Hepatic protoporphyrin production in human protoporphyria: Effects of intravenous hematin and analysis of erythrocyte protoporphyrin distribution
Hepatic protoporphyrin production in human protoporphyria: Effects of intravenous hematin and analysis of erythrocyte protoporphyrin distribution
复制标题
人原卟啉症中肝脏原卟啉的产生：静脉注射血红素的影响和红细胞原卟啉分布分析
DOI：
10.1016/0016-5085(80)90084-0
复制
发表时间：
1980
期刊：
影响因子：
29.4
通讯作者：
A. Lamola
中科院分区：
文献类型：
--
作者：
J. Lamon;M. Poh;A. Lamola
在2例患者中研究了具有非典型特征的人类原卟啉症，提示肝原卟啉合成增加。分析循环中的红细胞的年龄增加的原卟啉的分布表明，红细胞卟啉负担主要来自红细胞来源的情况下1，从肝来源的情况下2。两名患者均接受静脉注射血红素，以评估对原卟啉合成的任何负反馈效应。红细胞，粪便和血浆卟啉水平连续测量期间的基础，治疗和随访期。在病例1中，粪便和血浆中的血红素水平均显著（P< 0.001）下降，而红细胞水平保持不变。血红素产生的卟啉浓度没有明显的变化，在案件2。两例患者均在使用血红素后发生过敏性血管炎。基于本研究的数据和以前的数据，原卟啉运输和清除模型的开发，其中变量的临床和生化表现的人类原卟啉症有关的红细胞和肝脏来源的异常原卟啉池的相对贡献。
Human protoporphyria with atypical features suggesting increased hepatic protoporphyrin synthesis was investigated in 2 patients. Analysis of the distribution of protoporphyrin among circulating erythrocytes of increasing age indicated that the erythrocyte porphyrin burden derived predominantly from erythroid sources in case 1, and from hepatic sources in case 2. Intravenous hematin was administered to both patients to assess any negative feedback effect on protoporphyrin synthesis. Erythrocyte, fecal and plasma porphyrin levels were measured serially during basal, treatment, and follow-up periods. In case 1, a significant (P< 0.001) drop in both fecal and plasma levels accompanied hematin, while erythrocyte levels remained unchanged. Hematin produced no appreciable changes in porphyrin concentrations in case 2. Allergic vasculitis followed hematin use in both cases. Based on data of this study and on previous data, a model for protoporphyrin transport and clearance was developed in which the variable clinical and biochemical manifestations of human protoporphyria are related to the relative contributions of erythroid and hepatic sources to the abnormal protoporphyrin pools.