【急性间歇性卟啉症的诊断与治疗】。

Vrachebnoe delo Pub Date : 1991-12-01
E A Zakhariia, Ia N Romanishin, N A Zastavnaia, I V Temnik, S A Antonenko, M V Kinakh, V V Grigorenko
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引用次数: 0

摘要

对4例急性间歇性卟啉症(AIP)患者进行治疗和随访。结果表明,AIP尿色素在可见光范围内具有特定的吸收光谱。临床表现和病程严重程度与共卟啉和尿卟啉的排泄量及其特征光谱吸收有关。因此,在AIP发病机制中有重要意义的是共卟啉和尿卟啉异构体的形成水平,它们可能直接影响AIP的神经组织和临床表现。建议采用体外供体脾血浆置换法进行生物血液吸收。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
[The diagnosis and treatment of patients with acute intermittent porphyria].

Four patients with acute intermittent porphyria (AIP) were treated and followed-up. It was established that in AIP urinary pigments have specific absorption spectra in the visible light range. Clinical manifestation and the severity of the disease course correlated with the amount of excretion of copro- and uroporphyrin and their characteristic spectrum of light absorption. It is concluded that of significance in the AIP pathogenesis is the level of formation of copro- and uroporphyrin isomers which may directly effect the nervous tissue and clinical manifestations of AIP. It is recommended to use plasmapheresis in biohemosorption by means of extracorporeal donor spleen.

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