1950–1959 | 1960–1969 | 1970–1979 | 1980–1989 | 1990–1999 | 1950–1999 | |
Male patients | 846 | 861 | 861 | 1053 | 1333 | 4771 |
Mean age at onset | 33.3 | 32.9 | 33.6 | 34.9 | 36.4 | 34.5 |
% DMD treated* | 0.0 | 0.0 | 0.0 | 9.7 | 48.5 | 15.7 |
Observed deaths | 210 | 134 | 142 | 162 | 169 | 817 |
Expected deaths | 51.3 | 42.4 | 59.6 | 75.6 | 94.3 | 323.2 |
Patient-years of Observation | 12 475 | 10 192 | 13 121 | 16 121 | 20 443 | 72 352 |
EDR (95% CI) | 12.72 (10.52 to 15.16) | 8.99 (6.86 to 11.42) | 6.28 (4.57 to 8.21) | 5.36 (3.87 to 7.04) | 3.65 (2.45 to 5.00) | 6.82 (6.06 to 7.63) |
SMR (95% CI) | 4.09 (3.56 to 4.68) | 3.16 (2.65 to 3.75) | 2.38 (2.01 to 2.81) | 2.14 (1.83 to 2.50) | 1.79 (1.53 to 2.08) | 2.53 (2.36 to 2.71) |
Female patients | 1150 | 943 | 1313 | 1913 | 2754 | 8073 |
Mean age at onset | 32.6 | 32.3 | 32.5 | 33.3 | 34.9 | 33.5 |
% DMD treated* | 0.0 | 0.0 | 0.0 | 11.1 | 51.6 | 20.2 |
Observed deaths | 220 | 125 | 162 | 193 | 195 | 895 |
Expected deaths | 44.7 | 37.2 | 53.9 | 77.3 | 108.0 | 321.1 |
Patient-years of Observation | 17 108 | 14 321 | 20 161 | 29 562 | 42 772 | 1 23 992 |
EDR† (95% CI) | 10.25 (8.60 to 12.06) | 6.13 (4.67 to 7.80) | 5.36 (4.17 to 6.70) | 3.92 (3.03 to 4.91) | 2.03 (1.42 to 2.72) | 4.63 (4.17 to 5.12) |
SMR‡ (95% CI) | 4.92 (4.29 to 5.61) | 3.36 (2.80 to 4.00) | 3.01 (2.56 to 3.51) | 2.50 (2.16 to 2.88) | 1.81 (1.56 to 2.08) | 2.79 (2.61 to 2.98) |
All patients | 1996 | 1624 | 2174 | 2966 | 4087 | 12 847 |
% female | 57.6 | 58.1 | 60.0 | 64.5 | 67.4 | 62.8 |
Mean age at onset (SD) | 32.9 (9.47) | 32.6 (9.94) | 32.9 (10.01) | 33.9 (9.92) | 35.4 (10.60) | 33.9 (10.40) |
% DMD treated* | 0.0 | 0.0 | 0.0 | 10.6 | 50.6 | 18.5 |
Observed deaths | 430 | 259 | 304 | 355 | 364 | 1712 |
Expected deaths | 96.1 | 79.6 | 113.5 | 152.8 | 202.3 | 644.3 |
Patient-years of observation | 29 583 | 24 512 | 33 282 | 45 683 | 63 215 | 1 96 273 |
EDR† (95% CI) | 11.29 (9.95 to 12.73) | 7.32 (6.07 to 8.69) | 5.72 (4.73 to 6.81) | 4.43 (3.64 to 5.28) | 2.56 (1.98 to 3.18) | 5.44 (5.03 to 5.86) |
SMR‡ (95% CI) | 4.48 (4.06 to 4.92) | 3.25 (2.87 to 3.68) | 2.68 (2.39 to 3.00) | 2.32 (2.09 to 2.58) | 1.80 (1.62 to 1.99) | 2.66 (2.53 to 2.79) |
*Patients who had been treated with disease-modifying drugs within the 15-year observation time.
†Excess number of deaths per 1000 person-years.
‡Number of observed deaths/expected deaths.
EDR, excess death rate; SMR, standardised mortality ratio.