BT, Saal HM, Committee on Genetics. Health supervision for children with Marfan syndrome. Pediatrics. 2013;132(4):e1059–72. 20. Das BB, Taylor AL, Yetman AT. Left ventricular diastolic dysfunction in children and young adults with Marfan syndrome.

This is due to defective connective tissue and degeneration because of increased TGF-β signaling [16] (Fig. 1). Mitral valve prolapse (MVP) is defined as closing position displacement of one or both leaflets cephalad (or superior) into the left atrium past the annular plane. The prevalence of isolated MVP is 2–3 % in the general population with the significant risk of severe MR, heart failure, and infective endocarditis. 25 % of mitral valve prolapse patients are reported to have MFS. During ventricular contraction, the mitral valve has to withstand its force, and prolapse of posterior leaflet is more common [17].

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