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布卢姆综合征的免疫学研究。随访报告。

Immunological studies in Bloom's syndrome. A follow-up report.

作者信息

Weemaes C M, Bakkeren J A, Haraldsson A, Smeets D F

机构信息

Department of Pediatrics, University Hospital, Nijmegen, The Netherlands.

出版信息

Ann Genet. 1991;34(3-4):201-5.

PMID:1809227
Abstract

Longitudinal immunological studies were performed in five patients with Bloom's syndrome. Serum IgG concentration showed no age-related increase. IgA levels were below the 10th percentile in childhood, but rose to normal in all but one. IgM was decreased in four patients at diagnosis and remained so in three of them. Cellular immunity results were very variable, even within one patient. An immunologic attrition was not demonstrated.

摘要

对5例布卢姆综合征患者进行了纵向免疫学研究。血清IgG浓度未显示出与年龄相关的增加。IgA水平在儿童期低于第10百分位数,但除1例患者外,其他患者的IgA水平均升至正常。4例患者在诊断时IgM降低,其中3例患者的IgM水平持续降低。细胞免疫结果差异很大,即使在同一患者体内也是如此。未证实存在免疫耗竭。

相似文献

1
Immunological studies in Bloom's syndrome. A follow-up report.布卢姆综合征的免疫学研究。随访报告。
Ann Genet. 1991;34(3-4):201-5.
2
Humoral and cellular immune dysfunction in a patient with Bloom's syndrome and recurrent infections.一名患有布卢姆综合征并反复感染患者的体液和细胞免疫功能障碍。
J Clin Lab Immunol. 1989 Mar;28(3):151-4.
3
Bloom's syndrome: the German experience.
Ann Genet. 1991;34(3-4):179-97.
4
[Familial Bloom's syndrome associated with neuroblastoma].[与神经母细胞瘤相关的家族性布卢姆综合征]
Rev Paul Med. 1990 Jan-Feb;108(1):9-16.
5
A comparison of purine and pyrimidine pools in Bloom's syndrome and normal cells.
Cancer Biochem Biophys. 1983 Dec;7(1):19-25.
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DNA repair and the molecular mechanisms of Bloom's syndrome.DNA修复与布卢姆综合征的分子机制。
Crit Rev Oncog. 1990;2(1):19-33.
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Immunological alteration of the Bloom's syndrome uracil DNA glycosylase in Epstein-Barr virus-transformed human lymphoblastoid cells.布卢姆综合征尿嘧啶DNA糖基化酶在爱泼斯坦-巴尔病毒转化的人淋巴母细胞样细胞中的免疫改变
Mutat Res. 1990 Mar;243(3):241-8. doi: 10.1016/0165-7992(90)90097-4.
8
Long-term study of the immunodeficiency of Bloom's syndrome.布卢姆综合征免疫缺陷的长期研究。
Acta Paediatr. 1992 Jan;81(1):86-90. doi: 10.1111/j.1651-2227.1992.tb12088.x.
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Antibody responses in vivo in chromosome instability syndromes with immunodeficiency.伴有免疫缺陷的染色体不稳定综合征的体内抗体反应。
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引用本文的文献

1
Immunodeficiency in Bloom's Syndrome.布卢姆综合征的免疫缺陷。
J Clin Immunol. 2018 Jan;38(1):35-44. doi: 10.1007/s10875-017-0454-y. Epub 2017 Nov 2.
2
Clinical and laboratory findings in 8 patients with Bloom's syndrome.8例布卢姆综合征患者的临床及实验室检查结果
J Dermatol Case Rep. 2012 Mar 27;6(1):29-33. doi: 10.3315/jdcr.2012.1086.
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Educational paper: syndromic forms of primary immunodeficiency.教育论文:原发性免疫缺陷的综合征形式。
Eur J Pediatr. 2011 Mar;170(3):295-308. doi: 10.1007/s00431-011-1396-7. Epub 2011 Feb 22.
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Immunodeficiency, centromeric region instability, facial anomalies syndrome (ICF).免疫缺陷、着丝粒区域不稳定、面部异常综合征(ICF)
Orphanet J Rare Dis. 2006 Mar 1;1:2. doi: 10.1186/1750-1172-1-2.