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普通可变免疫缺陷:诊断与管理

Common variable immunodeficiency: diagnosis and management.

作者信息

Eisenstein E M, Sneller M C

机构信息

Mucosal Immunity Section, Laboratory of Clinical Investigation, National Institute of Allergy and Infectious Disease, National Institutes of Health, Bethesda, Maryland.

出版信息

Ann Allergy. 1994 Oct;73(4):285-92; quiz 293-4.

PMID:7943995
Abstract

OBJECTIVE

Common variable immunodeficiency is a heterogeneous syndrome characterized by humoral immunodeficiency, recurrent bacterial infections, and a variety of immunologic abnormalities. The goal of this article is to review the pathogenesis, clinical manifestations, diagnosis, and management of common variable immunodeficiency.

DATA SOURCES

References are limited to the English language literature. Sources include computerized databases and bibliographies of recent articles and books.

STUDY SELECTION

References that made important contributions to our understanding of the pathogenesis, clinical manifestations, diagnosis, and treatment of this disease were selected for inclusion in this review.

RESULTS AND CONCLUSION

Common variable immunodeficiency is an idiopathic state of immune dysregulation that results in impaired antibody synthesis and the development of recurrent bacterial infections. This syndrome is also associated with a variety of autoimmune and neoplastic disorders. Effective management of these patients includes intravenous immunoglobulin replacement, vigorous treatment of infections, and readiness to promptly evaluate and treat unusual autoimmune and neoplastic complications should they appear.

摘要

目的

普通可变型免疫缺陷是一种异质性综合征,其特征为体液免疫缺陷、复发性细菌感染以及多种免疫异常。本文的目的是综述普通可变型免疫缺陷的发病机制、临床表现、诊断及管理。

资料来源

参考文献仅限于英文文献。资料来源包括计算机化数据库以及近期文章和书籍的参考文献目录。

研究选择

对我们理解该疾病的发病机制、临床表现、诊断及治疗有重要贡献的参考文献被选入本综述。

结果与结论

普通可变型免疫缺陷是一种免疫调节异常的特发性状态,导致抗体合成受损及复发性细菌感染的发生。该综合征还与多种自身免疫性和肿瘤性疾病相关。对这些患者的有效管理包括静脉注射免疫球蛋白替代治疗、积极治疗感染,以及在出现自身免疫和肿瘤并发症时随时准备及时评估和处理。

相似文献

1
Common variable immunodeficiency: diagnosis and management.普通可变免疫缺陷:诊断与管理
Ann Allergy. 1994 Oct;73(4):285-92; quiz 293-4.
2
[Common variable immunodeficiency].[普通变异型免疫缺陷]
Recenti Prog Med. 1996 Dec;87(12):616-22.
3
Common variable immunodeficiency: a review.常见可变免疫缺陷:综述
Clin Exp Med. 2004 Apr;3(4):211-7. doi: 10.1007/s10238-004-0027-2.
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Common variable immunodeficiency: familial inheritance and autoimmune manifestations in two siblings.普通可变免疫缺陷:两例同胞的家族遗传及自身免疫表现
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[CVID (common variable immunodeficiency): heterogeneous clinical manifestation of the commonest symptomatic primary immunodeficiency disease].[普通变异型免疫缺陷病(CVID):最常见的有症状的原发性免疫缺陷病的异质性临床表现]
Schweiz Med Wochenschr. 2000 Oct 28;130(43):1590-9.
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Common variable immunodeficiency: an update on etiology and management.普通可变免疫缺陷:病因与管理的最新进展
Immunol Allergy Clin North Am. 2008 May;28(2):367-86, ix-x. doi: 10.1016/j.iac.2008.01.001.
7
Presence of Idiopathic Thrombocytopenic Purpura and autoimmune hemolytic anemia in the patients with common variable immunodeficiency.普通可变免疫缺陷患者中特发性血小板减少性紫癜和自身免疫性溶血性贫血的存在情况。
Iran J Allergy Asthma Immunol. 2008 Sep;7(3):169-75.
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[Common variable immunodeficiency--advances in diagnosis, pathogenesis and treatment].[常见可变免疫缺陷——诊断、发病机制及治疗进展]
Pol Merkur Lekarski. 2003 Jan;14(79):73-8.
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[Common variable immunodeficiency. A clinical approach].[普通可变免疫缺陷。临床处理方法]
Rev Invest Clin. 2010 Nov-Dec;62(6):577-82.
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Respiratory disorders in common variable immunodeficiency.普通可变免疫缺陷中的呼吸系统疾病
Respir Med. 2001 Mar;95(3):191-5. doi: 10.1053/rmed.2000.1020.

引用本文的文献

1
Diagnostic findings in 95 Finnish patients with common variable immunodeficiency.95例芬兰常见变异型免疫缺陷患者的诊断结果
J Clin Immunol. 2001 Mar;21(2):145-9. doi: 10.1023/a:1011012023616.
2
Role of antibodies in controlling viral disease: lessons from experiments of nature and gene knockouts.抗体在控制病毒性疾病中的作用:来自自然实验和基因敲除实验的经验教训。
J Virol. 2000 Nov;74(21):9813-7. doi: 10.1128/jvi.74.21.9813-9817.2000.
3
Detection of Bruton's tyrosine kinase mutations in hypogammaglobulinaemic males registered as common variable immunodeficiency (CVID) in the Japanese Immunodeficiency Registry.
在日本免疫缺陷登记处登记为常见变异型免疫缺陷(CVID)的低丙种球蛋白血症男性中检测布鲁顿酪氨酸激酶突变。
Clin Exp Immunol. 2000 Jun;120(3):512-7. doi: 10.1046/j.1365-2249.2000.01244.x.
4
A review of immunologic diseases of the dog.犬类免疫性疾病综述。
Vet Immunol Immunopathol. 1999 Aug 2;69(2-4):251-342. doi: 10.1016/s0165-2427(99)00059-8.
5
Common variable immunodeficiency with CD4+ T lymphocytopenia and overproduction of soluble IL-2 receptor associated with Turner's syndrome and dorsal kyphoscoliosis.伴有CD4+ T淋巴细胞减少及可溶性白细胞介素-2受体过度产生的常见变异型免疫缺陷,与特纳综合征及背部脊柱后凸侧凸相关。
J Clin Pathol. 1997 Oct;50(10):876-9. doi: 10.1136/jcp.50.10.876.