Oral Administration of the Thyrotropin-Releasing Hormone (TRH) Analogue, Taltireline Hydrate, in Spinal Muscular Atrophy
Oral Administration of the Thyrotropin-Releasing Hormone (TRH) Analogue, Taltireline Hydrate, in Spinal Muscular Atrophy
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DOI：
10.1177/0883073809333535
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发表时间：
2009-08
影响因子：
1.9
通讯作者：
Zenichiro Kato;M. Okuda;Y. Okumura;T. Arai;Takahide Teramoto;M. Nishimura;H. Kaneko;N. Kondo
中科院分区：
文献类型：
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作者：
Zenichiro Kato;M. Okuda;Y. Okumura;T. Arai;Takahide Teramoto;M. Nishimura;H. Kaneko;N. Kondo
脊髓性肌萎缩症（Spinal muscular atrophy，SMA）是由运动神经元端粒生存基因异常引起的脊髓前角神经元退行性疾病。脊髓性肌萎缩症尚无明确的治疗方法，但最近的报道表明静脉注射促甲状腺激素释放激素（TRH）而非口服给药的疗效。我们通过口服促甲状腺激素释放激素类似物，他替林水合物治疗了一名患有脊髓性肌萎缩III型的18岁男性患者。他的肌肉力量在治疗后显著增加，并且他没有表现出临床或实验室可识别的不良反应，包括在静脉内促甲状腺激素释放激素治疗中观察到的促甲状腺激素抑制。口服这种促甲状腺激素释放激素类似物应注意作为一种有前途的治疗脊髓性肌萎缩症。
Spinal muscular atrophy is an entity of neurodegenerative disorders at the anterior horn neuron of the spinal cord caused by telomeric survival motor neuron gene abnormality. There is no definitive treatment for spinal muscular atrophy, but recent reports have indicated the efficacy of intravenous injection, but not oral administration, of thyrotropin-releasing hormone (TRH). We treated an 18-year-old male patient with spinal muscular atrophy type III by oral administration of the thyrotropin-releasing hormone analogue, taltireline hydrate. His muscle strength increased significantly after the therapy, and he showed no clinical or laboratory identifiable adverse effects, including thyroid-stimulating hormone suppression that had been observed with intravenous thyrotropin-releasing hormone therapy. Oral administration of this thyrotropin-releasing hormone analogue should be noted as a promising therapy for spinal muscular atrophy.