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一个选择性IgA缺乏症家族中的免疫调节缺陷。

Immunoregulatory defects in a family with selective IgA deficiency.

作者信息

Levitt D, Cooper M D

出版信息

J Pediatr. 1981 Jan;98(1):52-8. doi: 10.1016/s0022-3476(81)80532-x.

DOI:10.1016/s0022-3476(81)80532-x
PMID:6969795
Abstract

Defects in regulation of the humoral immune system were examined in a family with selective IgA deficiency. Two patients (mother and son) were clinically well and had selective abnormalities of B lymphocytes that can differentiate into IgA plasma cells plus specific T cell suppression of IgA production. One patient (daughter) had several clinical problems and received nine monthly transfusions of normal plasma. Prior to therapy, this patient had abnormal immunoregulation of both B and T lymphocyte population that cooperated in the formation of all cases of immunoglobulin-producing cells. After treatment, her clinical problems were resolved and her cellular abnormalities appeared identical in those in her mother and brother.

摘要

在一个患有选择性IgA缺乏症的家族中,对体液免疫系统调节缺陷进行了研究。两名患者(母亲和儿子)临床状况良好,存在B淋巴细胞的选择性异常,这些B淋巴细胞可分化为IgA浆细胞,同时存在特异性T细胞对IgA产生的抑制作用。一名患者(女儿)有多种临床问题,接受了9次每月一次的正常血浆输血。在治疗前,该患者B淋巴细胞和T淋巴细胞群体的免疫调节均异常,这些细胞在所有免疫球蛋白产生细胞的形成过程中相互协作。治疗后,她的临床问题得到解决,其细胞异常情况与她的母亲和兄弟相同。

相似文献

1
Immunoregulatory defects in a family with selective IgA deficiency.一个选择性IgA缺乏症家族中的免疫调节缺陷。
J Pediatr. 1981 Jan;98(1):52-8. doi: 10.1016/s0022-3476(81)80532-x.
2
In-vitro analysis of defective IgA production in selective IgA deficiency in childhood.儿童选择性IgA缺乏症中缺陷性IgA产生的体外分析。
Clin Exp Immunol. 1985 Dec;62(3):746-52.
3
Disorders of regulatory T cells in patients with selective IgA deficiency and its relationship to associated autoimmune phenomena.选择性IgA缺乏症患者调节性T细胞紊乱及其与相关自身免疫现象的关系。
Clin Exp Immunol. 1982 Aug;49(2):410-8.
4
Selective immunoglobulin A deficiency associated with primary biliary cirrhosis in a family with liver disease.在一个患有肝脏疾病的家族中,选择性免疫球蛋白A缺乏症与原发性胆汁性肝硬化相关。
Gastroenterology. 1986 Feb;90(2):283-8. doi: 10.1016/0016-5085(86)90922-4.
5
Selective deficiency of immunoglobulin A2.免疫球蛋白A2选择性缺乏
J Clin Invest. 1983 Dec;72(6):1918-23. doi: 10.1172/JCI111155.
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Immunological study of IgA deficiency during anticonvulsant therapy in epileptic patients.癫痫患者抗惊厥治疗期间IgA缺乏的免疫学研究。
Clin Exp Immunol. 1983 Aug;53(2):423-8.
7
[Lymphocyte study in the selective deficit of IgA].
Recenti Prog Med. 1984 Dec;75(12):1061-75.
8
Hyper IgM immunodeficiency. A primary dysfunction of B lymphocyte isotype switching.高IgM免疫缺陷。B淋巴细胞同种型转换的原发性功能障碍。
J Clin Invest. 1983 Nov;72(5):1650-7. doi: 10.1172/JCI111124.
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Functional assessment of T and B lymphocytes in patients with selective IgM deficiency.选择性IgM缺乏症患者T和B淋巴细胞的功能评估
Clin Exp Immunol. 1982 Sep;49(3):670-6.
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IgA-containing plasma cells in the intestinal mucosa of children with selective IgA deficiency.
J Clin Lab Immunol. 1982 Dec;9(3):173-5.

引用本文的文献

1
Genetic linkage of IgA deficiency to the major histocompatibility complex: evidence for allele segregation distortion, parent-of-origin penetrance differences, and the role of anti-IgA antibodies in disease predisposition.IgA 缺乏症与主要组织相容性复合体的遗传连锁:等位基因分离畸变、亲本来源的外显率差异以及抗 IgA 抗体在疾病易感性中的作用的证据。
Am J Hum Genet. 1999 Apr;64(4):1096-109. doi: 10.1086/302326.
2
Hyper IgM immunodeficiency. A primary dysfunction of B lymphocyte isotype switching.高IgM免疫缺陷。B淋巴细胞同种型转换的原发性功能障碍。
J Clin Invest. 1983 Nov;72(5):1650-7. doi: 10.1172/JCI111124.
3
Anti-IgA antibodies and transfusion reactions in Canada.
加拿大的抗IgA抗体与输血反应
Can Med Assoc J. 1983 Feb 15;128(4):381-2.
4
Epstein-Barr virus-induced lymphoblastoid cell lines derived from the peripheral blood of patients with X-linked agammaglobulinemia can secrete IgM.源自X连锁无丙种球蛋白血症患者外周血的爱泼斯坦-巴尔病毒诱导的淋巴母细胞系可分泌IgM。
J Clin Immunol. 1984 Mar;4(2):143-50. doi: 10.1007/BF00915048.
5
Selective IgA deficiency: analysis of Ig production in vitro.选择性IgA缺乏症:体外Ig产生的分析
J Clin Immunol. 1984 May;4(3):235-41. doi: 10.1007/BF00914971.
6
Disorders of regulatory T cells in patients with selective IgA deficiency and its relationship to associated autoimmune phenomena.选择性IgA缺乏症患者调节性T细胞紊乱及其与相关自身免疫现象的关系。
Clin Exp Immunol. 1982 Aug;49(2):410-8.
7
Pokeweed mitogen and Staphylococcus aureus Cowan I induced immunoglobulin A synthesis by lymphocytes of IgA deficient blood donors.商陆有丝分裂原和金黄色葡萄球菌考恩I型可诱导IgA缺乏献血者淋巴细胞合成免疫球蛋白A。
Clin Exp Immunol. 1985 Nov;62(2):387-96.
8
Analysis of IgG subclass production in cell cultures from IgA deficient patients and in normal controls as a function of age.对IgA缺乏患者的细胞培养物以及正常对照中IgG亚类产生情况随年龄变化的分析。
Clin Exp Immunol. 1986 Aug;65(2):434-42.
9
Susceptibility to infections in children with selective IgA- and IgA-IgG subclass deficiency.
Eur J Pediatr. 1986 Aug;145(3):199-203. doi: 10.1007/BF00446065.
10
Immunoglobulin-producing cells in secretory immune system in patients with selective IgA deficiency.选择性IgA缺乏症患者分泌免疫系统中产生免疫球蛋白的细胞。
Gastroenterol Jpn. 1987 Aug;22(4):435-9. doi: 10.1007/BF02773810.