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严重联合免疫缺陷中的趋化性缺陷。

Chemotactic defects in severe combined immunodeficiency.

作者信息

Pahwa S G, Smithwick E M, Grimes E R, O'Reilly R J, Pahwa R N, Good R A

出版信息

J Pediatr. 1978 Jan;92(1):43-50. doi: 10.1016/s0022-3476(78)80068-7.

DOI:10.1016/s0022-3476(78)80068-7
PMID:338873
Abstract

Cellular and humoral components of leukotaxis were studied serially in four male infants with severe combined immunodeficiency disease. Two of the four, both lacking B and T cells initially, had a significant defect in neutrophil and monocyte chemotaxis. The other two, who had a high number of immunoglobulin-bearing cells (B cells), did not have these cellular abnormalities. It contrast, defective generation of chemotactic factor following endotoxin activation was observed in all patients. The defects were corrected coincident with or soon after successful engraftment of either bone marrow or fetal tissues. The reported deficiencies may be another manifestation of the heterogeneity in SCID.

摘要

对四名患有严重联合免疫缺陷病的男婴的趋化性细胞和体液成分进行了连续研究。四名中的两名最初同时缺乏B细胞和T细胞,其嗜中性粒细胞和单核细胞趋化性存在明显缺陷。另外两名有大量携带免疫球蛋白的细胞(B细胞),没有这些细胞异常。相比之下,所有患者在内毒素激活后趋化因子的生成均存在缺陷。这些缺陷在骨髓或胎儿组织成功植入时或之后不久得到纠正。所报告的缺陷可能是严重联合免疫缺陷病异质性的另一种表现。

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Chemotactic defects in severe combined immunodeficiency.严重联合免疫缺陷中的趋化性缺陷。
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