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消瘦症患者血清溶酶体水解酶的变化

Changes in serum lysosomal hydrolases in marasmus.

作者信息

Omene J A, Adamson I, Okolo A A, Glew R H

出版信息

Clin Chim Acta. 1979 Jan 15;91(2):213-9. doi: 10.1016/0009-8981(79)90459-5.

Abstract

The activities of several lysosomal hydrolases including beta-glucuronidase, acid phosphatase and hexosaminidase were compared in serum from 19 well-nourished subjects and 13 children (age 5--24 months) who were suffering from marasmus. The marasmic children exhibited growth retardation and muscle wastage but had normal serum protein values and absence of psychomotor retardation or oedema. Significant changes were observed in serum beta-glucuronidase and acid phosphatase activities. Compared to the control group, serum beta-glucuronidase (determined at pH 4.5 using the fluorogenic substrate, 4-methylumbelliferyl-beta-D-glucuronide) was 2.3-fold higher (p less than 0.001) in the marasmic children. In contrast, serum acid phosphatase values were approximately 50% lower (p less than 0.01) in the marasmic population. Serum hexosaminidase values in the two groups under study were not significantly different. Determination of the beta-glucuronidase to acid phosphatase ratio permitted effective discrimination (p less than 0.001) of serum from normal and protein-calorie malnourished children. The finding that the elevated value of the beta-glucuronidase : acid phosphatase ratio (0.64--1.37) decreased to within the normal range of values (0.10--0.43) after nutritional rehabilitation of several marasmic cases indicates that the determination of serum lysosomal hydrolases using fluorogenic substrates might provide a rapid and sensitive quantitative method for objectively evaluating the status of protein-calorie malnourished children and their responsiveness to nutritional therapy.

摘要

对19名营养状况良好的受试者以及13名患有消瘦症的儿童(年龄在5至24个月之间)的血清中的几种溶酶体水解酶的活性进行了比较,这些水解酶包括β-葡萄糖醛酸酶、酸性磷酸酶和己糖胺酶。患有消瘦症的儿童表现出生长发育迟缓以及肌肉萎缩,但血清蛋白值正常,且不存在精神运动发育迟缓或水肿。观察到血清β-葡萄糖醛酸酶和酸性磷酸酶的活性有显著变化。与对照组相比,患有消瘦症的儿童血清β-葡萄糖醛酸酶(使用荧光底物4-甲基伞形酮基-β-D-葡萄糖醛酸在pH 4.5条件下测定)高出2.3倍(p < 0.001)。相反,消瘦症人群的血清酸性磷酸酶值大约低50%(p < 0.01)。所研究的两组人群的血清己糖胺酶值没有显著差异。测定β-葡萄糖醛酸酶与酸性磷酸酶的比值能够有效区分正常儿童和蛋白质-热量营养不良儿童的血清(p < 0.001)。在对几例消瘦症病例进行营养康复后,β-葡萄糖醛酸酶:酸性磷酸酶比值的升高值(0.64 - 1.37)降至正常范围值(0.10 - 0.43)内,这一发现表明使用荧光底物测定血清溶酶体水解酶可能为客观评估蛋白质-热量营养不良儿童的状况及其对营养治疗的反应提供一种快速且灵敏的定量方法。

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