SEROTONIN-GABA TREATMENT IS HYPOTHESIZED FOR SELF-INJURY IN LESCH-NYHAN SYNDROME
SEROTONIN-GABA TREATMENT IS HYPOTHESIZED FOR SELF-INJURY IN LESCH-NYHAN SYNDROME
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DOI：
10.1016/0306-9877(92)90026-9
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发表时间：
1992-08-01
影响因子：
4.7
通讯作者：
GEDYE, A
中科院分区：
文献类型：
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作者：
GEDYE, A
Lesch-Nyhan患者的自我伤害运动被认为是非常严重的，目前难以治愈。Lesch-Nyhan综合征是一种嘌呤代谢的遗传性疾病，导致几种异常，其中之一是黄嘌呤水平升高。作者的理由是，血浆黄嘌呤升高引发了一系列事件，产生过多的内源性惊厥药和减少内源性抗惊厥药。建议治疗Lesch-Nyhan的自伤，需要减少两种内源性惊厥药（犬尿氨酸和喹啉酸）的产生，并增强两种内源性抗惊厥药（5-羟色胺和γ-氨基丁酸）。
The self-injurious movements of Lesch-Nyhan patients are considered extremely severe and currently intractable. Lesch-Nyhan syndrome is a genetic disorder of purine metabolism resulting in several abnormalities, one of which is elevated levels of xanthine. The author reasons that elevated plasma xanthine sets off a chain of events that produce excessive endogenous convulsants and diminished endogenous anticonvulsants. Treatment is proposed for self-injury in Lesch-Nyhan that entails reducing production of two endogenous convulsants (kynurenine and quinolinic acid) and enhancing two endogenous anticonvulsants (serotonin and gamma aminobutyric acid).