大骨节病患儿尿氨基多糖组分和二糖谱研究

STUDY ON DISACCHARIDES AND COMPONENTS OF GLYCOSAMINOGLYCAN IN URINE FROM CHILDREN WITH KASCHIN BECK DISEASE

用高效液相色谱等方法分析了大骨节病患儿和非患儿尿氨基多糖中四种二糖和3组分以及血、尿、发硒含量。结果显示大骨节病患儿尿氨基多糖总排泄量减少,硫酸化程度降低,表现为硫酸化二糖与非硫酸化二糖之比值和硫酸基含量低于对照组儿童;非患儿机体硒水平明显高于患儿;血、尿、发硒均与硫酸基呈显著的正相关。提示大骨节病患儿软骨的硫代谢障碍可能与低硒有关。

An experiment was carried out to analyze four disaccharides and three components of urinary glycosaminoglycan (GAG) as well as selenium (Se) contents of blood, urine, hair from the children with and without Kaschin-Beck disease (KBD) by the high-performance liquid chromatography and others. It was found that both excretive amount and degree of sulphation of GAG were decrease inKBD group——the ratio between sulphation and no-sulphation disaccharides and the content ofsulfato reduced; that the levels of Se in control group were obviously higher than that in KBD group; and that there was a positive correlation between the contents of Se and sulfato. These findings indicate that the impairment of sulphation in cartilage matrix of the children with KBD appears to be related to Se-deficiency.