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葡萄糖醛酸(简称糖醛酸)是除硫酸角质素外其他几种酸性粘多糖的组成成分。测定糖醛酸排出量可间接测出这些酸性粘多糖排出量。因此,尿及羊水糖醛酸定量可用于除Ⅳ型之外粘多糖病(MPS)的生后及产前诊断。对此,国内只有少数报告,有人用24小时尿测定,结果常受条件影响,差异较大。还有人用24小时尿测每排出1g肌酐糖醛酸的排出量。上述二法均需24小时尿,对小儿留取标本比较困难。我们用1次尿和24小时尿做了对比试验,二者结果基本一致,对正常小儿97例做了尿内糖醛酸含量测定,得出了不同年龄组的正常值。并对5例疑似MPS患儿进行了尿内糖醛酸排出量的测定,结果明显增高,经临床及X线检查,确诊粘多糖蓄积症。
Glucuronic acid (referred to as uronic acid) is in addition to keratan sulfate several other acidic mucopolysaccharide components. Determination of urinary excretion can be indirectly measured these acid mucopolysaccharide discharge. Therefore, urine and amniotic fluid uronic acid quantification can be used in post-natal and prenatal diagnosis of mucopolysaccharidosis (MPS) other than type IV. In this regard, only a few domestic reports, some people with 24-hour urine test, the results often affected by the conditions, the difference is larger. There are people with 24-hour urinary output per 1g creatinine excretion of uronic acid. The above two methods are required 24-hour urine samples taken more difficult for children. We use a urine and 24-hour urine contrast test done, the two results are basically the same, 97 cases of normal children urinary urinary acid content determination, obtained in different age groups of normal. Urine urinary excretion was measured in 5 cases of suspected MPS. The results were significantly higher, and confirmed by clinical and X-ray examination.