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迟发性皮肤卟啉病症状性患者的抗坏血酸状态及其对卟啉代谢的影响。

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

作者信息

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

出版信息

S Afr J Med Sci. 1975;40(4):185-96.

PMID:1209393
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.

摘要

对7名患有迟发性皮肤卟啉症的黑人男性患者的抗坏血酸状态以及大剂量抗坏血酸(每天口服1.5克,持续7天)对卟啉代谢过程的影响进行了研究。通过血清和白细胞中的抗坏血酸水平判断,发现这些患者的抗坏血酸储备已耗尽。大剂量抗坏血酸使抗坏血酸储备得到暂时补充:6名患者的血清铁浓度升高;抗坏血酸治疗开始几天后,尿铁排泄出现小高峰;血红蛋白浓度未受影响;尿中排出过量草酸盐;尿卟啉总排泄量和尿卟啉组成均未受到与抗坏血酸治疗相关的影响。在检查通过喂食六氯苯而患卟啉症的铁沉着病大鼠的抗坏血酸状态时,发现了进一步的证据,表明抗坏血酸缺乏在卟啉症的诱发中并不重要。与正常同窝仔鼠相比,患卟啉症大鼠的抗坏血酸储备及其氧化分解代谢并无差异。

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