Ohba N, Nishibu M, Nagata M, Shibayama M, Tanishima K, Hashimoto T
Clinical Laboratory, Kanazawa University School of Medicine Hospital, Japan.
Rinsho Byori. 1996 Jan;44(1):76-80.
We presented two cases with transient hyperphosphatasemia (TH) of infancy, whose serum alkaline phosphatase (EC 3.1.3.1, ALP) activity showed markedly increased and the atypical isoenzyme fractions were seen by electrophoresis. These isoenzymes migrated into normal bone ALP region (alpha 2-beta globulin fraction) and fast liver ALP region (fast alpha 2 globulin fraction). From various investigation such as, heat stability, inhibition test by amino acid, neuraminidase treatment and Triton X-100 treatment, former fraction seemed to derive from bone ALP and later fraction from liver ALP. From our study, increment of the activity of alpha 2-beta gl fraction was in advance one month before maximum ALP activity stage, and fast alpha 2 gl fraction followed increasing 3 weeks after that. On the other hand, decreasing of fast alpha 2 gl fraction showed a shorter delay than alpha 2-beta gl fraction. These results suggest that a differential exchange of sugar chain or an impaired clearance of the enzyme from circulation was possibly occurred. It seemed to be important to increase a study of such a predictive and prognostic change of ALP activity and isoenzyme fraction in TH cases.
我们报告了两例婴儿期短暂性高磷酸酶血症（TH）病例，其血清碱性磷酸酶（EC 3.1.3.1，ALP）活性显著升高，且电泳可见非典型同工酶组分。这些同工酶迁移至正常骨ALP区域（α2-β球蛋白组分）和快速肝脏ALP区域（快速α2球蛋白组分）。通过热稳定性、氨基酸抑制试验、神经氨酸酶处理和Triton X-100处理等各种研究，前一组分似乎源自骨ALP，后一组分源自肝脏ALP。根据我们的研究，α2-β球蛋白组分活性的增加在ALP活性达到最大值阶段前一个月就已出现，而快速α2球蛋白组分在其之后3周开始增加。另一方面，快速α2球蛋白组分的下降比α2-β球蛋白组分的延迟更短。这些结果表明，可能发生了糖链的差异交换或酶从循环中的清除受损。增加对TH病例中ALP活性和同工酶组分这种预测性和预后性变化的研究似乎很重要。
