Resin hemoperfusion in hepatic porphyrias.
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.
D000328:Adult; D002648:Child; D005260:Female; D006464:Hemoperfusion / Q000379:methods*; D006801:Humans; D007475:Ion Exchange Resins / Q000627:therapeutic use*; D008107:Liver Diseases / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D011137:Polystyrenes / Q000627:therapeutic use*; D011164:Porphyrias / Q000628:therapy*
D007475:Ion Exchange Resins; D011137:Polystyrenes; C003771:Amberlite XAD-2 resin