Jansen G, Muskiet F A, Schierbeek H, Berger R, van der Slik W
Clin Chim Acta. 1986 Jun 30;157(3):277-93. doi: 10.1016/0009-8981(86)90303-7.
A capillary gas chromatographic method for the profiling of trimethylsilylated mono- and disaccharides and polyols in urine, plasma and unwashed and washed erythrocytes is described. The prepurification method is based on the moderate inhibition of the derivatization experienced in the presence of physiological amounts of inorganic salts and the relative stability of the formed trimethylsilylethers towards treatment with water and dilute hydrochloric acid. Series to series quality control data for 31 sugars/polyols in a pooled urine are given. The method was used to establish age-dependent concentrations of 18 sugars/polyols in urines of 72 control persons on a free diet. Gas chromatographic profiles and quantitative data obtained from urines of pediatric patients with galactosemia treated with a diet low in lactose and galactose, type 1 hereditary tyrosinemia treated with a diet low in phenylalanine and tyrosine, and a neurological disorder with a high calorie gastric drip feeding, are presented and discussed. Examples of the profiling of sugars/polyols in the plasma, unwashed and washed erythrocytes of a healthy adult, and the plasma of a newborn with galactosemia prior to treatment, are given.
本文描述了一种毛细管气相色谱法,用于分析尿液、血浆以及未洗涤和洗涤过的红细胞中的三甲基硅烷基化单糖、双糖和多元醇。预纯化方法基于在生理量无机盐存在下衍生化受到适度抑制,以及所形成的三甲基硅烷基醚对水和稀盐酸处理具有相对稳定性。给出了混合尿液中31种糖/多元醇的系列间质量控制数据。该方法用于确定72名自由饮食的对照人员尿液中18种糖/多元醇的年龄依赖性浓度。展示并讨论了从接受低乳糖和半乳糖饮食治疗的半乳糖血症儿科患者、接受低苯丙氨酸和酪氨酸饮食治疗的1型遗传性酪氨酸血症患者以及采用高热量胃管滴注喂养的神经系统疾病患者的尿液中获得的气相色谱图谱和定量数据。还给出了健康成年人血浆、未洗涤和洗涤过的红细胞以及治疗前患有半乳糖血症的新生儿血浆中糖/多元醇分析的示例。