Estratto
La sindrome di Marfan è una malattia genetica caratterizzata da un’anomalia dell’elastina. È conosciuta come una malattia autosomica dominante, a penetranza variabile. L’anomalia patologica è data da una frammentazione del tessuto elastico della tonaca media dell’aorta. La combinazione di un’elevata quantità di tessuto elastico nell’aorta ascendente con la risposta allo stress all’eiezione ematica è probabilmente la causa della graduale, ma progressiva, dilatazione aortica.
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© 2007 Springer-Verlag Italia
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(2007). Sindrome di Marfan. In: Cardiopatie congenite dell’adulto. Springer, Milano. https://doi.org/10.1007/978-88-470-0526-6_19
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DOI: https://doi.org/10.1007/978-88-470-0526-6_19
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