New organic acid analysis for inherited organic acidemia

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Screening urine for inherited organic acidemia required complex and time consuming method.We have developed new organic acid analysis uring liquid partition chromatography. and gas chromatography mass spectrometry. This system was connected with reverse-search method using personal computor. This method enabled to abolish the mistakes due to manual procedure and save time. Maximal sensitivity is also due to liquid partition chromatography using silicic column liquid partition chromatography. Using this method, we found many patients with inherited organic acidemia and urea cycle disorders. This method was especially useful for the detection of methylcitric acid and other polar compound. Conventional method using organic solvent extraction used to fail the detection of polar cpmpounds.Because methylmalonic acidemia and propionic acidemia are major organic acidemia, the accurate detection and quantification is critically important for the diagnosis.