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作 者:黄欣秋[1] 吴志奎[2] 王琳[1] 张新华[3] 柴立民[2] 杜华[1] 王荣新[3]
机构地区:[1]广西壮族自治区人民医院儿科,南宁530021 [2]中国中医研究院广安门医院分子生物研究室,北京100053 [3]中国人民解放军第303医院血液科,南宁530021
出 处:《实用儿科临床杂志》2007年第23期1808-1809,1827,共3页Journal of Applied Clinical Pediatrics
基 金:国家自然科学基金重大研究计划项目资助(90409003);广西应用基础研究基金项目资助(桂科基0385004);广西自然科学基金项目资助(桂科自0447104)
摘 要:目的探讨β-地中海贫血基因突变型与益髓生血颗粒疗效的相关性。方法收集β-地中海贫血47例。男28例,女19例;年龄3—15岁。其中重型β-地中海贫血34例,中间型13例。均检查血液学指标及行血红蛋白电泳,基因分析采用PCR反向斑点杂交技术,治疗采用益髓生血颗粒10g/次,3次/d,3个月为1个疗程,疗程结束进行疗效评价。结果47例β-地中海贫血中有7种β基因突变类型。其中复合血红蛋白E(HbE)占18例;中药治疗后43例临床症状明显改善,Hb、RBC、网织红细胞(Ret)及胎儿血红蛋白(HbF)值均增高,治疗前后比较差异显著(Pa〈0.01)。其中β一纯合子21例中17例有效,β°与β^+双重杂合子7例、β-地中海贫血与地中海贫血HbE杂合子18例、1例β^+纯合子均有效。结论益髓生血灵颗粒治疗β-地中海贫血有较好疗效。Objective To investigate the relationship between mutation types in β - thalassemia and therapeutic effect of yisuishengxue granules. Methods Forty - seven patients of β - thalassemia were from 3 to β years old. β - thalassemia major patients were 34 cases,β - thalassemia intermedia patients were 13 cases. All of 47 patients were analyzed by blood routine examination and hemoglobin electrophoresis. Genes were detected by PCR reverse dot blot. Forty - seven patients were administrated by yisuishengxue granules ( 10 g per time, thrice per day for 3 months), elvaluation of the result was carried out. Results Forty - seven patients of β - thalassemia had 7 gene mutation types and 18 patients of them complicated HbE. After treatment, there was significant improvement in clinical symptom, the number of Fib, RBC, reticulooyte ( Ret), fetal hemoglobin (HbF) ( Pa 〈 0.01 ). Twenty - one cases of β - thalassemia patients of β° homozygote, 17 cases were effective certainly. Seven cases of β° and β^ + double heterozygous and 18 cases of β - Thal/HbE were effective. One case of β - thalassemia patients with β^ + homozygote was response. Condusion Chinese traditonal medicine( Yisuishengxue granules) treating β - thalassemia is effective.
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