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一名15个月大儿童早衰症(哈钦森-吉尔福德综合征)的免疫学方面

Immunological aspects of progeria (Hutchinson-Gilford syndrome) in a 15-month-old child.

作者信息

Harjacek M, Batinić D, Sarnavka V, Uzarević B, Mardesić D, Marusić M

机构信息

Department of Paediatrics, University Hospital Rebro, Croatia, Yugoslavia.

出版信息

Eur J Pediatr. 1990 Nov;150(1):40-2. doi: 10.1007/BF01959478.

DOI:10.1007/BF01959478
PMID:2079076
Abstract

A thorough analysis of the immunological status was conducted in a 15-month-old child with progeria (Hutchinson-Gilford syndrome). Total leukocyte and neutrophil counts were slightly increased, and monocytes were decreased. Percentage and numbers of CD4+ cells in the blood were mildly decreased as well as the CD4/CD8 cell ratio. CD20 (B-cell marker) bearing cells and cells bearing Ia-antigens were increased, as well as CD16 and CD56 marker-bearing cells (natural-killer cells, NK). Lymphocyte proliferation upon stimulation with phytohaemagglutinin and purified protein derivative were decreased, and with pokeweed mitogen increased. NK cell activity appeared increased, particularly at lower effector: target cell ratios.

摘要

对一名患有早老症(哈钦森-吉尔福德综合征)的15个月大儿童进行了全面的免疫状态分析。白细胞和中性粒细胞总数略有增加,单核细胞减少。血液中CD4+细胞的百分比和数量以及CD4/CD8细胞比值均轻度降低。携带CD20(B细胞标志物)的细胞和携带Ia抗原的细胞增加,以及携带CD16和CD56标志物的细胞(自然杀伤细胞,NK)增加。用植物血凝素和纯化蛋白衍生物刺激后淋巴细胞增殖减少,用商陆有丝分裂原刺激后淋巴细胞增殖增加。NK细胞活性似乎增加,尤其是在较低的效应细胞:靶细胞比值时。

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本文引用的文献

1
Report of a patient with typical progeria, including data from urinary hormone studies.一名典型早老症患者的报告,包括尿激素研究数据。
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Subpopulations of human T lymphocytes. XVII. Imbalance of T cell subsets in patients with progeria and Werner's syndrome.人类T淋巴细胞亚群。十七。早老症和沃纳综合征患者T细胞亚群的失衡。
Gerontology. 1981;27(4):181-6. doi: 10.1159/000212471.
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Requirement for OKT8+ suppressor cell proliferation for suppression by human newborn T cells.
人新生儿T细胞发挥抑制作用对OKT8 +抑制性细胞增殖的需求。
Clin Exp Immunol. 1981 Sep;45(3):468-74.
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The Hutchinson-Gilford progeria syndrome. Report of 4 cases and review of the literature.哈钦森-吉尔福德早衰综合征。4例报告并文献复习。
J Pediatr. 1972 Apr;80(4):697-724. doi: 10.1016/s0022-3476(72)80229-4.
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Lymphocyte proliferation and nucleoid sedimentation in a case of premature aging distinct from Werner's syndrome.一例不同于沃纳综合征的早衰病例中的淋巴细胞增殖和核仁沉降
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Natural killer (NKH-1+) cell number and activity in health and disease.健康与疾病状态下自然杀伤细胞(NKH-1+)的数量及活性
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