Table 1.
Baseline Characteristics of Patients Referred to the Neuromuscular Multidisciplinary Clinic
| Characteristic | All Patients (N=185) | Dystrophinopathies (n=32) | LGMD (n=33) | DM1 (n=60) | FSHD (n=20) | Non‐MD Myopathies (n=40) | P Valuea | P Valueb |
|---|---|---|---|---|---|---|---|---|
| Follow‐up time, y | 2.2 (1.4–3.5) | 2.2 (1.4–3.4) | 2.2 (1.7–3.6) | 2.3 (1.0–3.2) | 1.8 (0.9–3.4) | 2.3 (1.9–3.5) | 0.43 | 0.17 |
| Men/women, No. | 106 (57.3)/79 (42.7) | 32 (100) | 14 (42.4)/19 (57.6) | 30 (50)/30 (50) | 11 (55)/9 (45) | 19 (47.5)/21 (52.5) | <0.001 | 0.16 |
| Median age, y | 42 (25–54) | 22 (18–28.8) | 44 (27–57) | 42 (34.8–50) | 45 (24.8–52.5) | 54 (33.5–60) | <0.001 | 0.004 |
| Current/former smoker, No. | 22 (11.9) | 0 | 4 (12.1) | 11 (18.3) | 3 (15) | 4 (10) | 0.14 | 0.68 |
| Ambulation, No. | ||||||||
| Cane/walker | 16 (8.6) | 1 (3.1) | 3 (9.1) | 7 (11.7) | 2 (10) | 3 (7.5) | 0.70 | 0.77 |
| mWC/pWC | 49 (26.5) | 27 (84.4) | 12 (36.4) | 4 (6.7) | 3 (15) | 3 (7.5) | <0.001 | 0.002 |
| Comorbidities, No. | ||||||||
| Diabetes mellitus | 18 (9.7) | 0 | 8 (24.2) | 5 (8.3) | 2 (10) | 3 (7.5) | 0.03 | 0.59 |
| Dyslipidemia | 24 (13.0) | 2 (6.3) | 4 (12.1) | 7 (11.7) | 4 (20) | 7 (17.5) | 0.60 | 0.34 |
| Hypertension | 27 (14.6) | 2 (6.3) | 6 (18.2) | 4 (6.7) | 6 (30) | 9 (22.5) | 0.045 | 0.11 |
| Respiratory disease | 100 (54.1) | 27 (84.4) | 8 (24.2) | 32 (53.3) | 6 (30) | 27 (67.5) | <0.001 | 0.05 |
| SDOB | 47 (25.4) | 14 (43.8) | 5 (15.2) | 16 (36.7) | 6 (30) | 6 (15) | 0.07 | 0.09 |
| Anemia | 5 (2.7) | 3 (9.4) | 2 (6.1) | 0 | 0 | 0 | 0.022 | <0.001 |
| Lung volume recruitment, No. | 87 (47.0) | 26 (81.3) | 6 (18.2) | 27 (45) | 5 (25) | 23 (57.5) | <0.001 | 0.13 |
| Mechanical insufflation‐exsufflation, No. | 18 (9.7) | 13 (40.6) | 1 (3.0) | 1 (1.7) | 2 (10) | 1 (2.5) | <0.001 | 0.08 |
| Noninvasive ventilation, No. | 36 (19.5) | 12 (37.5) | 5 (15.2) | 8 (13.3) | 5 (25) | 6 (15) | 0.05 | 0.42 |
| Vitals, median | ||||||||
| HR, beats per min | 76 (67.5–86.5) | 86 (78.8–100) | 76 (70–83.5) | 70 (62.8–80) | 77.5 (71–80) | 73.5 (61–80) | 0.009 | 0.32 |
| SBP, mm Hg | 120 (110–132) | 108 (100–121) | 122 (112.5–131) | 114 (106–126.8) | 130 (120.5–133.8) | 126 (119.5–140) | <0.001 | <0.001 |
| DBP, mm Hg | 75 (70–82) | 71 (64–78) | 80 (70.5–84.5) | 74 (68.5–78) | 83.5 (76.5–87.5) | 76 (72–84) | 0.001 | 0.21 |
| Biomarkers, median | ||||||||
| BNP, pg/mL | 20 (12.5–42.5) | 20 (12–75) | 14.5 (10.5–47.5) | 21 (15–40) | 21 (14.5–35.5) | 20 (13–37) | 0.67 | 0.66 |
| CK, U/L | 274 (126–569.8) | 1277 (377.8–2380.8) | 526 (302.5–1759.5) | 237 (127.5–309) | 174.5 (159–395.3) | 86 (53–211) | <0.001 | <0.001 |
| Creatinine, μmol/L | 50.5 (29.3–67) | 20 (14.8–25) | 37 (25.3–56) | 57.5 (52.5–69.5) | 48 (39–60) | 58 (44.5–68.5) | <0.001 | 0.040 |
| Potassium, mmol/L | 4.3 (3.8–4.5) | 4.3 (4.0–4.5) | 4.1 (3.8–4.4) | 4.3 (3.9–4.5) | 4.3 (3.9–4.7) | 4.2 (4.0–4.4) | 0.86 | 0.78 |
Data are presented as median (interquartile range) or number (percentage). BNP indicates B‐type natriuretic peptide; CK, creatine kinase; DBP, diastolic blood pressure; DM1, type 1 myotonic dystrophy; FSHD, facioscapulohumeral muscular dystrophy; HR, heart rate; LGMD, limb‐girdle muscular dystrophy; mWC, manual wheelchair; pWC, power wheelchair; SBP, systolic blood pressure; SDOB, sleep‐disordered breathing.
a
Indicates statistical analysis comparing all patients.
b
Indicates statistical analysis comparing the cohorts of patients with muscular dystrophy (MD) and non‐MD myopathies.