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对威尔逊氏病患者的免疫学观察。

Immunological observations on patients with Wilson's disease.

作者信息

Czlonkowska A, Milewski B

出版信息

J Neurol Sci. 1976 Oct;29(2-4):411-21. doi: 10.1016/0022-510x(76)90188-x.

DOI:10.1016/0022-510x(76)90188-x
PMID:978218
Abstract

In 19 patients with Wilson's disease we found an increased humoral immune response, i.e. a higher level of IgG and IgM, a higher titre of antibodies against Kunin's CA antigen and a depressed cell-mediated immunity i.e. a lower response to DNCB and E. coli in skin tests, lower lymphocyte transformation when stimulated by Con A, PPD, Candida albicans and streptokinase and a lower production of macrophage migration inhibition factor. The changes observed in the group of patients with liver cirrhosis caused by other facotrs than Wilson's disease were similar but less pronounced. We also found that leukocytes of patients with Wilson's disease have an impaired bactericidal activity and that copper ions have an inhibitory effect on some tests for cell-mediated immunity. It seems probable that immunological abnormalities in Wilson's disease are caused by liver cirrhosis but we cannot exclude an inhibitory effect of copper ions upon the immune response and an associated effect upon leukocyte metabolism.

摘要

在19例威尔逊病患者中,我们发现其体液免疫反应增强,即IgG和IgM水平升高、抗库宁CA抗原的抗体滴度升高,而细胞介导免疫功能低下,即皮肤试验中对二硝基氯苯和大肠杆菌的反应较低、在刀豆蛋白A、结核菌素纯蛋白衍生物、白色念珠菌和链激酶刺激下淋巴细胞转化率较低以及巨噬细胞移动抑制因子产生减少。在由威尔逊病以外的其他因素引起肝硬化的患者组中观察到的变化相似,但不太明显。我们还发现,威尔逊病患者的白细胞杀菌活性受损,并且铜离子对某些细胞介导免疫试验有抑制作用。威尔逊病中的免疫异常似乎很可能是由肝硬化引起的,但我们不能排除铜离子对免疫反应的抑制作用以及对白细胞代谢的相关影响。

相似文献

1
Immunological observations on patients with Wilson's disease.对威尔逊氏病患者的免疫学观察。
J Neurol Sci. 1976 Oct;29(2-4):411-21. doi: 10.1016/0022-510x(76)90188-x.
2
The influence of prolonged treatment with D-penicillamine on the immune response in Wilson's disease.D-青霉胺长期治疗对威尔逊病免疫反应的影响。
Eur J Clin Pharmacol. 1977 Dec 2;12(4):265-71. doi: 10.1007/BF00607425.
3
[T-rosette test in Wilson's disease].[威尔逊病的T-玫瑰花结试验]
Neurol Neurochir Pol. 1975 Jul-Aug;9(4):441-6.
4
The effect of long-term treatment with d-penicillamine on the humoral immune response in patients with Wilson's disease.青霉胺长期治疗对威尔逊病患者体液免疫反应的影响。
Mater Med Pol. 1976 Jan-Mar;8(1):48-50.
5
[The onset of psychiatric disorders and Wilson's disease].[精神疾病与威尔逊氏病的发病]
Encephale. 2007 Dec;33(6):924-32. doi: 10.1016/j.encep.2006.08.009. Epub 2007 Sep 5.
6
Use of the stable isotope 65Cu test for the screening of Wilson's disease in a family with two affected members.使用稳定同位素65Cu检测对一个有两名患病成员的家庭进行威尔逊病筛查。
Ital J Gastroenterol Hepatol. 1998 Jun;30(3):270-5.
7
[The detection of antibodies against D-penicillamine. 3. Detection of antibodies against D-penicillamine in the serum of patients with Wilson's disease treated with D-penicillamine--preliminary results].
Pharmazie. 1986 Feb;41(2):126-7.
8
[Wilson's disease or hepatolenticular degeneration].[威尔逊病或肝豆状核变性]
Ther Umsch. 2018 Nov;75(4):241-248. doi: 10.1024/0040-5930/a000995.
9
[Serum immunoglobulin level in patients with Wilson's disease].[威尔逊病患者的血清免疫球蛋白水平]
Neurol Neurochir Pol. 1974 Sep-Oct;8(5):663-7.
10
[Cytochemical studies of peripheral white blood cells in Wilson's disease].[肝豆状核变性患者外周血白细胞的细胞化学研究]
Neurol Neurochir Pol. 1977 Mar-Apr;11(2):159-63.

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World J Hepatol. 2024 Aug 27;16(8):1111-1119. doi: 10.4254/wjh.v16.i8.1111.
2
Wilson's Disease-Crossroads of Genetics, Inflammation and Immunity/Autoimmunity: Clinical and Molecular Issues.威尔逊氏病——遗传学、炎症和免疫/自身免疫的交汇点:临床和分子问题。
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D-Penicillamine-Induced Myasthenia Gravis-A Probable Complication of Wilson's Disease Treatment-A Case Report and Systematic Review of the Literature.青霉胺诱发的重症肌无力——威尔逊病治疗可能出现的并发症——病例报告及文献系统综述
Life (Basel). 2023 Aug 10;13(8):1715. doi: 10.3390/life13081715.
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Prevalence and Significance of Autoantibody Seropositivity in Children with Wilson's Disease.肝豆状核变性患儿自身抗体血清阳性的患病率及意义
Diagnostics (Basel). 2023 Feb 17;13(4):768. doi: 10.3390/diagnostics13040768.
6
Autoantibodies in Wilson disease: Impact on clinical course.威尔逊病中的自身抗体:对临床病程的影响。
JIMD Rep. 2022 Jul 22;63(5):508-517. doi: 10.1002/jmd2.12317. eCollection 2022 Sep.
7
Epigenomic signatures in liver and blood of Wilson disease patients include hypermethylation of liver-specific enhancers.威尔逊病患者肝脏和血液中的表观基因组特征包括肝脏特异性增强子的过度甲基化。
Epigenetics Chromatin. 2019 Feb 1;12(1):10. doi: 10.1186/s13072-019-0255-z.
8
Concomitant immune-related events in Wilson disease: implications for monitoring chelator therapy.威尔逊病中的伴随免疫相关事件:对螯合剂治疗监测的意义
J Inherit Metab Dis. 2016 Jan;39(1):125-30. doi: 10.1007/s10545-015-9866-0. Epub 2015 Jun 12.
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Copper and anesthesia: clinical relevance and management of copper related disorders.铜与麻醉:铜相关疾病的临床相关性及管理
Anesthesiol Res Pract. 2013;2013:750901. doi: 10.1155/2013/750901. Epub 2013 May 13.
10
Wilson's disease-cause of mortality in 164 patients during 1992-2003 observation period.威尔逊氏病——1992年至2003年观察期内164例患者的死亡原因。
J Neurol. 2005 Jun;252(6):698-703. doi: 10.1007/s00415-005-0720-4. Epub 2005 Mar 2.