Bonafé J L, Salvayre R, Levade T, Nègre A, de Riols C, Charlet J P, Fernet P
Ann Dermatol Venereol. 1986;113(11):1059-63.
The activities of several lysosomal hydrolases (beta-galactosidase, beta-hexosaminidase, alpha-mannosidase, beta-glucuronidase and acid phosphatase) were determined in serum and blood lymphocytes from patients affected with scleroderma. Statistical comparisons between means of patient and control groups (Student's t test) showed a significant difference in serum beta-galactosidase and acid phosphatase between patients and controls (serum beta-galactosidase: 36.5 +/- 22 nmol/h/ml in the scleroderma group versus 24 +/- 13 nmol/h/ml in the control group; serum acid phosphatase 853 +/- 345 nmol/h/ml in the scleroderma group versus 634 +/- 295 nmol/h/ml in the control group). These differences were significant (p less than 0.01). Both enzyme activities were also significantly increased in the group with systemic scleroderma, but the difference was less in the group with localized scleroderma (this is discussed in terms of statistics and pathophysiology). The other enzyme activities determined were not significantly modified. The validity of these results is discussed, together with their diagnostic value and with the pathophysiological hypotheses put forward to explain the high levels found.
测定了硬皮病患者血清和血液淋巴细胞中几种溶酶体水解酶（β-半乳糖苷酶、β-己糖胺酶、α-甘露糖苷酶、β-葡萄糖醛酸酶和酸性磷酸酶）的活性。患者组与对照组均值之间的统计学比较（学生t检验）显示，患者与对照组血清β-半乳糖苷酶和酸性磷酸酶存在显著差异（血清β-半乳糖苷酶：硬皮病组为36.5±22 nmol/h/ml，对照组为24±13 nmol/h/ml；血清酸性磷酸酶：硬皮病组为853±345 nmol/h/ml，对照组为634±295 nmol/h/ml）。这些差异具有显著性（p<0.01）。系统性硬皮病组这两种酶的活性也显著升高，但局限性硬皮病组差异较小（从统计学和病理生理学角度进行了讨论）。所测定的其他酶活性未发生显著改变。讨论了这些结果的有效性，以及它们的诊断价值和为解释所发现的高水平提出的病理生理学假说。
