• S V Moiseev, E A Karovaikina, N M Bulanov, A S Moiseev, and V V Fomin.
• Sechenov First Moscow State Medical University (Sechenov University).
• Terapevt Arkh. 2019 Jun 15; 91 (6): 404640-46.

AbstractFabry disease is a rare disorder characterized by high frequency of severe organ manifestations in young patients. Aim. To determine predictors of clinical events in patients with Fabry disease. Materials and methods. We recruited 100 adult (over 18 years) patients with Fabry disease that was confirmed by enzymatic and genetic studies. The main outcome was a composite of end - stage renal disease, cardiac (arrhythmia and cardioverter/pacemaker implantation) and cerebrovascular (transient ischemic attack, stroke) events. Kaplan-Meier analysis was performed for event - free survival. Cox regression model was used to examine the risk of composite endpoint. Results and disscussion. Forty - seven of the 100 patients (38 males and 9 females) experienced clinical events. The median age of the first event was 39 [32; 49] years. In Kaplan-Meier analysis, the age of the first event was significantly lower among men than women, (p.

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