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目的 :研究和比较先天性红细胞生成性卟啉病 (CEP)患者、迟发性皮肤卟啉病 (PCT)患者和正常健康者的红细胞酶活性。方法 :荧光分光光度法和反相高效液相色谱法。结果 :CEP患者的红细胞尿卟啉原Ⅲ同合成酶 (URO -COS)的活性仅为正常健康者的 17% ,而PCT患者的尿卟啉原脱羧酶 (UROD)活性仅为正常健康者的4 9%。结论 :URO -COS和UROD可能是引起CEP和PCT的主要原因
PURPOSE: To study and compare the erythrocytes activity of patients with congenital erythropoietic porphyria (CEP), patients with delayed-onset skin porphyria (PCT) and normal controls. Methods: Fluorescence spectrophotometry and reversed-phase high performance liquid chromatography. Results: The activity of erythrocyte uroporphyrinogen Ⅲ and synthase (URO -COS) in CEP patients was only 17% of that in normal healthy subjects, while the activity of uroporphyrinogen decarboxylase (UROD) in PCT patients was only normal and healthy 4 9%. Conclusion: URO-COS and UROD may be the main cause of CEP and PCT