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红细胞锌原卟啉浓度在儿童小红细胞症调查中的应用价值

Usefulness of red cell zinc protoporphyrin concentration in the investigation of microcytosis in children.

作者信息

Hinchliffe R F, Lilleyman J S, Steel G J, Bellamy G J

机构信息

University of Sheffield, Department of Paediatrics, Children's Hospital, United Kingdom.

出版信息

Pediatr Hematol Oncol. 1995 Sep-Oct;12(5):455-62. doi: 10.3109/08880019509009475.

Abstract

The clinical usefulness of the measurement of red cell zinc protoporphyrin (ZPP), an indicator of iron-deficient erythropoiesis, was assessed in a group of UK children undergoing investigation for red cell microcytosis. Of 213 children studied, 136 had increased ZPP values. Of these, 86 also had reduced iron stores as indicated by serum ferritin concentration. The 50 children with increased ZPP and normal ferritin values could be divided into two main groups. One group comprised 28 children who had evidence of coexistent infection or inflammatory disease. The other included 21 children who had beta-thalassemia trait (n = 19) or disease (n = 2). Among the 77 children with normal ZPP values, 22 had reduced serum ferritin concentrations and 45 did not, nor did they have evidence of beta-thalassemia. Microcytosis in some of these children could have been due to alpha-thalassemia trait. Measurement of ZPP is a simple, quick, and relatively cheap method of confirming the presence of iron-deficient erythropoiesis even when inflammation makes serum ferritin measurements unreliable. It is not as sensitive as the ferritin assay to the early stages of iron deficiency, and its specificity is reduced by the occurrence of raised values in most children with beta-thalassemia trait. Where there is microcytosis, normal values, together with normal hemoglobin A2 and serum ferritin concentrations, are likely to indicate alpha-thalassemia trait.

摘要

对一组因红细胞小红细胞症而接受检查的英国儿童,评估了红细胞锌原卟啉(ZPP)测量值作为缺铁性红细胞生成指标的临床实用性。在研究的213名儿童中,136名儿童的ZPP值升高。其中,86名儿童的血清铁蛋白浓度表明其铁储备减少。ZPP值升高而铁蛋白值正常的50名儿童可分为两个主要组。一组包括28名有合并感染或炎症性疾病证据的儿童。另一组包括21名有β地中海贫血特征(n = 19)或疾病(n = 2)的儿童。在ZPP值正常的77名儿童中,22名儿童血清铁蛋白浓度降低,45名儿童血清铁蛋白浓度未降低,且他们也没有β地中海贫血的证据。这些儿童中的一些小红细胞症可能是由于α地中海贫血特征所致。测量ZPP是一种简单、快速且相对便宜的方法,即使在炎症使血清铁蛋白测量值不可靠时,也能确认缺铁性红细胞生成的存在。它对缺铁早期阶段的敏感性不如铁蛋白测定,并且由于大多数有β地中海贫血特征的儿童ZPP值升高,其特异性降低。在存在小红细胞症的情况下,正常的值以及正常的血红蛋白A2和血清铁蛋白浓度可能表明存在α地中海贫血特征。

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