| Budget Amount *help |
¥2,100,000 (Direct Cost: ¥2,100,000)
Fiscal Year 1996: ¥400,000 (Direct Cost: ¥400,000)
Fiscal Year 1995: ¥1,700,000 (Direct Cost: ¥1,700,000)
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| Research Abstract |
Organic acidurias are acute deteriorated fatal disorders. Clinical diagnosis is very difficult because pathognomonic symptoms are very few. It must requirechemical diagnosis by Gaschromatography/Massspectrometry (GC/MS). However, GC/MSsystem is so expensive that only few laboratories can make a diagnosis of organic aciduria. We have attempted to establish a high performance liquid chromatography (HPLC) method for the analysis of organic acids, which is less expensive and less laboratorious than GC/MS method.
The previous HPLC methods were less sensitive and less accurate for the analysis of organic acids. Our HPLC method was based on a separation technique of ion-exclusion and a detection technique of electron-conductivity. This method was enough sensitive to detect organic acids in the human neonate urine and enough accurate to separate known human organic acids. Moreover, analytical techniques were so simple that we could only inject urine sample into HPLC system without laboratorious extraction of organic acids from human urine nor derivatization of organic acids, which must be requied for GC/MS analysis.
By this method, we could detect the pathognomonic organic acid in the urinesfrom the patients with maple syrup urine disease, methylmalonic aciduria, alkaptonuria, isovaleric aciduria, glutaric aciduria, 3-hydroxy-3-methylglutaricaciduria, and lactic aciduria (mitochondria cytopathy).
Our HPLC method can make the diagnosis of organic acidurias earlier and earsier.
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