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血清免疫球蛋白水平未降低的抗体缺乏症

[Antibody deficiency without decrease in the level of serum immunoglobulins].

作者信息

Bellon G, Touraine J L, Gilly R

出版信息

Arch Fr Pediatr. 1984 Dec;41(10):673-9.

PMID:6532356
Abstract

The paper reports the case of a 14 year-old boy, born at full term with intra-uterine growth retardation (1,640 g) who presented with short stature, generalized eczema and recurrent infections. During the first years of life, hypogammaglobulinemia with antibody deficiency occurred. The in vitro T lymphocyte function was normal. The infections have become less severe. The plasma levels of IgA, IgG, IgM and IgE are normal. There is hyperimmunoglobulinemia D. The deficiency of antibodies against most of the tested antigens persists. T and B lymphocyte counts are normal. The in vitro lymphocyte proliferation with Concanavalin A, Phytohemagglutinin, Pokeweed mitogen and Nocardia is normal. The OKT4+ and OKT8+ cell counts are normal. The ratio "helper T cell/suppressive T cell activity" appears to be abnormal. A very prolonged maturation delay, possibly associated with fetal hypotrophy may be hypothesized.

摘要

该论文报告了一名14岁男孩的病例,他足月出生,出生时患有宫内生长迟缓(体重1640克),表现为身材矮小、全身性湿疹和反复感染。在生命的最初几年,出现了伴有抗体缺乏的低丙种球蛋白血症。体外T淋巴细胞功能正常。感染程度有所减轻。血浆中IgA、IgG、IgM和IgE水平正常。存在高免疫球蛋白血症D。针对大多数检测抗原的抗体缺乏仍然存在。T和B淋巴细胞计数正常。体外淋巴细胞对刀豆球蛋白A、植物血凝素、商陆有丝分裂原和诺卡氏菌的增殖反应正常。OKT4 +和OKT8 +细胞计数正常。“辅助性T细胞/抑制性T细胞活性”比值似乎异常。可能与胎儿发育迟缓相关的非常长时间的成熟延迟可以被推测。

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