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迟发性皮肤卟啉症两例在不同治疗阶段的卟啉尿排泄情况

Urinary excretion of porphyrins in two cases of porphyria cutanea tarda during a period of various treatments.

作者信息

Eriksen L, Thune P O

出版信息

Scand J Clin Lab Invest. 1978 May;38(3):273-7. doi: 10.1080/00365517809108423.

Abstract

The urinary excretion of porphyrins in two cases of porphyria cutanea tarda (PCT) has been followed during a period in which various treatments have been tried. In one patient pyridoxal-5-phosphate (P-5-P) injections lead to a dramatic increase in porphyrin excretion. In another P-5-P treatment was followed by a significant decrease in porphyrin excretion. p-Aminobenzoic acid (PABA) apparently had no effect on porphyrin excretion, while repeated phlebotomies had the expected effect. In both patients all variations in the total amount of porphyrins excreted were almost exclusively due to the variations in the amounts of heptacarboxylic porphyrin type III and uroporphyrin I. A secondary finding was an increase in hexa- and penta-carboxylic porphyrins type III without a concomitant increase in coproporphyrin III.

摘要

在对两例迟发性皮肤卟啉症(PCT)患者进行多种治疗的期间,对其卟啉的尿排泄情况进行了跟踪。在一名患者中,注射磷酸吡哆醛(P-5-P)导致卟啉排泄量急剧增加。在另一名患者中,P-5-P治疗后卟啉排泄量显著减少。对氨基苯甲酸(PABA)显然对卟啉排泄没有影响,而反复静脉放血则产生了预期效果。在两名患者中,排泄的卟啉总量的所有变化几乎完全归因于七羧基卟啉III型和尿卟啉I量的变化。一个次要发现是III型六羧基和五羧基卟啉增加,而粪卟啉III没有相应增加。

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