Erythrocyte uroporphyrinogen decarboxylase activity in 80 unrelated patients with porphyria cutanea tarda
Erythrocyte uroporphyrinogen decarboxylase activity in 80 unrelated patients with porphyria cutanea tarda
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80例无关迟发性皮肤卟啉症患者红细胞尿卟啉原脱羧酶活性
DOI：
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发表时间：
1992
影响因子：
10.3
通讯作者：
N. Simon
中科院分区：
文献类型：
--
作者：
F. Kószó;M. Morvay;Attila Dobozy;N. Simon
为了估计迟发性皮肤卟啉症（PCT）亚组的患病率，使用五羧基卟啉原III作为底物，测量了80名不相关的PCT患者及其45名亲属的红细胞尿卟啉原脱羧酶（UD）活性。通过对患者及其亲属119例的尿卟啉分析，对亚组进行了区分。在这些患者中，发现77.5%患有散发型PCT（I型PCT），22.5%患有家族型PCT（II型PCT），每个PCT患者以前都受到酒精，雌激素或其他肝损伤因素的影响。女性（9/15）中家族性PCT的相对频率高于男性（9/65），这表明II型PCT基因的遗传可能易患雌激素沉淀的PCT。II型PCT的发病年龄低于I型（42.6岁vs. 47.0岁）。研究结果表明，遗传性UD缺乏症携带者的促发因素风险增加。
To estimate the prevalence of the subgroups of porphyria cutanea tarda (PCT), erythrocyte uroporphyrinogen decarboxylase (UD) activity was measured in 80 unrelated patients with PCT, and in 45 of their relatives by using pentacarboxyl‐porphyrinogen III as substrate. The subgroups were differentiated by analysis of the urinary porphyrins of the patients and 119 of their relatives. Of the patients, 77·5% were found to be suffering from the sporadic form of PCT (type I PCT), and 22·5% from the familial form (type II PCT), Every patient with PCT had previously been affected by alcohol, oestrogen or some other liver‐damaging factor. The relative frequency of familial PCT was higher in females (nine of 15) than in males (nine of 65), which suggests that inheritance of the gene for type II PCT may predispose to oestrogen‐precipitated PCT. The onset of type II PCT occurred at a lower age than that of type I (42·6 vs. 47·0 years). The findings suggest an increased risk of precipitating factors in carriers of an inherited UD deficiency.
迟发性皮肤卟啉症的红细胞尿卟啉原脱羧酶活性：对 40 名连续患者的研究。
DOI：
--
发表时间：
1989
期刊：
The Journal of investigative dermatology
影响因子：
--
作者：
Held,JL;Sassa,S;Kappas,A;Harber,LC
通讯作者：
Harber,LC