egg-white and Biotinidase-Deficiency

Measurement of 3-hydroxyisovaleric acid (3HIA) in human urine has been shown to be a useful indicator of biotin status for a variety of clinical situations, including pregnancy. The work described herein presents a novel UPLC-MS/MS method for accurate and precise quantitation of urinary 3HIA. This method utilizes sample preparation prior to quantitation that has been simplified compared to the previous GC-MS method. To demonstrate the suitability of the UPLC-MS/MS method for human bio-monitoring, this method was used to measure 3-HIA in 64 human urine samples from eight healthy adults in whom marginal biotin deficiency had been induced experimentally by egg white feeding. 3HIA was detected in all specimens; the mean concentration [±standard deviation (SD)] was 80.6 ± 51 μM prior to inducing biotin deficiency. Mean excretion rate for 3HIA (expressed per mol urinary creatinine) before beginning the biotin-deficient diet was 8.5 ± 3.2 mmol 3HIA per mol creatinine and the mean increased threefold with deficiency. These specimens had been previously analyzed by GC-MS; the two data sets showed strong linear relationship with a correlation coefficient of 0.97. These results provide evidence that this method is suitable for bio-monitoring of biotin status in larger populations.

Topics: Adult; Biomarkers; Biotin; Biotinidase Deficiency; Calibration; Chromatography, Liquid; Creatinine; Egg White; Female; Humans; Male; Reference Standards; Reproducibility of Results; Sensitivity and Specificity; Tandem Mass Spectrometry; Valerates

Topics: Animals; Anti-Bacterial Agents; Biotin; Biotinidase Deficiency; Caproates; Egg White; Rats; Thiazolidines

Topics: Avitaminosis; Biotin; Biotinidase Deficiency; Cholesterol; Egg White; Humans

Topics: Animals; Avitaminosis; Biotinidase Deficiency; Egg White; Ovum; Swine; Vitamin B Complex; Vitamins