Table 3.
Body composition and resting energy expenditure in SOD1 vs. C9orf72 vs. controls.
| SOD1 (n = 22) | C9orf72 (n = 28) | NC (n = 73) | 3-group comparison | Median difference (95% CI); SOD1vs. NC | Median difference (95% CI); C9orf72vs. NC | Median difference (95% CI); C9orf72 vs. SOD1 | |
|---|---|---|---|---|---|---|---|
| BMI (kg/m2) | 28.0 (25.8–32.4) | 22.5 (20.7–25.9) | 24.9 (22.8–29.0) | p = 0.004 | 3.1 (−1.0 to 5.7) p = 0.12 (0.45) | −2.4 (−4.9 to −0.9) p = 0.007 (0.02) | −5.5 (−8.7 to −2.6) p = 0.004 (0.005) |
| FM (kg) | 25.8 (16.7–36.3) | 19.1 (13.0–21.3) | 21.4 (16.8–30.0) | p = 0.06 | 4.4 (−2.9 to 10.9) p = 0.25 (0.70) | −2.3 (−8.3 to 0.8) p = 0.09 (0.28) | −6.7 (−15.8 to −0.8) p = 0.02 (0.06) |
| Fat (%) | 32.2 (25.0–38.5) | 29.0 (21.5–33.3) | 27.6 (21.3–34.0) | 0.48 | 4.6 (−2.6 to 9.1) p = 0.26 (0.70) | 1.4 (−4.5 to 5.3) p = 0.93 (>0.99) | −3.2 (−8.9 to 3.0) p = 0.29 (0.99) |
| LBM (kg) | 64.1 (48.0–67.5) | 46.8 (43.6–52.0) | 58.0 (50.1–66.9) | p < 0.001 | 6.1 (−5.1 to 10.6) p = 0.57 (>0.99) | −11.2 (−14.5 to −4.8) p < 0.001 (0.001) | −17.3 (−19.8 to −3.9) p = 0.003 (0.007) |
| ECM (kg) | 28.9 (25.1–35.0) | 23.6 (20.5–26.3) | 26.9 (23.4–31.0) | p = 0.002 | 2.0 (–0.7 to 5.9) p = 0.11 (0.33) | −3.3 (−5.9 to −1.1) p = 0.005 (0.02) | −5.3 (−9.8 to −2.5) p < 0.001 (0.002) |
| BCM (kg) | 30.7 (22.3–36.6) | 23.7 (21.5–25.8) | 29.6 (25.6–36.6) | p = 0.001 | 1.1 (−5.4 to 4.6) p = 0.91 (>0.99) | −5.9 (−8.7 to −2.8) p < 0.001 (<0.001) | −7.0 (−10.5 to −0.1) p = 0.04 (0.04) |
| Cells (%) | 48.6 (46.4–55.6) | 51.8 (47.6–54.3) | 51.9 (50.0–55.5) | p = 0.16 | −3.3 (−5.5 to 0.4) p = 0.08 (0.26) | −0.1 (−3.5 to 0.9) p = 0.25 (0.70) | 3.2 (−2.8 to 5.4) p = 0.60 (>0.99) |
| ECM/BCM | 1.05 (0.80–1.16) | 0.93 (0.84–1.10) | 0.93 (0.80–1.00) | p = 0.16 | 0.12 (−0.02 to 0.22) p = 0.08 (0.26) | −0.00 (−0.13 to 0.03) p = 0.23 (0.66) | −0.12 (−0.20 to 0.11) p = 0.63 (>0.99) |
| BW (kg) | 46.9 (35.2–49.4) | 34.3 (31.9–38.1) | 42.5 (36.7–49.0) | p < 0.001 | 4.4 (−3.8 to 7.7) p = 0.57 (>0.99) | −8.2 (−10.6 to −3.5) p < 0.001 (0.001) | −12.6 (−14.5 to −2.8) p = 0.004 (0.008) |
| Phase Angle (°) | 5.4 (5.0–6.8) | 6.0 (5.1–6.5) | 6.0 (5.6–6.8) | p = 0.17 | −0.6 (−1.1 to 0.1) p = 0.09 (0.28) | 0.0 (−0.2 to 0.7) p = 0.22 (0.64) | 0.6 (−0.6 to 1.0) p = 0.62 (>0.99) |
| mRMR (kcal/d) | 1830 (1401–2080) | 1507 (1326–1670) | 1785 (1548–2133) | p = 0.001 | 45 (−249 to 184) p = 0.72 (>0.99) | −278 (−504 to −147) p < 0.001 (<0.001) | −323 (−532 to −69) p = 0.008 (0.04) |
| MR | 1.04 (0.95–1.12) | 1.05 (0.98–1.13) | 1.11 (1.02–1.16) | p = 0.02 | −0.07 (−0.12 to 0.00) p = 0.06 (0.08) | −0.06 (0.00 to –0.12) p = 0.05(0.07) | 0.01 (−0.08 to 0.07) p = 0.99 (>0.99) |
| NfL (pg/ml) | 12.5 (10.0–23.5) | 14.0 (10.5–19.0) | 15.0 (10.0–21.0) | p = 0.72 | −2.5 (−5.0 to 2.0) p = 0.44 (>0.99) | −1.0 (−4.0 to 3.0) p = 0.75 (>0.99) | 1.5 (−3.0 to 5.0) p = 0.61 (>0.99) |
Data are median (IQR), Wilcoxon Rank Sum test (2-group comparisons), Kruskal–Wallis (3-group comparisons).
NC: Non-Carriers. BMI: Body Mass Index. FM: Fat Mass. LBM: Lean Body Mass. ECM: Extracellular Mass. BCM: Body Cell Mass. BW: Body Water. mRMR: measured Resting Metabolic Rate. MR: Metabolic Ratio. NfL: Neurofilament light chain blood levels.
Bold values indicate statistical significance. Underlined values indicate p-values between 0.05 and 0.10. p-values adjusted for multiple testing (Dunn's test) are given in brackets.