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对患有严重非酮症高甘氨酸血症的新生儿双胞胎尝试进行士的宁治疗。

Strychnine treatment attempted in newborn twins with severe nonketotic hyperglycinemia.

作者信息

Steinmann B, Gitzelmann R

出版信息

Helv Paediatr Acta. 1979;34(6):589-99.

PMID:541222
Abstract

Strychnine, a potent antagonist of glycine was given to twins suffering from nonketotic hyperglycinemia at age 73 hours. Within hours of the onset of treatment favorable effects were observed such as improvements of muscle tone, movements, defense reactions, and probably breathing. Pyridoxine, N5-formyl-tetrahydrofolate and lipoic acid were given concomitantly with strychnine but failed to alter glycine levels in plasma and cerebrospinal fluid. The therapeutic trial was terminated after 2 1/2 days because success, though considerable, was judged inadequate.

摘要

士的宁是甘氨酸的强效拮抗剂,在一对患有非酮症高甘氨酸血症的双胞胎73小时大时给予了该药物。在开始治疗后的数小时内,观察到了有益效果,如肌张力、运动、防御反应以及可能的呼吸功能均有所改善。在给予士的宁的同时还给予了吡哆醇、N5-甲酰四氢叶酸和硫辛酸,但未能改变血浆和脑脊液中的甘氨酸水平。2.5天后该治疗试验终止,因为尽管取得了相当大的成功,但仍被判定效果欠佳。

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