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肿瘤伴发高雪氏病较为罕见,至今仅3例报道。本文报告的为第4例,该患者按何杰金氏病治疗了6年并经酶测定证实为高雪氏病。患者的葡萄糖脑甙脂酶的测定为3.6毫克分子4-Methylumbelliferyl/mg蛋白/分,其母测定值为24.3毫克分子4-Methylumbe-lliferyl/mg蛋白/分(高雪氏病此酶活力的变动范围为2.9～7.9毫克分子;正常值为35～45毫克分子)。患者双亲可有此酶的部份缺乏,其酶活力界于正常值
The incidence of tumors associated with high incidence of Shereb disease is relatively rare and only 3 cases have been reported so far. In this article, the fourth case was reported. The patient was treated with Hodgkin's disease for 6 years and confirmed by enzyme assay as Goethia disease. The patient's glucose cerebrospinal lipase assay was 3.6 milligrams 4-Methylumbelliferyl/mg protein/minute, and the parental determination value was 24.3 milligrams 4-Methylumbe-lliferyl/mg protein/minute (Changes in the activity of this enzyme in Goethia disease. The range is 2.9 to 7.9 millimoles; normal is 35 to 45 millimolar). The patient's parents may have a deficiency in this enzyme, and their enzyme activity is within normal limits.