n-methyl-n-(tert-butyldimethylsilyl)trifluoroacetamide and Hyperoxaluria--Primary

Primary hyperoxalurias (PH) are inborn errors of glyoxylate metabolism characterized by an increase in endogenous oxalate production. Oxalate overproduction may cause calcium-oxalate crystal formation leading to kidney stones, nephrocalcinosis, and ultimately kidney failure. Twenty-four hour urine oxalate excretion is an inaccurate measure for endogenous oxalate production in PH patients and not applicable in those with kidney failure. Treatment efficacy cannot be assessed with this measure during clinical trials. We describe the development and validation of a gas chromatography-tandem mass spectrometry method to analyze the samples obtained following a stable isotope infusion protocol of

Topics: Acetamides; Carbon Isotopes; Fluoroacetates; Gas Chromatography-Mass Spectrometry; Glycolates; Glyoxylates; Humans; Hydroxylamines; Hyperoxaluria, Primary; Isotope Labeling; Organosilicon Compounds; Oxalates; Tandem Mass Spectrometry