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Isr Med Assoc J. 2008 Mar;10(3):171-4.

The multidisciplinary approach to the Marfan patient.

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1
Department of Cardiac Surgery, Sheba Medical Center, Tel Hashomer, Israel. ehud.raanani@sheba.health.gov.il

Abstract

Progress in the past century has led to an improved understanding of the cause, pathophysiology and treatment of Marfan syndrome. The "Ghent criteria" constitute currently the most effective way of diagnosing or excluding Marfan syndrome. This system can also help to identify families with aortic aneurysms who do not have Marfan syndrome, but it should not be used to assess risk in such families. Despite the morbidity and mortality associated with Marfan syndrome, an appropriate multidisciplinary medical and surgical approach can improve and extend the lives of many patients. As knowledge of the consequences of fibrillin-1 deficiency develops, the treatment will continue to advance, providing improved length and quality of life for Marfan patients.

PMID:
18494225
[Indexed for MEDLINE]
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