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Advanced fibrosis at diagnosis has been independently associated with a smoking history of more than 10 pack, with an odds ratio (OR) of 1.63 (95% CI 1.192.24) [62]

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Since then, some studies showed no significant increase in the incidence of MVP in EDS compared to the general population [18,19,20], whereas others disclosed a higher MVP frequency (2867%) [120, 121, 122]