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[台湾地区轻型地中海贫血或血红蛋白H病患儿的血液学数据分析]

[Hematological data analysis in children with thalassemia trait or hemoglobin H disease in Taiwan].

作者信息

Yang C P, Hung I J

机构信息

Department of Pediatrics, Chang-Gung Memorial Hospital, Taipei, Taiwan, R.O.C.

出版信息

J Formos Med Assoc. 1991 Jun;90(6):591-7, 586.

PMID:1681007
Abstract

In the past several years, we have collected 91 cases diagnosed as beta-thalassemia trait, 94 cases as alpha-thalassemia trait and 43 cases as hemoglobin H disease. The ages ranged from 6 months to 15 years. The hematological data were analyzed. We calculated the mean +/- SD for each item of the erythrocyte indices (RBC, Hgb, Hct, MCV, MCH, MCHC, +/- RDW) and the Hb electrophoretic results (Hb A, Hb A2, Hb F, Hb H & Bart's) in each group, and tabulated them. Then, comparisons were made between beta and alpha-thalassemia traits or between the thalassemia trait and hemoglobin H disease. We found that each of the mean values for Hgb, Hct, MCV and MCH for children with the beta-thalassemia trait was significantly lower than that for children with the alpha-thalassemia trait. The mean MCV value of the beta-thalassemia trait group was 60.7 +/- 3.4 fl, and 87% of the MCV values in this group ranged from 55 to 65 fl. The mean MCV value for the alpha-thalassemia trait group was 63.3 +/- 3.3 fl, and 56% of the MCV values in this group ranged from 60-65 fl. The MCV values for all of the 185 children studied with either the beta- or alpha-thalassemia trait ranged from 54 to 71 fl, and were far below the normal ranges for MCV values for each age-specific group. The mean percentage of deviation of MCV from normal was greater than that of Hgb in both the beta- and alpha-thalassemia trait groups. A comparison of those with beta-thalassemia trait 6 years old and under and those over 6 years showed that both the mean values of RBC and Hgb were higher in the older subgroup, while the mean MCV values did not show any significant differences between these 2 subgroups.(ABSTRACT TRUNCATED AT 250 WORDS)

摘要

在过去几年中,我们收集了91例被诊断为β地中海贫血特征的病例、94例α地中海贫血特征的病例以及43例血红蛋白H病的病例。年龄范围为6个月至15岁。对血液学数据进行了分析。我们计算了每组红细胞指数(RBC、Hgb、Hct、MCV、MCH、MCHC、±RDW)各项以及Hb电泳结果(Hb A、Hb A2、Hb F、Hb H和Bart's)的均值±标准差,并制成表格。然后,对β地中海贫血特征与α地中海贫血特征之间或地中海贫血特征与血红蛋白H病之间进行了比较。我们发现,β地中海贫血特征患儿的Hgb、Hct、MCV和MCH的均值均显著低于α地中海贫血特征患儿。β地中海贫血特征组的平均MCV值为60.7±3.4 fl,该组87%的MCV值范围为55至65 fl。α地中海贫血特征组的平均MCV值为63.3±3.3 fl,该组56%的MCV值范围为60 - 65 fl。研究的185例β或α地中海贫血特征患儿的MCV值范围为54至71 fl,远低于各年龄组MCV值正常范围。β和α地中海贫血特征组中,MCV偏离正常的平均百分比均大于Hgb。对6岁及以下和6岁以上的β地中海贫血特征患儿进行比较发现,年龄较大亚组的RBC和Hgb均值较高,而这两个亚组的平均MCV值无显著差异。(摘要截选至250词)

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