Table 1.
Participant demographics
| BTHS (N = 9) | CON (N = 7) | P value | |
|---|---|---|---|
| Anthropometrics | |||
| Age (year) | 23 (7) | 26 (6) | 0.25 |
| Height (cm) | 177 (9) | 179 (4) | 0.53 |
| Weight (kg) | 63.5 (15) | 77 (7) | 0.04 |
| BMI (kg/m2) | 20 (4.4) | 24 (2.2) | 0.06 |
| Lean mass (kg) | 39.5 (7.6) | 57.7 (3.7) | 0.01 |
| Fat mass (kg) | 21.4 (8.5) | 14.6 (3.3) | 0.09 |
| Fat mass (%) | 36 (7) | 20 (2.8) | 0.01 |
| Arm fat (kg) | 1.2 (0.56) | 0.7 (0.19) | 0.08 |
| Arm lean (kg) | 1.9 (0.4) | 3.1 (0.4) | <0.01 |
| Leg fat (kg) | 3.6 (1.6) | 2.7 (0.8) | 0.27 |
| Leg lean (kg) | 6.1 (1.2) | 9.7 (0.9) | <0.01 |
| Trunk fat (kg) | 10.8 (4.5) | 6.4 (1.2) | 0.04 |
| Trunk lean (kg) | 20.2 (3.8) | 27.6 (1.8) | <0.01 |
| Skeletal muscle index (%) | 29 (0.01) | 39 (0.01) | <0.01 |
| Arm BMD (g/cm2) | 0.60 (0.05) (L)a | 0.84 (0.03) | <0.01 |
| Leg BMD (g/cm2) | 0.88 (0.11) (L)a | 1.4 (0.11) | <0.01 |
| Thoracic spine BMD (g/cm2) | 0.68 (0.10) (L)a | 0.93 (0.13) | <0.01 |
| Lumbar spine BMD (g/cm2) | 0.80 (0.11) (L)a | 1.15 (0.13) | <0.01 |
| Pelvic BMD (g/cm2) | 0.84 (0.12) (L)a | 1.23 (0.09) | <0.01 |
| Cardiac function/exercise tolerance | |||
| VO2peak (mL/kg/min) | 11.2 (1.9) | 47.8 (11) | <0.01 |
| Max work rate (W) | 48 (11) | 242.1 (41) | <0.01 |
| Creatinine (mg/dL) | 0.5 (0.1) | 0.8 (0.0) | <0.01 |
| Resting HR (bpm) | 82.2 (9.4) | 63.1 (6.8) | <0.01 |
| Resting SBP (mmHg) | 102 (8.9) | 127 (8.4) | <0.01 |
| Resting DBP (mmHg) | 60 (14) | 74 (9) | 0.05 |
| LVM 2D | 136 (29) | 139 (25) | 0.81 |
| Ejection fraction (%) | 59 (15) | 60 (6) | 0.80 |
| Fractional shortening (%) | 31 (9) | 40 (9) | 0.07 |
| E/A | 1.7 (0.4) | 1.8 (0.2) | 0.50 |
| Global strain (%) | −16 (3.5) | −18 (2.6) | 0.18 |
| Total CK (U/L) | 79.3 (51.7) | 90.4 (26.6) | 0.67 |
| BNP (pg/mL) | 44.4 (39) | 11.7 (2.9) | 0.17 |
| Blood chemistries | |||
| Glucose (fasting) | 97.8 (27.6) | 87.8 (6.8) | 0.41 |
| HCT (%) | 41.6 (3.7) | 41.4 (2.4) | 0.90 |
| ANC (cells/mm3) | 1.0 (0.7) | 2.8 (0.4) | <0.01 |
Values are mean (SD)
VO2peak peak oxygen consumption, BMD bone mineral density, HCT hematocrit, ANC absolute neutrophil count, HR heart rate, SBP systolic blood pressure, DBP diastolic blood pressure, LVM 2D left ventricular mass measured by 2-dimensional echocardiography, E/A early to late filling ratio, CK creatine kinase, BNP brain natriuretic peptide
a“L” denotes that the values are <1 standard deviation below age-specific normative values for male bone mineral density as reported by 1999–2006 NHANES survey