Protoporphyrin metabolic balance in human protoporphyria.

Protoporphyrin metabolic balance in human protoporphyria.
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人原卟啉症中的原卟啉代谢平衡。

DOI:
10.1016/s0016-5085(85)80085-8
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发表时间:
1985
期刊:
影响因子:
29.4
通讯作者:
Poh-Fitzpatrick,MB
Poh-Fitzpatrick,MB
中科院分区:
医学1区
文献类型:
--
作者:
Poh-Fitzpatrick,MB

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5例单纯性原卟啉症患者在5-7天内连续测定红细胞、血浆和粪便原卟啉水平,结果表明,短期维持不同的稳定稳态平衡,以促进原卟啉的产生、在红细胞和血浆中的蓄积以及粪便排泄。患者在研究单位住院,进行饮食控制和标本采集标准化。发现每个患者在这些隔室中定量地分配原卟啉,其模式每天都是可重复的,并且是该患者的特征。这些观察结果部分证实了先前提出的假设,即原卟啉分配模式可以为这些患者的前瞻性分类提供标准,这些患者最终可能被证明具有不同的症状性肝病发展风险。
Short-term maintenance of different stable steady state balances for protoporphyrin production, accumulation in erythrocytes and plasma, and fecal excretion was demonstrated in 5 patients with uncomplicated protoporphyria in whom red blood cell, plasma, and fecal protoporphyrin levels were determined on 5-7 sequential days. The patients were hospitalized in a research unit for dietary control and standardization of specimen collection. Each patient was found to quantitatively partition protoporphyrin among these compartments in a pattern that was reproducible from day to day, and characteristic for that patient. These observations partially substantiate a previously proposed hypothesis that protoporphyrin partitioning patterns may provide a criterion for prospective classification of these patients into groups that may ultimately be shown to have different risk for development of symptomatic liver disease.
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