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与地中海贫血特征和血红蛋白E相关的锌原卟啉升高。

Elevated zinc protoporphyrin associated with thalassemia trait and hemoglobin E.

作者信息

Graham E A, Felgenhauer J, Detter J C, Labbe R F

机构信息

Department of Pediatrics, University of Washington, Seattle, USA.

出版信息

J Pediatr. 1996 Jul;129(1):105-10. doi: 10.1016/s0022-3476(96)70196-8.

DOI:10.1016/s0022-3476(96)70196-8
PMID:8757569
Abstract

OBJECTIVE

Increased zinc protoporphyrin/heme (ZPP/H) ratio has been used in pediatrics to screen for iron deficiency and lead poisoning. This study was conducted to determine whether common hereditary hemoglobin disorders (alpha- and beta-thalassemia traits, hemoglobin E) found in U.S. minority groups are associated with an increase in the ZPP/H ratio in an iron-sufficient population.

METHODS

The database was compiled from hemoglobinopathy screens performed between 1987 and 1993 at a regional referral laboratory in Washington State. ZPP/H ratio and hemoglobin type were obtained for 326 subjects between the ages of 15 and 49 years of age who were iron sufficient (serum ferritin levels > or = 50 micrograms/L).

RESULTS

The mean ZPP/H ratio was significantly higher (p < 0.01) for subjects with beta-thalassemia trait (87 +/- 32 micromol/mol), (alpha-thalassemia trait (73 +/- 37 micromol/mol), and hemoglobin E disorders (73 +/- 24 micromol/mol) than for subjects with normal hemoglobin values (60 +/- 8 micromol/mol). Fifty-one percent of subjects with beta-thalassemia trait, 22% with hemoglobin E, and 20% with alpha-thalassemia trait had elevated ZPP/H ratios (> 80 micromol/mol), compared with only 1.5% with normal hemoglobin values.

CONCLUSIONS

The ZPP/H ratio is elevated in common hereditary hemoglobin disorders that mimic the microcytic anemia of iron deficiency, even in individuals without associated nutritional iron deficiency. For children who are treated for presumed iron deficiency, failure of the ZPP/H ratio to return to normal after adequate iron treatment, especially if microcytosis persists, indicates that a hereditary hemoglobin disorder may be present.

摘要

目的

锌原卟啉/血红素(ZPP/H)比值升高已用于儿科筛查缺铁和铅中毒。本研究旨在确定美国少数族裔中常见的遗传性血红蛋白疾病(α和β地中海贫血特征、血红蛋白E)在铁充足人群中是否与ZPP/H比值升高有关。

方法

数据库来自1987年至1993年在华盛顿州一家区域转诊实验室进行的血红蛋白病筛查。获取了326名年龄在15至49岁之间铁充足(血清铁蛋白水平≥50微克/升)的受试者的ZPP/H比值和血红蛋白类型。

结果

β地中海贫血特征的受试者(87±32微摩尔/摩尔)、α地中海贫血特征的受试者(73±37微摩尔/摩尔)和血红蛋白E疾病的受试者(73±24微摩尔/摩尔)的平均ZPP/H比值显著高于血红蛋白值正常的受试者(60±8微摩尔/摩尔)(p<0.01)。β地中海贫血特征的受试者中有51%、血红蛋白E的受试者中有22%、α地中海贫血特征的受试者中有20%的ZPP/H比值升高(>80微摩尔/摩尔),而血红蛋白值正常的受试者中只有1.5%。

结论

常见的遗传性血红蛋白疾病即使在没有相关营养性缺铁的个体中,也会出现ZPP/H比值升高,这些疾病可模拟缺铁性小细胞贫血。对于因疑似缺铁而接受治疗的儿童,如果在充分补铁治疗后ZPP/H比值未能恢复正常,尤其是如果小细胞症持续存在,则表明可能存在遗传性血红蛋白疾病。

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