Proficiency testing results for organic acid analysis (unit, mmol/mol creatinine)
| Year | Trial | Specimen no. | Intended response | Reported respons | No. | Reported components | No. | Median | Minimum | Maximum |
|---|---|---|---|---|---|---|---|---|---|---|
| 2018 | 1st | CMO-18-01 | Tyrosinemia type I | Tyrosinemia type I | 5 | 4-Hydroxyphenylacetic acid | 5 | 350 | 145.7 | 903.4 |
| 4-Hydroxyphenylpyruvic acid | 5 | 1,224.6 | 281.5 | 1,527.8 | ||||||
| Succinylacetone | 5 | 347.3 | 157.5 | 465.6 | ||||||
| CMO-18-02 | Propionic aciuria | Propionic acidemia | 5 | 3-Hydroxypropionic acid | 5 | 324.3 | 104.2 | 467.2 | ||
| Methylcitric acid | 5 | 55.1 | 15.6 | 422.8 | ||||||
| CMO-18-03 | No abnormality | No abnormality | 5 | |||||||
| 2nd | CMO-18-04 | Glutaric acidemia type II | Glutaric acidemia type II | 4 | Ethylmalonic acid | 4 | 345.5 | 122.9 | 486.3 | |
| Glutaric acid | 4 | 583.6 | 190.2 | 1,116.4 | ||||||
| Lactic acid | 3 | 265.1 | 171.4 | 954.1 | ||||||
| CMO-18-05 | Combined malonic/methylmalonic aciduria | Combined malonic/methylmalonic aciduria | 3 | Malonic acid | 3 | 40.2 | 40.1 | 188.2 | ||
| Methylmalonic acidemia | 1 | Methylmalonic acid | 4 | 312.1 | 130.1 | 364.5 | ||||
| CMO-18-06 | No abnormality | No abnormality | 4 | |||||||
| 2019 | 1st | CMO-19-01 | Methylmalonic acidemia | Methylmalonic acidemia | 4 | 3-Hydroxypropionic acid | 4 | 207.3 | 131.2 | 395.4 |
| Methylcitric acid | 3 | 35.1 | 19.6 | 40.9 | ||||||
| Methylmalonic acid | 4 | 707.2 | 150 | 898 | ||||||
| CMO-19-02 | Ornithine transcarbamylase deficiency/urea cycle disorder | Ornithine transcarbamylase deficiency | 2 | Orotic acid | 4 | 835.1 | 86.6 | 4,482.4 | ||
| Orotic aciduria | 1 | Oxalic acid | 2 | 117.7 | 822.2 | |||||
| Urea cycle disorder | 1 | Uracil | 4 | 1,183.3 | 88.3 | 2,042.6 | ||||
| CMO-19-03 | No abnormality | No abnormality | 4 | Others | 3 | |||||
| 2nd | CMO-19-04 | Glutaric acidemia type I | Glutaric acidemia type I | 4 | 3-Hydroxyglutaric acid | 3 | 48.8 | 22.6 | 62.1 | |
| Glutaconic acid | 3 | 192.3 | 123.4 | 220.2 | ||||||
| Glutaric acid | 4 | 240.6 | 134.9 | 280 | ||||||
| CMO-19-05 | Maple syrup urine disease | Maple syrup urine disease | 4 | 2-Hydroxyisovaleric acid | 4 | 296.5 | 251.9 | 1,347.6 | ||
| 2-keto isocaporic acid | 4 | 484.8 | 9.9 | 1,031.2 | ||||||
| 2-keto-3-methylvaleric acid | 3 | 201.9 | 4.9 | 382.5 | ||||||
| CMO-19-06 | No abnormality | No abnormality | 4 |