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Immunodeficiency最新文献

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Combined immunodeficiency (CID) with oligoclonal gammopathy. 联合免疫缺陷(CID)与寡克隆γ病。
Immunodeficiency Pub Date : 1993-01-01
E G Davies, M Sharland, E Kaminski, J Nisbet, R S Periera
{"title":"Combined immunodeficiency (CID) with oligoclonal gammopathy.","authors":"E G Davies, M Sharland, E Kaminski, J Nisbet, R S Periera","doi":"","DOIUrl":"","url":null,"abstract":"","PeriodicalId":79340,"journal":{"name":"Immunodeficiency","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"1993-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"19158268","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
A retrospective single-center study of clinical presentation and outcome in 117 patients with severe combined immunodeficiency. 117例严重联合免疫缺陷患者的临床表现和预后的回顾性单中心研究。
Immunodeficiency Pub Date : 1993-01-01
J L Stephan, V Vlekova, F Le Deist, G De Saint Basile, J Donadieu, A Durandy, S Blanche, C Griscelli, A Fischer
{"title":"A retrospective single-center study of clinical presentation and outcome in 117 patients with severe combined immunodeficiency.","authors":"J L Stephan, V Vlekova, F Le Deist, G De Saint Basile, J Donadieu, A Durandy, S Blanche, C Griscelli, A Fischer","doi":"","DOIUrl":"","url":null,"abstract":"","PeriodicalId":79340,"journal":{"name":"Immunodeficiency","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"1993-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"19158273","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Carrier detection in X-linked immunodeficiencies. I: A PCR-based X chromosome inactivation assay at the MAOA locus. x连锁免疫缺陷的携带者检测。1:基于聚合酶链反应的MAOA位点X染色体失活试验。
Immunodeficiency Pub Date : 1993-01-01
R W Hendriks, Z Y Chen, H Hinds, R K Schuurman, I W Craig
{"title":"Carrier detection in X-linked immunodeficiencies. I: A PCR-based X chromosome inactivation assay at the MAOA locus.","authors":"R W Hendriks,&nbsp;Z Y Chen,&nbsp;H Hinds,&nbsp;R K Schuurman,&nbsp;I W Craig","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>We developed an X chromosome inactivation PCR assay, based on differential methylation of the 5' CpG island of the monoamine oxidase A gene (MAOA), close to a highly polymorphic region just downstream of the first exon. The assay provides a method to determine the carrier status of females from pedigrees with X-linked immunodeficiency diseases (XLID).</p>","PeriodicalId":79340,"journal":{"name":"Immunodeficiency","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"1993-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"19158277","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Analysis of MHC class II transcription in two type III bare lymphocyte syndrome patients of a single family. 同一家族2例III型裸淋巴细胞综合征患者MHCⅱ类转录分析。
Immunodeficiency Pub Date : 1993-01-01
M Lambert, M Van Eggermond, C Verheij, J Van Ree, P Van den Elsen
{"title":"Analysis of MHC class II transcription in two type III bare lymphocyte syndrome patients of a single family.","authors":"M Lambert,&nbsp;M Van Eggermond,&nbsp;C Verheij,&nbsp;J Van Ree,&nbsp;P Van den Elsen","doi":"","DOIUrl":"","url":null,"abstract":"","PeriodicalId":79340,"journal":{"name":"Immunodeficiency","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"1993-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"19158293","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Functional T cell immunodeficiency characterized by defective TCR/CD3 induced tyrosylphosphorylation. 以TCR/CD3缺陷诱导酪氨酸磷酸化为特征的功能性T细胞免疫缺陷。
Immunodeficiency Pub Date : 1993-01-01
C Hivroz, F Le Deist, H A Buc, C Griscelli, A Fischer
{"title":"Functional T cell immunodeficiency characterized by defective TCR/CD3 induced tyrosylphosphorylation.","authors":"C Hivroz,&nbsp;F Le Deist,&nbsp;H A Buc,&nbsp;C Griscelli,&nbsp;A Fischer","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Defective T cell activation has been recognized in a number of patients with primary immunodeficiencies (ID). A distal defect resulting in abnormal production of IL2, IL3, IL4 and IFN gamma was found to be associated with reduced binding of the NF-AT transcription factor to the promoters of the genes coding for these lymphokines. Defect in early steps of T cell activation have been described in primary IDs, consisting in defective calcium flux and phosphoinositides turnover following TCR/CD3 ligation. We herein report a primary ID found in a 13 year old girl. It consists in a partially defective T cell proliferation which could be related to a defective Tyrosine phosphorylation.</p>","PeriodicalId":79340,"journal":{"name":"Immunodeficiency","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"1993-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"19158481","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Is the IFN-gamma-induced enhancement of superoxide production in CGD-phagocytes caused by increased expression of the p47-phox cytosolic protein. 是否ifn - γ诱导的cgd吞噬细胞超氧化物生成的增强是由p47-phox细胞质蛋白表达增加引起的?
Immunodeficiency Pub Date : 1993-01-01
V Jendrossek, U Damm, J Liese, B H Belohradsky, M Gahr
{"title":"Is the IFN-gamma-induced enhancement of superoxide production in CGD-phagocytes caused by increased expression of the p47-phox cytosolic protein.","authors":"V Jendrossek,&nbsp;U Damm,&nbsp;J Liese,&nbsp;B H Belohradsky,&nbsp;M Gahr","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Interferon-gamma (IFN-gamma) had been shown to increase superoxide production of cultured monocytes from patients with chronic granulomatous disease (CGD). To elucidate the mechanism of the IFN-gamma-induced improvement of superoxide production of cultured monocytes from patients with CGD we examined the influence of IFN-gamma on the expression and the activity of the NADPH-oxidase in the monocytic cell-line Mono Mac 6. After cultivation of Mono Mac 6-cells in the presence of 500 U/ml IFN-gamma the superoxide production was found to be increased as well as the expression of the p47-phox cytosolic protein of the phagocytic NADPH-oxidase.</p>","PeriodicalId":79340,"journal":{"name":"Immunodeficiency","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"1993-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"19159051","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Cell-mediated immunity and immunosuppression in herpes simplex virus infection. 单纯疱疹病毒感染的细胞介导免疫和免疫抑制。
Immunodeficiency Pub Date : 1993-01-01
C R Rinaldo, D J Torpey
{"title":"Cell-mediated immunity and immunosuppression in herpes simplex virus infection.","authors":"C R Rinaldo,&nbsp;D J Torpey","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Innate and adaptive forms of cellular immunity have important, interactive roles in host resistance to herpes simplex virus (HSV) infection. Hence, suppression of non-HSV specific and anti-HSV specific cellular immune responses can predispose the host to severe HSV infection. Studies using depletion and adoptive transfer of selected subpopulations of NK cells, macrophages, and CD4+ and CD8+ T lymphocytes indicate that each of these is of significance in protection against infection with HSV. Further evidence suggests that cytokines such as interferons alpha and gamma, interleukin 2 and leukocyte migration inhibition factor also have central roles in these cell functions during HSV infection. Of importance is that HSV itself can result in transient suppression of several innate and adaptive cellular immune responses during acute episodes of infection in normal adults. Mechanisms by which HSV may mediate this immune dysfunction include enhanced activity of suppressor T cells and soluble suppressor factors, decreases in cytokine production, decreases in expression of major and minor histocompatibility antigens and direct inhibition of cytotoxic effector cell function. Knowledge of anti-HSV cellular immunity and of the immunosuppressive properties of HSV are of importance in the development of appropriate treatment and vaccine strategies for this herpesvirus.</p>","PeriodicalId":79340,"journal":{"name":"Immunodeficiency","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"1993-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"19160185","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Molecular analysis of X-linked immunodeficiency with hyper-IgM and X-linked lymphoproliferative syndrome. x连锁免疫缺陷伴高igm和x连锁淋巴细胞增生性综合征的分子分析。
Immunodeficiency Pub Date : 1993-01-01
L D Notarangelo, E Mantuano, S Bione, E Gimbo, S Giliani, A Caraffini, D Purtilo, C Farr, A G Ugazio, D Toniolo
{"title":"Molecular analysis of X-linked immunodeficiency with hyper-IgM and X-linked lymphoproliferative syndrome.","authors":"L D Notarangelo,&nbsp;E Mantuano,&nbsp;S Bione,&nbsp;E Gimbo,&nbsp;S Giliani,&nbsp;A Caraffini,&nbsp;D Purtilo,&nbsp;C Farr,&nbsp;A G Ugazio,&nbsp;D Toniolo","doi":"","DOIUrl":"","url":null,"abstract":"","PeriodicalId":79340,"journal":{"name":"Immunodeficiency","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"1993-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"19158281","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Intestinal immunoglobulin production after oral cholera vaccination in patients with IgA deficiency with or without IgG subclass deficiency. 伴有或不伴有IgG亚类缺乏的IgA缺乏患者口服霍乱疫苗接种后肠道免疫球蛋白的产生。
Immunodeficiency Pub Date : 1993-01-01
D E Nilssen, V Friman, K Theman, J Björkander, A Kilander, J Holmgren, L A Hanson, P Brandtzaeg
{"title":"Intestinal immunoglobulin production after oral cholera vaccination in patients with IgA deficiency with or without IgG subclass deficiency.","authors":"D E Nilssen,&nbsp;V Friman,&nbsp;K Theman,&nbsp;J Björkander,&nbsp;A Kilander,&nbsp;J Holmgren,&nbsp;L A Hanson,&nbsp;P Brandtzaeg","doi":"","DOIUrl":"","url":null,"abstract":"","PeriodicalId":79340,"journal":{"name":"Immunodeficiency","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"1993-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"19158265","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Safety of rapid subcutaneous gammaglobulin infusions in patients with primary antibody deficiency. 一抗缺乏患者快速皮下注射丙种球蛋白的安全性。
Immunodeficiency Pub Date : 1993-01-01
A Gardulf, H Björvell, R Gustafson, L Hammarström, C I Smith
{"title":"Safety of rapid subcutaneous gammaglobulin infusions in patients with primary antibody deficiency.","authors":"A Gardulf,&nbsp;H Björvell,&nbsp;R Gustafson,&nbsp;L Hammarström,&nbsp;C I Smith","doi":"","DOIUrl":"","url":null,"abstract":"","PeriodicalId":79340,"journal":{"name":"Immunodeficiency","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"1993-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"18520235","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
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