Table 1 Demographic features and clinical characteristics of 362 DMD/BMD patients and carriers
DMD | BMD | Functionally symptomatic carrier DMD | Functionally asymptomatic carrier DMD | Functionally symptomatic carrier BMD | Functionally asymptomatic carrier BMD | |
|---|---|---|---|---|---|---|
n | 55 | 88 | 10 | 130 | 4 | 75 |
Median age (yr) (range) | 12 (8–44) | 30 (8–59) | 56 (27–69) | 44 (8–68) | 47 (22–64) | 44 (16–72) |
Median disease duration (yr) (range) | 10 (4–41) | 20 (1–52) | 10 (0–36) | na | 26 (7–44) | na |
Median vital capacity (%) (range) | 52 (14–84) | 88 (18–161) | 87 (69–125) | 106 (61–203) | 115 (41–122) | 108 (61–166) |
Median diastolic blood pressure (mmHg) (range) | 66 (44–87) | 76 (48–112) | 82 (67–99) | 83 (56–116) | 77 (73–90) | 77 (60–108) |
Hypertension + | 2 (4%) | 26 (30%) | 7 (70%) | 54 (42%) | 1 (25%) | 23 (31%) |
Wheelchair bound | 34 (62%) | 12 (14%) | 0% | na | 0% | na |
Treatment # | 3 (6%) | 17 (19%) | 3 (30%) | 14 (11%) | 1 (25%) | 4 (5%) |
Any Symptom CHF* | 6 (11%) | 15 (17%) | 7 (70%) | 30 (23%) | 3 (75%) | 19 (25%) |
Obesity § | 0 | 5 (6%) | 6 (30%) | 19 (16%) | 1 (25%) | 9 (13%) |