Table 2.
Clinical signs and symptoms in Fabry cohorts combined from AGAL-009-00 and AGAL-008-00
| Clinical signs and symptoms | Number (%) of patients |
M vs. F, P-value | ||
|---|---|---|---|---|
| All, 139 (100) | Male, 92 (66.2) | Female, 47 (33.8) | ||
| Cardiovascular (any of the following)a | 84 (60.4%) | 61 (66.3%) | 23 (48.9%) | <0.048 |
| Dyspnoea | 23 (16.5%) | 15 (16.3%) | 8 (17.0%) | <0.914 |
| Angina | 15 (10.8%) | 8 (8.7%) | 1 (2.1%) | <0.137 |
| Chest pain | 9 (6.5%) | 12 (13.0%) | 3 (6.4%) | <0.231 |
| Oedema | 39 (28.1%) | 35 (38.0%) | 4 (8.5%) | <0.021 |
| Hypertension | 43 (30.9%) | 27 (29.3%) | 16 (34.0%) | <0.571 |
| Hypotension | 4 (2.9%) | 3 (3.3%) | 1 (2.1%) | <0.071 |
| Murmur | 28 (20.1%) | 22 (23.9%) | 6 (12.8%) | <0.121 |
| ECG abnormalities (any of the following)a | 88 (63.3%) | 72 (78.3%) | 16 (34.0%) | <0.001 |
| Bradycardia | 23 (16.5%) | 20 (21.7%) | 3 (6.4%) | <0.021 |
| Conduction abnormality | 26 (18.7%) | 21 (22.8%) | 5 (10.6%) | <0.001 |
| PR < 120 ms | 3 (2.1%) | 3 (3.3%) | 0 (0%) | <0.211 |
| PR > 200 ms | 5 (3.6%) | 3 (3.3%) | 2 (4.3%) | <0.766 |
| RBBB | 8 (5.7%) | 8 (8.7%) | 0 (0%) | <0.037 |
| LBBB | 1 (0.7%) | 1 (1.1%) | 0 (0%) | <0.473 |
| Other (IRBB, LAHB) | 9 (6.5%) | 9 (9.8%) | 0 (0%) | <0.027 |
| LVH (for ≥35 years old., n = 105, 67 M and 38 F) | 53 (50.5%) | 47 (70.1%) | 6 (15.8%) | <0.001 |
| RVH | 3 (2.2%) | 1 (2.1%) | 2 (2.2%) | <0.986 |
| Dermatologicalb | 116 (83.4%) | 83 (90.2%) | 33 (70.2%) | <0.003 |
| Neuralb | 91 (65.5%) | 58 (63.0%) | 33 (70.2%) | <0.400 |
| HEENTb | 87 (62.6%) | 57 (62.0%) | 30 (63.8%) | <0.829 |
| GIb | 79 (56.8%) | 60 (65.2%) | 19 (40.4%) | <0.005 |
| Renalb | 75 (53.9%) | 59 (64.1%) | 16 (34.0%) | <0.001 |
| Musculoskeletalb | 55 (39.5%) | 42 (45.7%) | 13 (27.7%) | <0.040 |
| Hypercholesterolaemia | 25 (18.0%) | 17 (18.5%) | 8 (17.1%) | <0.832 |
aHypertension (SBP ≥ 140 or DBP ≥ 90), hypotension (SBP < 90), conduction abnormality (any of the following: short or long PR interval, right bundle branch block, left bundle branch block, other intraventricular conduction delay), left ventricular hypertrophy (LVH), and right ventricular hypertrophy (RVH); LVH criteria (Sokolow–Lyon) were used to examine only subjects ≥35 years old.
bNon-cardiovascular review of systems is as follows: Dermatologic (angiokeratomas, hypohydrosis, anhydrosis), HEENT (corneal or lens abnormalities, headache, hypacousia, vertigo), GI (abdominal pain, diarrhoea), Renal (hematuria, proteinuria), Neural (acroparesthesia), Musculoskeletal (arthralgia, myalgia, pain).
Bold values indicate P ≤ 0.05.