Table 3.
Nutrient Metabolism
| Variable | Control (n=5) | BTHS (n=5) |
|---|---|---|
| Basal | ||
| Energy Expenditure (kcal/day) | 1746 ± 139 (1732-1879) | 1329 ± 202* (1122-1585) |
| Energy Expenditure (kcal/kgFFM/day) | 37.9 ± 6.6 (33.3-49.6) | 43.5 ± 3.8 (33.6-56.1) |
| Endogenous Glucose Ra (μmol/min) | 1053 ± 129 (867-1194) | 733 ± 101* (631-880) |
| Endogenous Glucose Ra (μmol/kgFFM/min) | 22.7 ± 3.0 (18.9-26.7) | 24.0 ± 4.5 (20.5-31.5) |
| Carbohydrate oxidation (g/day) | 198.6 ± 103.2 (68.0-328.0) | 119.5 ± 33.8 (84.6-159.3) |
| Carbohydrate oxidation (g/kgFFM/day) | 4.2 ± 2.4 (1.3-6.8) | 3.7 ± 0.6 (2.7-4.3) |
| Palmitate Ra (μmol/min) | 84.0 ± 46.5 (41.0-151.7) | 62.4 ± 16.9 (45.7-84.4) |
| Palmitate Ra (μmol/kgFM/min) | 11.9 ± 4.4 (6.3-12.2) | 4.6 ± 2.7** (2.4-8.4) |
| Palmitate Ra (μmol/kgFFM/min) | 1.8 ± 0.9 (0.8-3.0) | 2.0 ± 0.3 (1.6-2.3) |
| FAOX (μmol/min) | 10.8 ± 6.0 (5.0-19.6) | 9.8 ± 1.0 (8.9-11.1) |
| FAOX (μmol/kgFFM/min) | 0.23 ± 0.11 (0.10-0.39) | 0.31 ± 0.08 (0.28-0.44) |
| Non-oxidative FA Disposal (μmol/min) | 70.2 ± 38.9 (40.4-126.4) | 50.6 ± 15.1 (35.6-70.1) |
| Non-oxidative FA Disposal (μmol/ kgFFM/min) | 1.5 ± 0.7 (0.7-2.5) | 1.6 ± 0.3 (1.3-1.8) |
| Fasting Serum Leucine (μM) | 144.6 ± 23.8 (121.7-177.0) | 144.1 ± 29.5 (126.5-190.2) |
| Leucine Ra (μmol/min) | 9071 ± 2008 (6522-11243) | 8211 ± 1436 (7236-10016) |
| Leucine Ra (μmol/kgFFM/min) | 193.1 ± 28.7 (172.1-227.8) | 271.4 ± 69.3 (195.7-361.8) |
| Acetate Correction Factor (%) | 31 ± 6 (27-41) | 28 ± 7 (20-37) |
| Hyperinsulinemia | ||
| Energy Expenditure (kcal/day) | 1770 ± 75 (1642-1834) | 1424 ± 212* (1130-1639) |
| Energy Expenditure (kcal/kgFFM/day) | 38.2 ± 3.4 (35.2-43.3) | 47.3 ± 12.7 (32.0-65.4) |
| Carbohydrate oxidation (g/day) | 302.7 ± 53.7 (255.1-365.3) | 284.1 ± 110.1 (182.9-462.0) |
| Carbohydrate oxidation (g/kgFFM/day) | 6.3 ± 1.8 (4.6-9.2) | 9.1 ± 3.8 (5.0-14.8) |
| Endogenous Glucose Ra (μmol/min) | 248.4 ± 117.3 (97.4-402.2) | 300.9 ± 220.6 (85.4-596.6) |
| Endogenous Glucose Ra (μmol/kgFFM/min) | 5.2 ± 2.1 (2.1-7.9) | 9.4 ± 6.6** (2.5-19.6) |
| Palmitate Ra (μmol/min) | 22.2 ± 7.0 (17.6-33.8) | 23.6 ± 3.2 (21.5-25.2) |
| Palmitate Ra (μmol/kgFM/min) | 3.6 ± 1.4 (1.9-5.2) | 1.6 ± 0.4* (1.4-2.1) |
| Palmitate Ra (μmol/kgFFM/min) | 0.5 ± 0.1 (0.4-0.7) | 0.8 ± 0.4 (0.6-1.4) |
| FAOX (μmol/min) | 7.0 ± 1.0 (5.7-8.1) | 7.4 ± 1.6 (5.4-9.1) |
| FAOX (μmol/kgFFM/min) | 0.15 ± 0.02 (0.12-0.18) | 0.25 ± 0.13 (0.21-0.46) |
| Non-oxidative FA Disposal (μmol/min) | 15.2 ± 6.2 (11.9-25.7) | 16.2 ± 2.3 (15.0-18.1) |
| Non-oxidative FA Disposal (μmol/kgFFM /min) | 0.32 ± 0.10 (0.25-0.50) | 0.54 ± 0.24 (0.36-0.91) |
| Fasting Serum Leucine (μM) | 90.5 ± 35.1 (60.6-146.3) | 61.3 ± 7.5** (50.7-69.5) |
| Leucine Ra (μmol/min) | 5908 ± 1456 (4504-8383) | 4959 ± 563 (4236-5627) |
| Leucine Ra (μmol/ kgFFM /min) | 125.8 ± 21.8 (115.1-164.1) | 167.9 ± 58.0 (111.7-260.1) |
| Delta baseline→hyperinsulinemia (μmol/ kgFFM /min) | -67.2 ± 25.5 (-53.2- -112.7) | -103.5 ± 28.7 (-60.5- 136.6) |
| Delta baseline→hyperinsulinemia (%) | 34 ± 9 (31-49) | 39 ± 9 (31-48) |
BTHS: Barth syndrome, Ra: rate of appearance, Rd: rate of disappearance, FAOX: fatty acid oxidation, μM: micromolar, FFM: fat free mass, FM: fat mass
*
p<0.01
**
p<0.05