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58, p0.001) on linear regression analysis. Age and ochronosis scores were significantly related to Vmax on multiple regression analysis (p0.005). Nitisinone decreased sHGA, 24-h urine HGA (uHGA , ochronosis scores and AKUSSI significantly at all visits post-nitisinone. Nitisinone decreased Vmax change scores at final visit comparison, with a similar pattern at earlier visits. Aortic valve disease is highly prevalent in this NAC cohort, and strongly associated with ochronosis and disease severity. Nitisinone decreases ochronosis a
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