本研究首次将肽链合成抑制剂鼻烟肼［（２０～３０ｍｇ／（ｋｇ·ｄ），７～１４日］试用于６例β地中海贫血（β地贫）患儿，显示了较好的疗效。治疗后患儿血红蛋白、红细胞计数、网织红细胞相对计数、红细胞压积比均显著增高，肝脾缩小，临床症状改善。体外肽链合成结果显示患儿α肽链合成明显抑制，β／α肽链合成速率比率升高，提示异烟肼的治疗作用是通过抑制α链合成实现的。研究结果表明异烟肼治疗β地贫是副作用小而有效的方法。 This study, for the first time, tested the efficacy of the peptide synthesis inhibitor, nosigenin (20-30 mg / (kg · d), day 7-14) in 6 children with β-thalassemia (β thalassemia). After treatment, the hemoglobin, erythrocyte count, reticulocyte count, hematocrit ratio were significantly increased, liver and spleen decreased, the clinical symptoms were improved.Extracellular peptide synthesis in children showed that α-peptide synthesis was significantly inhibited, β / α peptide The rate of chain synthesis increased, suggesting that the therapeutic effect of isoniazid is achieved through inhibition of α-chain synthesis. The results show that isoniazid treatment of β thalassemia is a side effect of small and effective method.