| 试用马利兰治疗β—地中海贫血 |
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| 引用本文： | 梁植权,刘利娟.试用马利兰治疗β—地中海贫血[J].中国医学科学院学报,1991,13(2):133-136. |
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| 作者姓名： | 梁植权 刘利娟 |
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| 作者单位： | 中国医学科学院基础医学研究所 北京
(梁植权，杨克恭，陈松森，敖朝晖，刘德培，贾佩臣)，中国人民解放军第303医院 南宁
(刘利娟，王荣新，查丹玉，金和谦)，中国人民解放军第303医院 南宁(黄有文) |
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| 摘 要： | 对3例重型β-地中海贫血(β-地贫)患儿试用马利兰治疗。患儿每日口服马利兰(0.2mg/kg),20d后,Hb,HbF,RBC等主要血液学指数均有所改善。临床症状缓解,肝脾肿大减轻。2例患儿姐妹染色体互换(SCE)有轻度增加,停药后即可恢复,1例基本无变化,提示所用剂量的马利兰对患者染色体DNA无明显损伤。用多聚酶链反应(PCR)和寡聚核苷酸探针杂交测定13例经马利兰治疗的重型β-地贫患儿的DNA,在22条β-地贫染色体中发现有7种β-珠蛋白基因突变类型,提示马利兰对不同突变类型β-地贫均有疗效。
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| 关 键 词： | 马利兰 地中海贫血 |
Further study on the treatment of severe beta-thalassemia with myleran] |
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| Liang Zhiquan,et al.Further study on the treatment of severe beta-thalassemia with myleran][J].Acta Academiae Medicinae Sinicae,1991,13(2):133-136. |
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| Authors: | Liang Zhiquan |
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| Institution: | Institute of Basic Medical Sciences, Beijing. |
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| Abstract: | In a previous paper we reported the results of treatment of five patients with beta-thalassemia major with myleran. Three more cases are reported in this paper. After 40-50 days of oral administration of myleran, major hematological findings (Hb, HbF, RBC) began to increase and remained at higher level for 4-5 months before dropping back to the original. The results of analysis of sister chromatid exchange (SCE) showed no irreversible damage on chromosomal DNA of the patients at the dosage used (0.2 mg/kg.d). It was also demonstrated that the effect of treatment does not seem to be related to the types of beta-globin gene mutations of the patients. |
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