腺苷脱氨酶缺乏型联合免疫缺陷患者分泌型免疫球蛋白A的局部缺乏

Regional deficiency of secretory IgA in a patient with combined immunodeficiency of the ADA deficient type.

作者信息

Moore E C, Laffin R J, Tomasi T, Pickering R J, Radl J, Meuwissen H J

出版信息

Acta Paediatr Scand. 1979 May;68(3):453-8. doi: 10.1111/j.1651-2227.1979.tb05038.x.

DOI:10.1111/j.1651-2227.1979.tb05038.x

Abstract

The IgA system in a patient with SCID and ADA deficiency showed heterogeneity. Serum IgA and stool secretory IgA (SIgA) levels were normal, but with altered kappa/lambda and A1/A2 subclass ratios; IgA in saliva and urine was deficient. Amounts of secretory component were normal. Jejunal and rectal biopsies showed prominent lymphonodular hyperplasia, but no cells containing IgA. A normal serum IgA level therefore does not always predict an intact secretory IgA system.

摘要

患有重症联合免疫缺陷病（SCID）和腺苷脱氨酶（ADA）缺乏症的患者的IgA系统表现出异质性。血清IgA和粪便分泌型IgA（SIgA）水平正常，但κ/λ和A1/A2亚类比例发生改变；唾液和尿液中的IgA缺乏。分泌成分的量正常。空肠和直肠活检显示明显的淋巴小结增生，但未发现含IgA的细胞。因此，正常的血清IgA水平并不总是预示着分泌型IgA系统完整。

相似文献

1

Regional deficiency of secretory IgA in a patient with combined immunodeficiency of the ADA deficient type.腺苷脱氨酶缺乏型联合免疫缺陷患者分泌型免疫球蛋白A的局部缺乏

Acta Paediatr Scand. 1979 May;68(3):453-8. doi: 10.1111/j.1651-2227.1979.tb05038.x.

2

Amelioration of neurologic abnormalities after "enzyme replacement" in adenosine deaminase deficiency.腺苷脱氨酶缺乏症“酶替代”治疗后神经功能异常的改善

N Engl J Med. 1980 Aug 14;303(7):377-80. doi: 10.1056/NEJM198008143030706.

3

Secretory antibodies in IgA-deficient and immunosuppressed individuals.IgA 缺乏和免疫抑制个体中的分泌性抗体。

J Clin Immunol. 1986 Jul;6(4):284-91. doi: 10.1007/BF00917328.

4

Abnormal platelet aggregation in severe combined immunodeficiency disease with adenosine deaminase deficiency.伴有腺苷脱氨酶缺乏的重症联合免疫缺陷病中的异常血小板聚集。

Br J Haematol. 1978 Jun;39(2):189-94. doi: 10.1111/j.1365-2141.1978.tb01088.x.

5

Pathologic findings in adenosine deaminase deficient-severe combined immunodeficiency. II. Thymus, spleen, lymph node, and gastrointestinal tract lymphoid tissue alterations.腺苷脱氨酶缺乏型重症联合免疫缺陷的病理发现。II. 胸腺、脾脏、淋巴结及胃肠道淋巴组织改变

Am J Pathol. 1989 Dec;135(6):1145-56.

6

Immunoglobulin levels during follow-up of children with selective IgA deficiency.选择性IgA缺乏症患儿随访期间的免疫球蛋白水平。

Scand J Immunol. 1992 Jun;35(6):719-25. doi: 10.1111/j.1365-3083.1992.tb02980.x.

7

Purine nucleoside metabolism in the erythrocytes of patients with adenosine deaminase deficiency and severe combined immunodeficiency.腺苷脱氨酶缺乏和严重联合免疫缺陷患者红细胞中的嘌呤核苷代谢

J Clin Invest. 1976 Apr;57(4):1025-35. doi: 10.1172/JCI108344.

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

Severe combined immunodeficiency disease with adenosine deaminase deficiency.伴有腺苷脱氨酶缺乏的重症联合免疫缺陷病

Tohoku J Exp Med. 1979 Oct;129(2):197-202. doi: 10.1620/tjem.129.197.

10

Heterogeneity of biochemical, clinical and immunological parameters in severe combined immunodeficiency due to adenosine deaminase deficiency.腺苷脱氨酶缺乏所致重症联合免疫缺陷中生化、临床及免疫学参数的异质性。

Clin Exp Immunol. 1987 Dec;70(3):491-9.

引用本文的文献

1

The IgA immune system in epileptics on anticonvulsant therapy.接受抗惊厥治疗的癫痫患者的IgA免疫系统。

Eur J Clin Pharmacol. 1984;27(1):81-3.

2

Pathologic findings in adenosine deaminase deficient-severe combined immunodeficiency. II. Thymus, spleen, lymph node, and gastrointestinal tract lymphoid tissue alterations.腺苷脱氨酶缺乏型重症联合免疫缺陷的病理发现。II. 胸腺、脾脏、淋巴结及胃肠道淋巴组织改变

Am J Pathol. 1989 Dec;135(6):1145-56.

文献AI研究员

20分钟写一篇综述，助力文献阅读效率提升50倍。

用中文搜PubMed

大模型驱动的PubMed中文搜索引擎

文档翻译

学术文献翻译模型，支持多种主流文档格式。