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[卟啉性神经病的血红素治疗]

[Hematin treatment of porphyric neuropathy].

作者信息

Navarro F, Herrerías J M, Pérez Cano R, Araquistain J M, Garrido Peralta M

出版信息

Rev Iber Endocrinol. 1976 Nov-Dec;23(138):571-82.

PMID:1013578
Abstract

The authors report a case of acute intermittent porphiria with peripheral motor neuropathy and a syndrome of inadequate ADH excretion in a male patient aged 36 years. Treatment with hypertonic glucose and prostigmine did not yield any beneficial results, while a subsequent treatment with hematine (4 mg/kg/day for a period of four days) produced a dramatic improvement in the clinical picture and in ALA and PGB levels. A back-to-normal shift of plasma and urinary electrolytes was also observed. It appears that there was a close relation between the administration of hematine and the clinical-biochemical remission.

摘要

作者报告了一例36岁男性急性间歇性卟啉病患者,伴有周围运动神经病和抗利尿激素分泌不足综合征。高渗葡萄糖和新斯的明治疗未取得任何有益效果,而随后使用血红素(4mg/kg/天,共4天)治疗后,临床症状以及δ-氨基-γ-酮戊酸(ALA)和尿卟啉原(PGB)水平均有显著改善。还观察到血浆和尿液电解质恢复至正常水平。血红素给药与临床生化缓解之间似乎存在密切关系。

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