Table 1

Fifteen-year excess mortality by onset cohort

1950–19591960–19691970–19791980–19891990–19991950–1999
Male patients846861861105313334771
Mean age at onset33.332.933.634.936.434.5
% DMD treated*0.00.00.09.748.515.7
Observed deaths210134142162169817
Expected deaths51.342.459.675.694.3323.2
Patient-years of
Observation
12 47510 19213 12116 12120 44372 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 patients11509431313191327548073
Mean age at onset32.632.332.533.334.933.5
% DMD treated*0.00.00.011.151.620.2
Observed deaths220125162193195895
Expected deaths44.737.253.977.3108.0321.1
Patient-years of
Observation
17 10814 32120 16129 56242 7721 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 patients1996162421742966408712 847
% female57.658.160.064.567.462.8
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*0.00.00.010.650.618.5
Observed deaths4302593043553641712
Expected deaths96.179.6113.5152.8202.3644.3
Patient-years of observation29 58324 51233 28245 68363 2151 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.