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先天性红细胞生成性卟啉病经超活性炭治疗后化学病理学的快速改善

Rapid improvement in the chemical pathology of congenital erythropoietic porphyria with treatment with superactivated charcoal.

作者信息

Tishler P V, Winston S H

机构信息

Brockton/West Roxbury Veterans Administration Medical Center, MA.

出版信息

Methods Find Exp Clin Pharmacol. 1990 Nov;12(9):645-8.

PMID:2084460
Abstract

Non-absorbable sorbents that bind porphyrins in the gastrointestinal tract may be useful in the treatment of porphyrias whose manifestations result from porphyrin excess. To test this, we assessed the effect of oral charcoal on porphyrin economy in a patient with a probable congenital erythropoietic porphyria. Treatment with a superactivated charcoal (Super Char), 25 g three times daily, was associated with a precipitous drop in erythrocyte porphyrin (from 21.4 +/- 2.9 [SD] to 7.4 +/- 0.4 nmole/ml; p less than 0.025) and plasma porphyrin (from 1.56 +/- 0.24 to 0.70 +/- 0.08 nmole/ml; p less than 0.01). Urinary porphyrin excretion appeared to rise, from 103 +/- 45 to 160 +/- 30 mumole/d, but the change was not statistically significant. Constipation appeared to limit compliance with the charcoal regimen by the end of the study period. Nonetheless, superactivated charcoal may be a useful therapy in this disfiguring porphyria.

摘要

在胃肠道中结合卟啉的不可吸收吸附剂可能有助于治疗因卟啉过量而导致症状的卟啉病。为了验证这一点,我们评估了口服活性炭对一名疑似先天性红细胞生成性卟啉病患者卟啉代谢的影响。使用超级活性炭(Super Char)进行治疗,每日3次,每次25克,结果红细胞卟啉急剧下降(从21.4±2.9[标准差]降至7.4±0.4纳摩尔/毫升;p<0.025),血浆卟啉也急剧下降(从1.56±0.24降至0.70±0.08纳摩尔/毫升;p<0.01)。尿卟啉排泄量似乎有所上升,从103±45升至160±30微摩尔/天,但变化无统计学意义。到研究期末,便秘似乎限制了患者对活性炭治疗方案的依从性。尽管如此,超级活性炭可能是治疗这种毁容性卟啉病的一种有效疗法。

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Methods Find Exp Clin Pharmacol. 1990 Nov;12(9):645-8.
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引用本文的文献

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Congenital erythropoietic porphyria: Recent advances.先天性红细胞生成性卟啉病:最新进展。
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Inducing iron deficiency improves erythropoiesis and photosensitivity in congenital erythropoietic porphyria.诱导缺铁可改善先天性红细胞生成性卟啉病的红细胞生成和光敏性。
Blood. 2015 Jul 9;126(2):257-61. doi: 10.1182/blood-2014-07-584664. Epub 2015 May 13.