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[唐氏综合征患儿的免疫特征]

[The immunological profile of children with Down's syndrome].

作者信息

Colombo M L, Girardo E, Saitta M, Ricci B M, Maina D

出版信息

Minerva Pediatr. 1989 Jan;41(1):1-4.

PMID:2525223
Abstract

In 1965 Benda demonstrated that bioptic and autoptic material of children with Down's syndrome showed hypoplasia of the thymus with poor histological differentiation between the cortex and medulla and impairment of Hassal's corpuscles which were also fewer than normal. In the seventies studies revealed the increased susceptibility to infections and higher incidence of leucosis in Down's syndrome patients as well as changes of immunologic defences (in particular cell mediated immunity). This study examines 35 children (16 boys and 19 girls) aged 6 months-20 years. Subjects were divided into a group of 18 cases in poor health with a history of recurrent infections and a group of 17 children in good health. Skin tests were performed by inoculating 0.1 ml of a solution formed by 1 ml physiological solution and 0.1 ml tetanus toxoid. Skin reaction was evaluated 48 hours later. Lymphocyte typing tests were performed with the rosette method and with monoclonal antibodies for T lymphocytes and with the determination of surface immunoglobulins for B lymphocytes. OKT4 (T helper), OKT8 (T suppressor) subsets assayed and the OKT4/OKT8 ratio was determined. Skin tests were negative in 3 cases (8.6%). The number of B lymphocytes was normal in all children. Total number of lymphocytes was decreased in 51.4% of cases. Two subjects had a reduction of OKT4 and 14 had an increased of OKT8 and 16 a significantly lower OKT4/OKT8 ratio. It is clear that skin tests were normal also in those children with low total lymphocyte values. The most closely related parameter to mobility was the OKT4/OKT8 ratio and the most distantly related on was the skin test. Only 3 cases had modifications of all 3 parameters together. Apart from the constant and complete immunological deficit described by many authors and which we cannot confirm the results of this study are in agreement with those of other authors.

摘要

1965年,本达证明,唐氏综合征患儿的活检和尸检材料显示胸腺发育不全,皮质和髓质之间的组织学分化较差,哈氏小体受损,其数量也比正常情况少。在70年代,研究表明唐氏综合征患者对感染的易感性增加,白血病发病率更高,以及免疫防御发生变化(特别是细胞介导免疫)。本研究对35名年龄在6个月至20岁的儿童(16名男孩和19名女孩)进行了检查。受试者被分为一组18例健康状况不佳且有反复感染史的病例和一组17名健康状况良好的儿童。通过接种由1毫升生理盐水和0.1毫升破伤风类毒素形成的溶液0.1毫升进行皮肤试验。48小时后评估皮肤反应。采用玫瑰花结法、针对T淋巴细胞的单克隆抗体以及针对B淋巴细胞的表面免疫球蛋白测定进行淋巴细胞分型试验。检测OKT4(辅助性T细胞)、OKT8(抑制性T细胞)亚群并确定OKT4/OKT8比值。3例(8.6%)皮肤试验呈阴性。所有儿童的B淋巴细胞数量正常。51.4%的病例淋巴细胞总数减少。2名受试者OKT4减少,14名受试者OKT8增加,16名受试者OKT4/OKT8比值显著降低。很明显,淋巴细胞总数较低的儿童皮肤试验也正常。与活动性最密切相关的参数是OKT4/OKT8比值,与活动性最不相关的是皮肤试验。只有3例所有3个参数同时发生改变。除了许多作者描述的持续且完全的免疫缺陷(我们无法证实)外,本研究结果与其他作者的结果一致。

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