Table 2:
Association between clinical covariates, PBA metabolite concentrations, and PBA metabolite ratios. All statistically significant associations have been marked in bold font.
| Variable | No. Observations | Value | Unadjusted Linear Regression Result (p-value) | ||||
|---|---|---|---|---|---|---|---|
| logPAA | logPBA | logPAGN | log(PAA:PAGN) | ||||
| Mean (SD) age at first assay (years) | 68 | 14.7 (14.8) | 0.0212 | 0.4088 | 0.1064 | 0.0404 | |
| Mean (SD) body surface area m2 | 68 | 1.1 (0.6) | 0.0032 | 0.6787 | 0.0799 | 0.0036 | |
| Diagnosis N (%) | 68 | CPS1D | 4 (5.9) | 0.0616 | 0.3435 | 0.1404 | 0.0599 |
| OTCD | 32 (47.1) | ||||||
| ASS1D | 15 (22.1) | ||||||
| ASLD | 4 (5.9) | ||||||
| ARG1D | 10 (14.7) | ||||||
| Others | 3 (4.4) | ||||||
| Mean (SD) GPB dose in g/m2/day | 68 | 4.9 (4.6) | 0.1243 | 0.4966 | 0.4639 | 0.0867 | |
| Mean (SD) NaPBA dose in g/m2/day | 68 | 3.4 (4.8) | 0.0019 | 0.3315 | 0.0401 | 0.0042 | |
| Mean (SD) Ammonia μmol/L | 46 | 34.4 (74.2) | 0.7397 | 0.4809 | 0.2871 | 0.6929 | |
| Mean (SD) AST U/L | 43 | 53.5 (40.8) | 0.7901 | 0.8684 | 0.3973 | 0.6485 | |
| Mean (SD) ALT U/L | 43 | 93.4 (147.1) | 0.3319 | 0.4976 | 0.4519 | 0.0179 | |
| Mean (SD) GGT U/L | 7 | 49.7 (47.0) | 0.0883 | 0.5375 | 0.3547 | 0.0144 | |
| Mean (SD) Glutamine μmol/L | 62 | 610.4 (261.2) | 0.0019 | 0.9903 | 0.0428 | 0.0013 | |
| Mean (SD) Glycine μmol/L | 62 | 249.0 (110.4) | 0.0042 | 0.3528 | 0.0652 | 0.0026 | |