Rheumatology Advance Access originally published online on February 28, 2007
Rheumatology 2007 46(6):902-903; doi:10.1093/rheumatology/kem020
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© The Author 2007. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org
EDITORIALS |
Lessons from Marfan syndrome
Academic Unit of Musculoskeletal Disease, University of Leeds, UK
Correspondence to: Clinical Pharmacology Unit, Chapel Allerton Hospital, Chapeltown Road, Leeds LS7 4SA, UK. E-mail: howard.bird@leedsth.nhs.uk
| The first 10% of the full text of this article appears below. |
Amongst the inherited abnormalities of connective tissue, Marfan syndrome continues to command particular attention, not least because of the realization that these individuals are best managed, long-term, in a specialist tertiary referral centre at which facilities for the monitoring of cardiac function and the size of the aortic diameter exist. Surgical repair is available if the aortic root widens to 5 cm diameter, preferably before it becomes torn and the risk to patients is reduced if blood pressure is monitored meticulously.
Nevertheless, there is still ample scope for diagnostic confusion. Although standard diagnostic criteria are published [1, 2], there is still substantial evidence of overlap between the different