[The diagnosis and treatment of patients with acute intermittent porphyria].

Abstract

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.