Ascorbate status of patients with porphyria cutanea tarda symptomatica and its effect on porphyrin metabolism.

V A Percy, D Naidoo, S M Joubert, R J Pegoraro
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Abstract

The ascorbate status and the effect of loading doses of ascorbic acid (1,5 g per day by mouth for 7 days) on the porphyric process were studied in 7 black men with porphyria cutanea tarda symptomatica. It was found that the ascorbate stores were depleted in these patients as judged by serum and leucocyte ascorbate levels. Temporary repletion of the ascorbate stores was effected by the loading doses of ascorbic acid: Serum iron concentrations increased in 6 patients; urinary iron excretion showed small peaks a few days after ascorbic acid therapy commenced; haemoglobin concentration was not affected; excessive amounts of oxalate were excreted in the urine; neither total urinary porphyrin excretion nor the composition of the urinary porphyrins was affected in a way which could be related to ascorbate therapy. Further evidence that ascorbate depletion is not important in the induction of porphyria was found when the ascorbate status of siderotic rats, rendered porphyric by hexachlorobenzene-feeding, was examined. Stores of ascorbate and the oxidative catabolism of ascorbate were not different in the prophyric rats as compared with normal litter mates.

迟发性皮肤卟啉症患者抗坏血酸状况及其对卟啉代谢的影响。
研究了7例黑人迟发性皮肤卟啉症患者的抗坏血酸状态和抗坏血酸负荷剂量(1.5 g /天,口服7天)对卟啉过程的影响。结果发现,根据血清和白细胞抗坏血酸水平判断,这些患者的抗坏血酸储备被耗尽。抗坏血酸负荷剂量影响抗坏血酸储存的暂时补充:6例患者血清铁浓度升高;尿铁排泄在抗坏血酸治疗开始后几天出现小高峰;血红蛋白浓度未受影响;过量的草酸从尿液中排出;尿卟啉总量和尿卟啉的组成都没有受到与抗坏血酸治疗有关的影响。进一步的证据表明,抗坏血酸消耗在诱导卟啉症中并不重要,当检查六氯苯喂养的鼠的抗坏血酸状态时,发现卟啉症。预防组大鼠抗坏血酸的储存量和抗坏血酸的氧化分解代谢与正常仔鼠相比无显著差异。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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