120 Chapter 4 Table 1: FD patients’ characteristics All Men Women Number of patients, n (%) 92 (100%) 34 (37%) 58 (63%) General characteristics FD patients Age at FD diagnosis (years), median (range) 28 (0.03-67) 20 (5-53) 36 (0.03-67) Clinical diagnosis, n (%) 25/87 (29%) 18/31 (58%) 7/56 (12%) Diagnosis through family screening, n (%) 62/87 (71%) 13/31 (42%) 49/56 (88%) Age at first echocardiogram (years), median (range) 39 (18-72) 27 (18-56) 42 (18-72) Age at last echocardiogram (years), median (range) 51 (24-82) 39 (25-62) 53 (24-82) Cumulative follow up (years), median (range) 12 (5-16) 10 (5-16) 12 (5-15) Enzyme replacement therapy (ERT) Number of patients on ERT, n (%) 85 (92%) 34 (100%) 51 (88%) Age at start ERT (years), median (range) 41 (13-71) 28 (13-54) 44 (16-71) ERT duration (years), median (range) 11 (1-21) 12 (1-21) 10 (1-16) Laboratory findings Available NT-proBNP results, n samples / n echocardiograms (%) 164/184 (89%) 58/68 (85%) 106/116 (91%) Available hs-TnT results, n samples / n echocardiograms (%) 111/184 (60%) 42/68 (62%) 69/116 (60%) Untreated baseline plasma LysoGb3 (nmol/l), median (range) 11 (3-149) 98 (31-149) 8 (3-24) eGFR- CKD EPI at first echocardiogram (ml/min), median (range) 107 (17-143) 112 (17-143) 105 (46-138) eGFR- CKD EPI at last echocardiogram (ml/min), median (range) 84 (15-148) 89 (15-148) 82 (22-130) Albuminuria/ 24 hours at first echocardiogram (mg/24 hours), median (range) 53 (0-4,236) 73 (4-4,236) 36 (0-2,598) Albuminuria/ 24 hours at last echocardiogram (mg/24 hours), median (range) 41 (3-3,498) 58 (3-3,498) 37 (5-1,331) Occurrence of Atrial fibrillation (AF) after the first echocardiogram Atrial fibrillation (AF), n (%) 14 (100%) 6 (43%) 8 (57%) Age at first AF episode (years), median (range) 55 (41-67) 49 (41-61) 63 (48-67)
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