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A urine volume corresponding to 0.25 mg of creatinine is acidified,
then extracted with ethyl acetate. After separation and evaporation
of the solvent phase, the dry residue is sialylated and analyzed by
capillary GC-MS. When indicated, oximation of 2-keto acids is
performed by reaction with hydroxylamine hydrate. (Sweetman L:
Organic acid analysis. In Techniques in Diagnostic Human
Biochemical Genetics. Edited by FA Hommes. Wiley-Liss, New
York, 1991, pp 143-176; ; Hoffman GF, Feyh P: Organic acid
analysis. In Physician's Guide to the Laboratory Diagnosis of
Metabolic Diseases. Edited by N Blau, M Duran, ME Blaskovics
KM Gibson. Berlin, NY, Springer, 2003, pp 27-44)

