Table 4.
Laboratory results for Biomarkers (Ammonia, Glutamine, Citrulline and Arginine (n, mean+ standard deviation, median, (range)),
| Biomarker | ASD Citrullinemia | ASA | ARGD |
|---|---|---|---|
|
Ammonia (Reference range: 28–80 umol/L) |
|||
| Number of hyperammonemic episodes | 63 5.6± 7.08 3 (1–32) |
63 4.14± 6.09 1 (1–27) |
15 3.93± 5.82 1 (1–22) |
| Concurrent level | 23 44.71± 43.49 30 (9–206) |
20 25.08± 17.28 22.5 (9.0–82.0) |
2 5.28, 67.0 36.14 (5.28–67.0) |
| Mean lifetime level | 62 127.22± 110.51 108.86 (6.30–539.25) |
59 139.59± 164.89 80 (1–882) |
16 78.87± 51.84 77.5 (5.28–169.33) |
| Standard deviation score* | 53 151.06± 168.03 101.22 (4.03–780.32) |
48 169.02± 251.57 83.76 (3.27–1164.06) |
12 46.46± 34.87 35.31 (5.80–108.93) |
| Number of “spikes”** | 63 4.56± 6.16 2 (0–30) |
60 3.47± 5.67 1 (0–25) |
16 2.38± 4.77 0.5 (0–19) |
|
Glutamine Reference range: 332-1084 umol/L |
|||
| Concurrent | 23 603.55± 1.95.49 606 (121–932) |
24 638.31± 198.80 626 (36–1020) |
3 515.00± 74.28 488 (458–599) |
| Mean | 63 687.16± 205.71 657.65 (347.00–1442.00) |
63 706.94± 222.85 682.36 (334.0–1997.0) |
16 632.71± 167.81 594.6 (374.5–1026.2) |
| Standard deviation score | 61 197.78± 174.11 163.54 (1.41–1223.86) |
55 231.45± 347.49 139.63 (24.04–2444.84) |
16 161.62± 77.86 146.54 (61.94–326.75) |
|
Citrulline Reference range: 2-50 umol/L |
|||
| Concurrent | 23 1804.48± 1284.18 2161.0 (86.4–4766.0) |
24 159.68± 51.15 156 (77–284) |
3 28.33± 5.03 29.0 (23–33) |
| Mean | 62 1857.97± 1141.31 1836.56 (71.31–4752.00) |
63 194.37± 98.06 168.68 (42.00–554.33) |
16 24.16± 5.43 23.44 (17.76–34.67) |
| Standard deviation score | 61 700.62± 810.50 508.00 (26.66–5106.23) |
54 92.36± 105.43 51.01 (6.01–566.31) |
16 6.16± 4.07 5.81 (0–15.82) |
|
Arginine Reference range: 16-149 umol/L |
|||
| Concurrent | 23 69.15± 50.90 54.7 (5.0–194.0) |
24 76.39± 45.89 64 (20–220) |
3 388.67± 85.19 362 (320–484) |
| Mean | 62 128.17± 185.30 101.94 (45.30–1507.33) |
63 107.60± 66.51 95.8 (26.6–444.0) |
16 371.16± 121.26 353.98 (181—535.33) |
| Standard deviation score | 61 96.29± 319.46 44.04 (2.92–2511.93) |
54 71.169± 69.61 55.46 (4.24–445.27) |
16 105.86± 64.13 113.38 (0–283.89) |
n=number of participants
*
Standard deviation score = the mean degree of dispersion in laboratory values around the mean value for the individual participant was labeled the standard deviation score.
**
Ammonia “spikes” = an ammonia level greater than 10 umol/L above the mean for the individual participant