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迟发性皮肤卟啉病患者治疗及长期随访期间血浆尿卟啉的测定

Measurement of plasma uroporphyrin during treatment and long-term follow-up of patients with Porphyria cutanea tarda.

作者信息

Adjarov D G, Kerimova M A

机构信息

Institute of Gastroenterology and Nutrition, Medical Academy, Sofia, Bulgaria.

出版信息

Clin Chim Acta. 1989 May 15;181(2):143-50. doi: 10.1016/0009-8981(89)90181-2.

DOI:10.1016/0009-8981(89)90181-2
PMID:2736777
Abstract

.1 ml plasma was precipitated and extracted with 3 ml 0.61 mol/l trichloroacetic acid. After centrifugation the fluorescence intensity of the supernatant was measured using uroporphyrin as a standard. The porphyrins with a higher number of carboxylic groups (uroporphyrin and heptacarboxyporphyrin) were effectively extracted and the others, poorly or very poorly. Thus, the determined parameter was a mixture of chiefly highly carboxylated porphyrins. The results were expressed in nmol uroporphyrin/l. The coefficient of variation was 4%. No quenching of the fluorescence was established in higher plasma porphyrin concentrations. The range of the control group (n = 33) was 0-1.4 nmol/l. The data of the patients, needing treatment were within the interval of 17.9-448 nmol/l (n = 17). The correlation coefficient with the urinary uroporphyrin was 0.97. The values in the cases with remission (n = 74) ranged from 0 to 12.8 nmol/l. The method is particularly convenient for monitoring the effect of treatment in patients with Porphyria cutanea tarda, as well as for their long-term follow-up.

摘要

取0.1 ml血浆,用3 ml 0.61 mol/l三氯乙酸沉淀并萃取。离心后,以上尿卟啉为标准测定上清液的荧光强度。羧基数目较多的卟啉(尿卟啉和七羧基卟啉)能被有效萃取,而其他卟啉的萃取效果较差或极差。因此,所测定的参数主要是高度羧化卟啉的混合物。结果以每升纳摩尔尿卟啉表示。变异系数为4%。在较高的血浆卟啉浓度下未发现荧光猝灭现象。对照组(n = 33)的范围为0 - 1.4 nmol/l。需要治疗的患者的数据在17.9 - 448 nmol/l范围内(n = 17)。与尿卟啉的相关系数为0.97。缓解期患者(n = 74)的值在0至12.8 nmol/l之间。该方法对于监测迟发性皮肤卟啉症患者的治疗效果以及长期随访特别方便。

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