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卟啉病性神经病变:使用精氨酸血红素预防病情进展

Porphyric neuropathy: prevention of progression using haeme-arginate.

作者信息

Muthane U B, Vengamma B, Bharathi K C, Mamatha P

机构信息

Department of Neurology, St John's Medical College and Hospital, Bangalore, India.

出版信息

J Intern Med. 1993 Dec;234(6):611-3. doi: 10.1111/j.1365-2796.1993.tb01022.x.

DOI:10.1111/j.1365-2796.1993.tb01022.x
PMID:8258754
Abstract

We report on a patient with acute intermittent porphyria (AIP) who, during treatment with hypertonic glucose, developed peripheral neuropathy. Once haeme-arginate was started, the progression of the neuropathy was attenuated. This suggests that hypertonic glucose may be inadequate in preventing the development of neuropathy in a patient with porphyria. However, haeme-arginate, if started early, can attenuate the progression of porphyric neuropathy.

摘要

我们报告了一名患有急性间歇性卟啉病(AIP)的患者,该患者在接受高渗葡萄糖治疗期间出现了周围神经病变。一旦开始使用血红素精氨酸,神经病变的进展就会减缓。这表明高渗葡萄糖可能不足以预防卟啉病患者神经病变的发生。然而,血红素精氨酸如果早期开始使用,可以减缓卟啉性神经病变的进展。

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Therapy Follows Diagnosis: Old and New Approaches for the Treatment of Acute Porphyrias, What We Know and What We Should Know.治疗遵循诊断:急性卟啉病治疗的新旧方法,我们所知道的和我们应该知道的。
Diagnostics (Basel). 2022 Jul 3;12(7):1618. doi: 10.3390/diagnostics12071618.