Recovery from a severe attack of acute intermittent porphyria during coated-resin hemoperfusion.
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Biomedical subjects
Publications and source records attributed to M Jirsa.
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In three types of hepatic porphyrias, 32 hemoperfusions over polyhema-coated Amberlite XAD-2 resin were performed. An attack of acute intermittent porphyria subsided after a 6-h hemoperfusion. During the procedure, 13 L of plasma was completely cleared of porphyrins. In a child with variegate porphyria, the laboratory changes were slight and no clinical effect of hemoperfusion was seen. In a patient suffering from porphyria cutanea tarda resistant to standard therapy, 30 hemoperfusions were performed during a period of 10 months. The total plasma porphyrin concentration increased sharply following the start of the treatment, and preceding the fifth procedure it reached a peak of four times the initial value. Since then, the plasma porphyrin level decreased, eventually to one-half the starting value. The mean drop in porphyrin level during each perfusion was 22%. Similar changes were observed in the amount of porphyrins excreted into the urine, which, following an early rise, decreased to 25% of the initial value. However, the patient's skin vulnerability improved only slightly. There were no untoward effects of the treatment.
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A single two-compartment model suitable for studying the production and elimination of porphyrins from cells was prepared. Chloroquine with increasing concentrations:-- Inhibits the total production of porphyrins. Reduces intracellular concentration of porphyrins. Increases transversal permeation of porphyrins through the cellular membrane.
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