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红细胞输注、胸腺激素和脱氧胞苷对腺苷脱氨酶缺乏所致重症联合免疫缺陷的影响。

Effect of red cell transfusions, thymic hormone and deoxycytidine in severe combined immunodeficiency due to adenosine deaminase deficiency.

作者信息

Davies E G, Levinsky R J, Webster D R, Simmonds H A, Perrett D

出版信息

Clin Exp Immunol. 1982 Nov;50(2):303-10.

PMID:6983936
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC1536687/
Abstract

A child with severe combined immunodeficiency due to adenosine deaminase deficiency was treated with thymic hormone (TP1) alone, thymic hormone with red cell transfusions and finally with a combination of these and parenteral deoxycytidine. TP1 alone produced no benefit while the addition of red cell transfusions resulted in only partial immunological restoration despite improvement in clinical and biochemical findings. Deoxycytidine given subcutaneously produced no additional benefit in any of the parameters measured.

摘要

一名因腺苷脱氨酶缺乏导致严重联合免疫缺陷的儿童,首先单独接受胸腺激素(TP1)治疗,之后接受胸腺激素与红细胞输注联合治疗,最后接受这些治疗与胃肠外脱氧胞苷联合治疗。单独使用TP1没有效果,而添加红细胞输注尽管临床和生化指标有所改善,但仅导致部分免疫恢复。皮下注射脱氧胞苷在任何测量参数上均未产生额外益处。

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1
Effect of red cell transfusions, thymic hormone and deoxycytidine in severe combined immunodeficiency due to adenosine deaminase deficiency.红细胞输注、胸腺激素和脱氧胞苷对腺苷脱氨酶缺乏所致重症联合免疫缺陷的影响。
Clin Exp Immunol. 1982 Nov;50(2):303-10.
2
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Accumulation of deoxyribonucleotides as a possible mediator of immunosuppression in hereditary deficiency of adenosine deaminase.脱氧核糖核苷酸的积累可能是腺苷脱氨酶遗传性缺乏时免疫抑制的介质。
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Overproduction of adenine deoxynucleosides and deoxynucletides in adenosine deaminase deficiency with severe combined immunodeficiency disease.腺苷脱氨酶缺乏伴重症联合免疫缺陷病中腺嘌呤脱氧核苷和脱氧核苷酸的过量产生。
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Comparison of red cell transfusion and polyethylene glycol-modified adenosine deaminase therapy in an adenosine deaminase-deficient child: measurement of erythrocyte deoxyadenosine triphosphate as a useful tool.腺苷脱氨酶缺乏患儿红细胞输血与聚乙二醇修饰腺苷脱氨酶治疗的比较:以红细胞三磷酸脱氧腺苷测定作为一种有用工具
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[Transplantation of T cell-depleted marrow from parents in a case of severe combined immunodeficiency with adenosine deaminase deficiency].[在一例伴有腺苷脱氨酶缺乏的重症联合免疫缺陷病例中,移植来自父母的T细胞去除的骨髓]
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引用本文的文献

1
Prenatal diagnosis of three cases of severe combined immunodeficiency: severe T cell deficiency during the first half of gestation in fetuses with adenosine deaminase deficiency.三例重症联合免疫缺陷的产前诊断:腺苷脱氨酶缺乏胎儿在妊娠前半期严重T细胞缺乏。
Clin Exp Immunol. 1984 May;56(2):223-32.
2
Lymphocyte subpopulations in primary immunodeficiency disorders.原发性免疫缺陷病中的淋巴细胞亚群
Arch Dis Child. 1983 May;58(5):346-51. doi: 10.1136/adc.58.5.346.

本文引用的文献

1
Reciprocal relationship between erythrocyte ATP and deoxy-ATP levels in inherited ADA deficiency.遗传性腺苷脱氨酶缺乏症中红细胞ATP与脱氧ATP水平之间的相互关系。
Biochem Pharmacol. 1982 Mar 15;31(6):947-51. doi: 10.1016/0006-2952(82)90325-2.
2
The differentiation and function of human T lymphocytes.人类T淋巴细胞的分化与功能。
Cell. 1980 Apr;19(4):821-7. doi: 10.1016/0092-8674(80)90072-0.
3
Treatment of cell-mediated immunodeficiency with calf thymic hormone (T.P.I.).用小牛胸腺激素(T.P.I.)治疗细胞介导的免疫缺陷。
Pediatr Res. 1982 Jul;16(7):573-8. doi: 10.1203/00006450-198207000-00018.
4
Discrete stages of human intrathymic differentiation: analysis of normal thymocytes and leukemic lymphoblasts of T-cell lineage.人类胸腺内分化的离散阶段:T细胞系正常胸腺细胞和白血病淋巴母细胞的分析
Proc Natl Acad Sci U S A. 1980 Mar;77(3):1588-92. doi: 10.1073/pnas.77.3.1588.
5
Human T lymphocyte subpopulations defined by Fc receptors and monoclonal antibodies. A comparison.由Fc受体和单克隆抗体定义的人T淋巴细胞亚群。一项比较。
J Exp Med. 1980 Apr 1;151(4):969-74. doi: 10.1084/jem.151.4.969.
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Transplantation for acute leukaemia with HLA-A and B nonidentical parental marrow cells fractionated with soybean agglutinin and sheep red blood cells.
Lancet. 1981 Aug 15;2(8242):327-31. doi: 10.1016/s0140-6736(81)90647-4.
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Studies on adenine and adenosine metabolism by intact human erythrocytes using high performance liquid chromatography.利用高效液相色谱法对完整人红细胞中腺嘌呤和腺苷代谢的研究。
Biochim Biophys Acta. 1976 Jun 23;437(1):1-5. doi: 10.1016/0304-4165(76)90342-1.
8
The effect of adenosine on mitogenesis of ADA-deficient lymphocytes.腺苷对腺苷脱氨酶缺乏淋巴细胞有丝分裂的影响。
Clin Immunol Immunopathol. 1978 Aug;10(4):446-58. doi: 10.1016/0090-1229(78)90157-5.
9
Identification and quantitation of adenine deoxynucleotides in erythrocytes of a patient with adenosine deaminase deficiency and severe combined immunodeficiency.腺苷脱氨酶缺乏症和严重联合免疫缺陷患者红细胞中腺嘌呤脱氧核苷酸的鉴定与定量分析
J Biol Chem. 1978 Mar 10;253(5):1619-26.
10
Primary and secondary abnormalities of T cell subpopulations.T细胞亚群的原发性和继发性异常。
Clin Exp Immunol. 1978 Dec;34(3):388-92.