Body Composition, Muscle Strength, and Physical Function of Patients with Bethlem Myopathy and Ullrich Congenital Muscular Dystrophy

Table 2

Muscle strength and measured physical function. Summaries of individual data grouped according to sex.

Variable	Women (𝑛=5)	Men (𝑛=3)
Hand grip (N)	47.3±18.8; (range: 12–73)	99.0±22.8; (range: 67–119)
Elbow flexion (N)	38.8±13.7; (range: 10–52)	99.3±25.0; (range: 59–133)
Knee extension (N)	76.2±38.2; (range: 24–136)	126.3±77.6; (range: 50–225)
Knee flexion (N)	76.7±31.5; (range: 18–107)	123.0±55.4; (range: 54–185)
Megascore (N)	478±199; (range: 160–680)	895±286; (range: 651–1210)
10 m (1/𝑇)	0.11±0.07; (range: 0–0.17)	0.13±0.04; (range: 0.09–0.18)
4 steps (1/𝑇)	0.14±0.09; (range: 0–0.22)	0.24±0.09; (range: 0.17–0.33)
6MWD (m)	335±190.2; (range: 0–458)	411±104.5; (range: 320–525)
FVC (mL)	1788±863; (range: 460–2670)	3687±832; (range: 2760–4370)
% FVC	49±23.0; (range: 14–72)	73.0±17.3; (range: 53–84)

N: Newton, 𝑇: time, 6MWD: 6 minute walking distance, FVC: forced vital capacity.