Table 1.
Baseline factors and cognitive scores for participants with Fabry disease and controls
| n | Fabry disease (n = 54) | n | REGARDS controls (n = 216) | p-value | |
|---|---|---|---|---|---|
| Age M (SD) | 54 | 55.7 (6.7) | 216 | 56.0 (6.6) | 0.074 |
| White race n (%) | 54 | 53 (98) | 216 | 212 (98) | 0.99 |
| Men n (%) | 54 | 17 (31) | 216 | 68 (31) | 0.99 |
| Education n (%) | 54 | 216 | 0.99 | ||
| <HS (%) | 2 (4) | 8 (4) | |||
| HS grad (%) | 8 (15) | 32 (15) | |||
| Some college (%) | 16 (30) | 64 (30) | |||
| College grad (%) | 28 (52) | 112 (52) | |||
| Quality of life M (SD) | 53 | 214 | |||
| SF-12 MCS scorea,b | 47.7 (11.1) | 53.1 (9.0) | 0.002 | ||
| SF-12 PCS scorea,b | 38.0 (11.4) | 48.0 (9.9) | 0.001 | ||
| Perceived stress, PSS-4 scorea,b M (SD) | 53 | 6.4 (2.3) | 214 | 3.2 (2.7) | <0.0001 |
| Elevated depressive symptoms CES-D-4b n (%) | 54 | 15 (28) | 214 | 21 (10) | 0.007 |
| Income n (%) | 54 | 216 | 0.33 | ||
| <$20 K (%) | 3 (6) | 26 (12) | |||
| $20 K–$34 K (%) | 10 (19) | 54 (25) | |||
| $35 K–$74 K (%) | 20 (37) | 67 (31) | |||
| >=$75 K (%) | 15 (28) | 54 (25) | |||
| Refused (%) | 6 (11) | 15 (7) | |||
| Systolic blood pressurec M (SD) (mmHg) | 17 | 120 (17) | 216 | 120 (15) | 0.96 |
| Diastolic blood pressurec M (SD) (mmHg) | 16 | 74 (8.9) | 216 | 76 (11.0) | 0.96 |
| Hypertension n (%) | 54 | 38 (70) | 216 | 81 (38) | <0.0001 |
| Diabetesd n (%) | 54 | 5 (9) | 213 | 24 (11) | 0.56 |
| Heart disease d n (%) | 54 | 31 (57) | 206 | 36 (17) | <0.0001 |
| Atrial fibrillationc,d n (%) | 38 | 17 (45) | 206 | 10 (5) | <0.0001 |
| LVHc,d n (%) | 26 | 19 (73) | 81 | 2 (2) | <0.0001 |
| Dyslipidemia d n (%) | 54 | 38 (70) | 211 | 102 (47) | 0.0044 |
| Total cholesterolc,d M (SD) (mg/dL) | 26 | 180 (42) | 210 | 201 (36) | 0.021 |
| LDLc,d M (SD) (mg/dL) | 26 | 96 (30) | 210 | 119 (31) | 0.0006 |
| HDLc,d M (SD) (mg/dL) | 26 | 63 (22) | 210 | 53 (17) | 0.013 |
| Triglyceridesc,d M (SD) (mg/dL) | 26 | 102 (56) | 211 | 152 (69) | 0.0019 |
| Kidney function, eGFR ml/min/1.73 m2c,d M (SD) | 27 | 63 (26) | 211 | 85 (20) | 0.0001 |
| History of stroke e n (%) | 54 | 6 (11) | 216 | 10 (5) | 0.071 |
REGARDS stands for the Reasons for Geographic and Racial Differences in Stroke study
Four controls were randomly selected from all possible REGARDS participants who could be matched to each Fabry disease study participant on age (±2.5 years), race, sex, and education
Variables in bold print were used in subsequent generalized estimating equation models
Missing data:
a1 FD ppt refused to answer these questions
b2 REGARDS ppts refused to answer these questions
cMissing data for FD ppts on these variables was due to lack of available information in the Fabry Registry or physicians’ medical records
dMissing data for REGARDS ppts on these variables was due to missing or inadequate blood samples or ECG data
e14 FD ppts responded “don’t know” regarding self-reported stroke and had no stroke history documentation; these ppts were classified as “no stroke”