Laboratory Medicine and Quality Assurance

Table. 11.

Proficiency testing results for organic acid analysis (unit, mmol/mol creatinine)

Year Trial Specimen no. Intended response Reported response No. Reported components No. Median Minimum Maximum
2020 1st CMO-20-01 Tyrosinemia type I Tyrosinemia type I 4 4-Hydroxyphenylacetic 4 388.8 298.3 487.9
4-Hydroxyphenyllactic 3 708.6 307.1 985.4
4-Hydroxyphenylpyruvic 4 678.9 352.5 834.2
4-OH phenyllactic acid 1 561.3
Lactic 1 119.5
Succinylacetone 4 813.2 585.1 55,604.9
CMO-20-02 Ketosis Ketosis 4 3-Hydroxybutyric 4 231 63.9 831
Acetoacetic 3 696.7 115 1,763.5
Lactic 1 157
CMO-20-03 No abnormality No abnormality 3
Others 1 Lactic 1 171.8
2nd CMO-20-04 Short chain acyl CoA dehydrogenase deficiency Short chain acyl CoA dehydrogenase deficiency 3 3-Hydroxybutyric 4 175 106.1 230
Ethylmalonic encephalopathy 1 Methylsuccinic 3 56.6 33.6 69.6
CMO-20-05 Propionic acidemia Propionic acidemia 4 3-Hydroxypropionic 4 725.9 342 2,459
Ethylmalonic 4 289.2 56.5 769.5
Methylcitric 4 150.1 41 296.3
Propionylglycine 4 195.6 99.9 356.2
CMO-20-06 No abnormality No abnormality 4
2021 1st CMO-21-01 Ornithine transcarbamylase deficiency Ornithine transcarbamylase deficiency 3 Orotic 3 101.5 86.7 447.3
No abnormality 1 Uracil 3 54.8 43.4 275.8
CMO-21-02 No abnormality No abnormality 3
Urea cycle disorder 1 Orotic 1 18.3
CMO-21-03 No abnormality No abnormality 4
2nd CMO-21-04 Methylmalonic acidemia Methylmalonic acidemia 4 3-Hydroxypropionic 4 259.2 172.5 406.7
Methylmalonic 4 530.4 233.9 905
Methylcitric 4 95.1 7.1 166.3
CMO-21-05 No abnormality No abnormality 4
CMO-21-06 No abnormality No abnormality 4
Lab Med Qual Assur 2022;44:143~158 https://doi.org/10.15263/jlmqa.2022.44.3.143
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