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伴有腺苷脱氨酶缺乏的重症联合免疫缺陷病

Severe combined immunodeficiency disease with adenosine deaminase deficiency.

作者信息

Yokoyama S, Hayashi T, Yoshimura Y, Irimada K, Saito T, Akiba T, Tsuchiya S

出版信息

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

Abstract

Severe combined immunodeficiency disease with adenosine deaminase deficiency was reported of a 1-year-2-month-old boy, who showed clinical picture of pulmonary candidiasis. The serum immunoglobulin level and absolute lymphocyte count were markedly diminished. But immunological study revealed both T- and B-cell surface markers on the peripheral lymphocytes, in spite of complete deficiency of both systems.

摘要

据报道,一名1岁2个月大的男孩患有腺苷脱氨酶缺乏的重症联合免疫缺陷病,表现出肺部念珠菌病的临床症状。血清免疫球蛋白水平和绝对淋巴细胞计数显著降低。然而,免疫学研究显示外周淋巴细胞上同时存在T细胞和B细胞表面标志物,尽管这两个系统完全缺乏。

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