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[患有发育性胸廓缺陷儿童的免疫紊乱]

[Immunologic disorders in children with developmental thoracic defects].

作者信息

Samsygin S A, Dolgina E N, Luk'ianov S V, Rudakov S S

出版信息

Khirurgiia (Mosk). 1990 Aug(8):85-9.

PMID:2259182
Abstract

As the result of immunological examination of 21 children with developmental defects of the chest and analysis of the course of the postoperative period in 136 children, among which 36 had hereditary syndromes of systemic connective-tissue dyshistogenesis, it was found that suppurative complications of thoracoplasty, which are encountered in 15% of children with isolated developmental chest defects and in 33.3% of those with the above mentioned syndromes, were caused to a great measure by disorders of the immune status. The most serious immunological deviations were encountered in the Marfan syndrome due to impaired phagocytic activity of neutrophils and monocytes, decreased number of T, T active, and B lymphocytes, and diminished function of T helpers. In unclassified complexes of developmental defects with Marfaneic ++ phenotypes, the immunological disorders were similar, but less deep. In the Ehlers-Danlos syndrome, a decrease of the number of immunocompetent cells, function of T helpers, and neutrophils was mainly revealed. In isolated forms of funnel chest the function of monocytes and the number of immunoglobulins are mainly decreased.

摘要

通过对21例胸部发育缺陷患儿进行免疫学检查,并分析136例患儿术后病程,其中36例患有全身性结缔组织发育异常的遗传综合征,发现胸廓成形术的化脓性并发症在15%的孤立性胸部发育缺陷患儿和33.3%的上述综合征患儿中出现,在很大程度上是由免疫状态紊乱引起的。在马凡综合征中,由于中性粒细胞和单核细胞的吞噬活性受损、T细胞、活化T细胞和B淋巴细胞数量减少以及T辅助细胞功能减弱,出现了最严重的免疫偏差。在具有马凡样++表型的未分类发育缺陷复合体中,免疫紊乱相似,但程度较轻。在埃勒斯-当洛综合征中,主要表现为免疫活性细胞数量减少、T辅助细胞功能和中性粒细胞减少。在孤立性漏斗胸形式中,主要是单核细胞功能和免疫球蛋白数量减少。

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