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本文通过测定迟发性皮肤卟啉病(PCT)血清总卟啉浓度来监测其生化过程。作者从18例PCT患者在确诊及治疗期或缓解期测得的血清总卟啉及24小时尿标本中尿卧啉和粪卟啉值,发现血清总卟啉和24小时尿中卟啉排出量呈明显线性相关。一例经二个月的治疗后,其血与尿卟啉水平下降一致,另一例4年中未予任何治疗,其血尿卟啉水平也相平行。 PCT患者由于尿卟啉原脱羧酶的缺陷,肝脏过度产生贮存以尿卟啉(8羧基卟啉),7羧基卟啉和异构粪卧啉为主的卟啉。这些卟啉进入血循环,并输送至皮肤及排出部位。尿卟啉从尿中排出,卟啉原经脱羧后再氧化成为粪卟啉(4羧基卟啉)主要从粪中排出。PCT中的血卟啉主要是8和7羧基卟啉,从这样的患者测24小时尿中尿卟啉与粪卟啉之比通
In this paper, the biochemical process was monitored by measuring the total serum concentration of porphyrin in patients with tardive porphyria (PCT). The author from 18 cases of PCT patients in the diagnosis and treatment or remission of serum total porphyrin and 24-hour urine samples urinary and fecal porphyrin value, found that total serum porphyrin and 24-hour urinary porphyrin emission Obviously linear correlation. One case after two months of treatment, the blood and uroporphyrin levels decreased consistent with another 4 years without any treatment, the hematuria and porphyrin levels are parallel. Due to the defect of uroporphyrinogen decarboxylase in PCT patients, the liver is excessively stored with uroporphyrin (8-carboxy-porphyrin), 7-carboxy-porphyrin and isoprenoid-dominated porphyrin. These porphyrins enter the bloodstream and are delivered to the skin and to the site of excretion. Urine porphyrin excreted from the urine, porphyrin after decarboxylation and then oxidized to coproporphyrin (4 carboxyl porphyrin) is mainly excreted from the feces. Hematoporphyrin in PCT is mainly 8 and 7 carboxyl porphyrin, from such patients measured 24-hour urine uroporphyrin and coproporphyrin ratio