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地中海贫血患者的血红蛋白水平及血液需求量

Haemoglobin levels and blood requirement in thalassaemia.

作者信息

Gabutti V, Piga A, Nicola P, Vullo C, Capra L, Di Palma A, Masera G, Terzoli S, Mauri R

出版信息

Arch Dis Child. 1982 Feb;57(2):156-8. doi: 10.1136/adc.57.2.156.

DOI:10.1136/adc.57.2.156
PMID:7065714
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC1627514/
Abstract

The relationship between blood requirement and the mean level of maintained haemoglobin was examined in 392 patients with homozygous beta-thalassaemia. Pre- and post-transfusional haemoglobin levels and the amounts of blood transfused were measured during a 1-year period. No significant differences were noted in the blood requirements of patients (splenectomised or not) irrespective of the haemoglobin level. It may be supposed that if the mean haemoglobin level is high the haematopoietic activity is inhibited, and hence the bone marrow mass and total blood volume are reduced. High haemoglobin levels may thus be obtained with no increase in blood intake.

摘要

在392例纯合子β地中海贫血患者中研究了血液需求量与维持的血红蛋白平均水平之间的关系。在1年期间测量了输血前后的血红蛋白水平及输血量。无论血红蛋白水平如何,(脾切除或未脾切除的)患者的血液需求量均未发现显著差异。可以推测,如果血红蛋白平均水平较高,造血活性会受到抑制,从而骨髓量和总血容量会减少。因此,可不增加血液摄入量而维持较高的血红蛋白水平。

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Haemoglobin levels and blood requirement in thalassaemia.地中海贫血患者的血红蛋白水平及血液需求量
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引用本文的文献

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Management of thalassaemia major.重型地中海贫血的管理
Arch Dis Child. 1983 Dec;58(12):1026-30. doi: 10.1136/adc.58.12.1026.
2
Insulin dependent diabetes in thalassaemia.地中海贫血中的胰岛素依赖型糖尿病
Arch Dis Child. 1988 Jan;63(1):58-62. doi: 10.1136/adc.63.1.58.
3
Current concepts in the management of thalassemia.地中海贫血管理的当前概念。
Indian J Pediatr. 1987 May-Jun;54(3):379-89. doi: 10.1007/BF02748923.
4
Studies of variations of subcutaneously infused desferrioxamine and iron movements in thalassaemia children.地中海贫血患儿皮下输注去铁胺及铁代谢变化的研究。
Eur J Pediatr. 1989 Apr;148(6):503-6. doi: 10.1007/BF00441542.

本文引用的文献

1
TRANSFUSION THERAPY IN COOLEY'S ANEMIA: GROWTH AND HEALTH AS RELATED TO LONG-RANGE HEMOGLOBIN LEVELS. A PROGRESS REPORT.库利贫血的输血治疗:与长期血红蛋白水平相关的生长和健康。一份进展报告。
Ann N Y Acad Sci. 1964 Oct 7;119:736-47. doi: 10.1111/j.1749-6632.1965.tb54075.x.
2
Behaviour of myeloid precursors in homozygous beta thalassaemia.纯合子β地中海贫血中髓系前体细胞的行为。
Br J Haematol. 1980 Aug;45(4):599-605. doi: 10.1111/j.1365-2141.1980.tb07183.x.
3
New approaches to the transfusion management of thalassemia.地中海贫血输血管理的新方法。
Blood. 1980 Jan;55(1):55-60.
4
Correlation between transfusion requirement, blood volume and haemoglobin level in homozygous beta-thalassaemia.纯合子β地中海贫血患者输血需求、血容量与血红蛋白水平之间的相关性
Acta Haematol. 1980;64(2):103-8. doi: 10.1159/000207218.
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Internal regulation of iron absorption.铁吸收的内部调节
Nature. 1975 Jul 24;256(5515):328-9. doi: 10.1038/256328a0.
6
Total management of thalassaemia major.重型地中海贫血的全面管理。
Arch Dis Child. 1977 Jun;52(6):489-500. doi: 10.1136/adc.52.6.489.
7
Iron absorption in the thalassemia syndromes and its inhibition by tea.地中海贫血综合征中的铁吸收及其受茶的抑制作用。
N Engl J Med. 1979 Jan 4;300(1):5-8. doi: 10.1056/NEJM197901043000102.
8
Erythropoiesis and the effect of transfusion in homozygous beta-thalassemia.纯合子β地中海贫血中的红细胞生成及输血影响
N Engl J Med. 1978 Apr 6;298(14):776-8. doi: 10.1056/NEJM197804062981406.