From: Consequences of a global enzyme shortage of agalsidase beta in adult Dutch Fabry patients
 | Before Shortage | During Shortage |  |  |  |  | ||
---|---|---|---|---|---|---|---|---|
Male (n = 14) | Mean | SD | Mean | SD | Mean difference | CI (95%) Mean difference | p | |
 |  |  |  |  |  | Upper | Lower |  |
Physical functioning | 60.0 | 30.1 | 59.6 | 26.5 | 0.4 | -9.5 | 103 | 0.75 |
Role physical | 43.2 | 40.4 | 32.4 | 35.3 | 10.7 | -11.9 | 33.2 | 0.24 |
Bodily pain | 64.3 | 24.9 | 60.1 | 19.2 | 4.2 | -7.7 | 16.1 | 0.53 |
General health | 31.2 | 17.4 | 36.5 | 24.8 | -5.4 | -14 | 3.2 | 0.34 |
Vitality | 50.2 | 17.5 | 50.0 | 19.6 | 0.12 | -7.5 | 7.7 | 0.7 |
Social functioning | 66.5 | 30.3 | 59.8 | 21.9 | 6.7 | -4 | 17.4 | 0.18 |
Role emotional | 57.1 | 51.4 | 66.3 | 39.6 | -9.1 | -29.5 | 11.3 | 0.4 |
Mental health | 74.2 | 17.4 | 78.8 | 13.3 | -4.6 | -12.1 | 2.8 | 0.25 |
 | Before Shortage | During shortage |  |  |  |  | ||
Female (n = 16) | Mean | SD | Mean | SD | Mean difference | CI (95%) Mean difference | p | |
 |  |  |  |  |  | Upper | Lower |  |
Physical functioning | 61.5 | 26.3 | 58.4 | 24.8 | 3.1 | -1.9 | 8.1 | 0.10 |
Role physical | 36.8 | 39.8 | 27.6 | 40.2 | 9.2 | -3.4 | 21.9 | 0.11 |
Bodily pain | 62.3 | 23.7 | 61.0 | 23.5 | 1.3 | -6.8 | 9.5 | 0.74 |
General health | 41.4 | 18.9 | 37.6 | 20.9 | 3.8 | 0 | 7.6 | 0.03 |
Vitality | 42.7 | 22.1 | 36.5 | 23.1 | 6.1 | 2.8 | 9.5 | 0.003 |
Social functioning | 68.5 | 18.6 | 63.1 | 27.3 | 5.5 | -0.9 | 11.8 | 0.11 |
Role emotional | 72.4 | 34.5 | 57.3 | 35.5 | 15.1 | -2.7 | 32.9 | 0.15 |
Mental health | 70.7 | 15.6 | 66.4 | 19.6 | 4.3 | -1.1 | 9.7 | 0.10 |