Dhar G J, Bossenmaier I, Petryka Z J, Cardinal R, Watson C J
Ann Intern Med. 1975 Jul;83(1):20-30. doi: 10.7326/0003-4819-83-1-20.
The present study was carried out in five cases of hepatic porphyria, including three of acute intermittent porphyria, one of variegate porphyria, and one of porphyria cutanea tarda in clinical remission. In two cases of acute intermittent porphyria (in relapse), a marked lowering effect on serum and urine porphobilinogen and delta-aminolevulinic acic was observed, together with prompt and gratifying clinical improvement. In a third case, in chemical remission but with longstanding psychoneurosis, no significant effects were noted, nor were any observed in the case of porphyria cutanea tarda. Although clinical improvements occurred in the case of variegate porphyria, the results were inconclusive for reasons given. Hematin was generally well tolerated. Preliminary reference is made to a transitory renal injury, without sequelae, where an excess of hematin was given in relation to time. Limits of tolerance are proposed. In the light of these observations the basic mechanism of the acute attack is diccussed.
本研究对5例肝性卟啉病患者进行，其中包括3例急性间歇性卟啉病、1例迟发性皮肤卟啉病（临床缓解期）和1例杂合性卟啉病。在2例急性间歇性卟啉病（复发期）患者中，观察到血清和尿中卟胆原及δ-氨基乙酰丙酸有显著降低作用，同时临床症状迅速且显著改善。第3例处于化学缓解期但有长期精神神经症的患者，未观察到显著效果，迟发性皮肤卟啉病患者也未观察到任何效果。虽然杂合性卟啉病患者出现了临床改善，但由于所述原因，结果尚无定论。血红素总体耐受性良好。初步提及了在给予过量血红素时出现的短暂性肾损伤，但无后遗症，并提出了耐受限度。根据这些观察结果，对急性发作的基本机制进行了讨论。
