Clinical features differ substantially between Caucasian and Asian populations of Marfan syndrome.
Circ J
; 77(11): 2793-8, 2013.
Article
em En
| MEDLINE
| ID: mdl-23986082
ABSTRACT
BACKGROUND:
Prevention of aortic dissection and sudden death in patients with Marfan syndrome (MFS) requires accurate diagnosis. MFS is diagnosed by the Ghent criteria, which are primarily based on clinical features of Caucasian MFS populations. We determined whether the Ghent criteria apply to Asian MFS populations. METHODS ANDRESULTS:
In this multicenter study, we included 255 adult MFS patients according to the Ghent criteria of 2010. Patients were excluded if they were neither Caucasian nor Asian. The Asian MFS population (n=49) had a smaller body surface area (BSA 1.8 m² vs. 2.0 m², P<0.001), a more severely affected aortic root (absolute aortic diameter 42.9 mm vs. 43.3mm, P=0.802; corrected for BSA 24.9 mm vs. 21.7 mm, P<0.001; Z-score 4.5 vs. 3.6, P=0.013), and more often a positive systemic score (75.5% vs. 60.0%, P=0.045), but less frequently ectopia lentis (24.5% vs. 48.1%, P=0.004) compared with the Caucasian population (n=206).CONCLUSIONS:
The Ghent criteria do not necessarily apply to Asian MFS populations, resulting in a more severely affected cardiovascular system. This may be related to under diagnosis of MFS by multiple factors, including the use of Z-score, and genetic and racial differences. The Ghent criteria should be adapted for Asian populations in order to accurately diagnose MFS.
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Bases de dados:
MEDLINE
Assunto principal:
Aorta
/
Povo Asiático
/
População Branca
/
Síndrome de Marfan
Tipo de estudo:
Clinical_trials
Limite:
Adult
/
Aged
/
Female
/
Humans
/
Male
/
Middle aged
País/Região como assunto:
Asia
/
Europa
Idioma:
En
Revista:
Circ J
Assunto da revista:
ANGIOLOGIA
/
CARDIOLOGIA
Ano de publicação:
2013
Tipo de documento:
Article