37 Cardiac outcomes in Fabry disease Table 3: Firth’s penalized Cox regression Outcome Covariate Coef Exp (coef) 95% CI MACE Phenotype, classical 2.99 19.89** 2.61-2554.03 Sex, male 2.79 16.35** 1.94-2138.95 Phenotype:Sex -1.19 0.31 0.002-2.96 CVD Phenotype, classical 2.29 9.84* 1.13-1292.61 Sex, male 1.88 6.56 0.51-916.78 Phenotype:Sex 0.81 2.25 0.01-38.56 HF hospitalization Phenotype, classical 2.36 10.56* 1.23-1383.10 Sex, male 2.35 10.51* 1.08-1407.18 Phenotype:Sex 0.38 1.47 0.01-22.14 SVA Phenotype, classical 1.07 2.91 0.23-404.25 Sex, male 1.81 6.14 0.58-831.73 Phenotype:Sex 0.55 1.74 0.01-39.94 MI Phenotype, classical 2.42 11.19* 1.41-1446.63 Sex, male 2.21 9.12 0.94-1221.07 Phenotype:Sex -1.28 0.28 0.00-3.26 *P<0.05, **P<0.008 Table 4: Hazard ratio’s for the comparison of different patient groups. These HR where obtained from Firth’s penalized Cox regression models in which sex and phenotype where combined in 1 variable with 4 patient groups Comparison MACE CVD HF SVA MI Men classical FD vs Men nonclassical FD 6.1 (2.4-17.0)** 22.2 (5.0-19.3)** 15.5 (3.7-82.7)** 5.1 (0.9-41.1) 3.1 (1.0-11.7) Men classical FD vs Women classical FD 5.0 (2.3-11.0)** 14.8 (4.3-67.0)** 15.4 (4.1-73.8)** 10.7 (1.9-92.0)** 2.5 (1.0-6.7) Men non-classical FD vs Women classical FD 0.8 (0.3-2.0) 0.7 (0.1-2.7) 1 (0.3-3.4) 2.1 (0.4-13.0) 0.8 (0.2-2.4) Cardiovascular death In men with classical FD, 10 out of 15 deaths (67%) were cardiovascular deaths (event rate 6.5 per 1000 patient years). CVD was also the major cause of death in women with classical FD (6/6 deaths (100%), event rate 1.9) and men with nonclassical FD (2/3 deaths (67%), event rate 1.7). KM analysis showed a significant difference between the four subgroups (figure 4A, see supplemental figure 3 for CR analysis). Having a classical phenotype increased the risk of 2
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