Abstract:
:Chronic Granulomatous Disease (CGD) is a rare primary immunodeficiency due to a defect in one of the NADPH oxidase complex subunits; 70 % of cases are X-linked, due to a CYBB mutation, resulting in defective production of gp91PHOX. Female carriers of X-linked CGD have previously been considered to be unaffected. It is increasingly recognized that they may suffer from similar problems to CGD patients. This review will examine the literature about clinical manifestations of disease in X-linked carriers of CGD.
journal_name
J Clin Immunoljournal_title
Journal of clinical immunologyauthors
Battersby AC,Cale AM,Goldblatt D,Gennery ARdoi
10.1007/s10875-013-9939-5subject
Has Abstractpub_date
2013-11-01 00:00:00pages
1276-84issue
8eissn
0271-9142issn
1573-2592journal_volume
33pub_type
杂志文章,评审abstract:PURPOSE:Poikiloderma with neutropenia (PN) is a genodermatosis currently described in 77 patients, all presenting with early-onset poikiloderma, neutropenia, and several additional signs. Biallelic loss-of-function mutations in USB1 gene are detected in all molecularly tested patients but genotype-phenotype correlation...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-018-0508-9
更新日期:2018-05-01 00:00:00
abstract::Augmentation of the cytolytic activity of human natural killer cells and of antibody-dependent cell-mediated cytotoxicity has been attributed to human interferons. With the purification to homogeneity of human leukocyte interferon, it became possible to test directly whether pure interferon could increase the activity...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/BF00922756
更新日期:1981-07-01 00:00:00
abstract::We examined sera from 160 HIV-infected individuals for antibodies reactive to HIV-1 gp160 epitopes defined by seven synthetic peptides. Seropositive individuals were placed into three groups based upon levels of circulating CD4+ cells. These groups consisted of individuals with (1) more than 400 CD4+ cells, (2) 200-40...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/BF00918790
更新日期:1991-01-01 00:00:00
abstract::Alloimmune diseases can occur in pregnancy and after blood transfusions, where antibodies are formed, targeting foreign cells and tissues for destruction by myeloid cells through IgG Fc-receptors (FcγR). In pregnancy, antibodies against human blood group or platelet antigens (e.g. HPA1-a) cause life-threatening anemia...
journal_title:Journal of clinical immunology
pub_type: 杂志文章,评审
doi:10.1007/s10875-016-0253-x
更新日期:2016-05-01 00:00:00
abstract::Rat T lymphoblasts arrested in the G1 phase of the cell cycle by interleukin-2 (IL-2) deprivation can be forced to proceed to the S phase when they are stimulated with IL-2 or the phorbol ester phorbol 12,13-dibutyrate (PDBu). When PDBu is used as a stimulus, extracellular regulated kinase 2 (ERK2) is activated by thr...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1023/a:1027375828134
更新日期:1997-11-01 00:00:00
abstract::A 20-year health follow-up study of 159 initially healthy blood donors with a severe deficiency of serum IgA ( < 0.05 x 10(-3) g/L) and of 45 donors with decreased serum IgA (0.05 x 10(-3)-0.8 g/L) was carried out. The findings indicate that persons with a severe deficiency of and decreased serum IgA who are healthy a...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/BF01540915
更新日期:1996-05-01 00:00:00
abstract::Intravenous immunoglobin (i.v.IG) is generated from the plasma of more than 10,000 healthy subjects. Originally i.v.IG was employed to supplement the missing Igs in subjects with immune-deficiencies. Later on it was given successfully to patients with autoimmune conditions, in some of which the benefits were confirmed...
journal_title:Journal of clinical immunology
pub_type: 杂志文章,评审
doi:10.1023/b:joci.0000019809.55787.ec
更新日期:2004-03-01 00:00:00
abstract:PURPOSE:The specific antibody response to the unconjugated 23-valent pneumococcal polysaccharide vaccine is one of the most common tests used to assess for possible humoral immunodeficiency. The results can be difficult to interpret because most people have been immunized with one or more of the pneumococcal vaccines a...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-017-0406-6
更新日期:2017-07-01 00:00:00
abstract::Administration of a tumor necrosis factor (TNF) inhibitor-encoding adenoviral vector decreases the severity of colonic inflammation in a DBA/2-->B6D2F1 murine model of colonic graft-versus-host disease (GVHD). The present studies evaluated the effect of TNF blockade on the splenic and colonic T-cell responses. cDNA en...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1023/a:1006676315093
更新日期:2000-09-01 00:00:00
abstract::Murine B16 melanoma cell line is poorly immunogenic and highly aggressive. We recently reported that the transmembrane staphylococcal enterotoxin A (TM-SEA) anchors onto B16 cells and stimulates lymphocyte proliferation. The purpose of the study was to investigate whether vaccination with B16 cells bearing membrane-an...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1023/B:JOCI.0000025451.41948.94
更新日期:2004-05-01 00:00:00
abstract::Treatment of primary immunodeficiency (PI) is typically initiated with intravenous immunoglobulin (IVIG) loading and then continued with IVIG or subcutaneous IgG (SCIG). This prospective, open-label, multicenter, 6-month study evaluated a new regimen of initiating IgG therapy with SCIG in 18 previously untreated patie...
journal_title:Journal of clinical immunology
pub_type: 临床试验,杂志文章,多中心研究
doi:10.1007/s10875-011-9588-5
更新日期:2011-12-01 00:00:00
abstract::Matrix metalloproteinases (also named matrixin or MMPs) are a major group of enzymes that regulate cell-matrix composition by using zinc for their proteolytic activities. They are essential for various normal biological processes such as embryonic development, morphogenesis, reproduction tissue resorption, and remodel...
journal_title:Journal of clinical immunology
pub_type: 杂志文章,评审
doi:10.1007/s10875-006-9022-6
更新日期:2006-07-01 00:00:00
abstract::This study shows that specific humoral immunity could be transferred from marrow donors to marrow recipients. Peripheral blood lymphocytes from long-term human marrow recipients produced IgG anti-tetanus toxoid antibody after in vitro tetanus toxoid stimulation. Antitetanus toxoid antibody biosynthesis was induced usi...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/BF00915378
更新日期:1986-09-01 00:00:00
abstract:PURPOSE:The present study was designed to evaluate the efficacy and safety of a novel, 10% liquid formulation of intravenous immunoglobulin, stabilized with 250 mmol/L L-proline (Privigen), in patients with primary immunodeficiency disease. MATERIALS AND METHODS:Eighty adults and children diagnosed with common variabl...
journal_title:Journal of clinical immunology
pub_type: 杂志文章,多中心研究
doi:10.1007/s10875-008-9231-2
更新日期:2009-01-01 00:00:00
abstract::Rapidly progressive glomerulonephritis (RPGN) is characterized by rapid and progressive loss of renal function and the presence of crescentic glomerulonephritis (CGN). Early diagnosis and appropriate treatment is mandatory to prevent death and/or renal failure. We have evaluated an ANCA-GBM dot-blot diagnostic test in...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1023/B:JOCI.0000029115.79266.ae
更新日期:2004-07-01 00:00:00
abstract::The aim of this study was to research the expression of IL-37 in systemic lupus erythematosus (SLE) patients and the effect of glucocorticoid on IL-37. Thirty newly diagnosed severe SLE patients receiving prednisone 1 mg/kg/day for 14 consecutive days and 30 healthy subjects were enrolled into this study. The plasma l...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-012-9791-z
更新日期:2013-01-01 00:00:00
abstract::The spectrum of illness attributed to Epstein-Barr virus (EBV) includes patients with symptoms persisting for more than 1 year without any other obvious underlying disease. High titers of antibodies to EBV, either IgG antiviral capsid antigen or anti-early antigen, can be demonstrated. In this study, 13 patients diagn...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/BF00915168
更新日期:1985-01-01 00:00:00
abstract::The concentrations of seven immunoglobulin isotypes (IgA, IgE, IgM, IgG1, IgG2, IgG3, and IgG4) were measured in the sera of 207 Finnish blood donors, and they were allotyped with anti-Gm antibodies: anti-f, anti-a, anti-x, and anti-n. The above population could be divided into 12 phenotypes, and significant differenc...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/BF00918793
更新日期:1991-01-01 00:00:00
abstract::Aging represents a complex remodeling in which both innate and adaptive immunities deteriorate. Age-related changes in humoral immunity are responsible for the reduced vaccine responses observed in elderly individuals. Although T cell alterations play a significant role in age-related humoral immune changes, alteratio...
journal_title:Journal of clinical immunology
pub_type: 杂志文章,评审
doi:10.1007/s10875-010-9501-7
更新日期:2011-06-01 00:00:00
abstract:PURPOSE:Tumor necrosis factor alpha-induced protein 3 (TNFAIP3, A20) is a negative regulator of the nuclear factor-κB (NF-κB) pathway. It has recently been recognized that TNFAIP3 deficiency leads to early onset of autoinflammatory and autoimmune syndrome resembling Behçet's disease. Here, we report a novel mutation in...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-019-00604-9
更新日期:2019-02-01 00:00:00
abstract::COX2 (prostaglandin G/H synthase, PTGS2) is a well-validated target in the fields of both oncology and inflammation. Despite their significant toxicity profile, non-steroidal anti-inflammatory drugs (NSAIDs) have become standard of care in the treatment of many COX2-mediated inflammatory conditions. In this report, we...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-007-9070-6
更新日期:2007-03-01 00:00:00
abstract:PURPOSE:Although many studies have investigated Mendelian susceptibility to mycobacterial disease (MSMD) worldwide, there is no report of the long-term clinical management and prognosis for MSMD in China. METHODS:This is a cohort study from January 2000 to June 2018. Three hundred and twenty-four patients with bacillu...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-019-00672-x
更新日期:2019-08-01 00:00:00
abstract::Autism is a complex polygenic neurodevelopmental disorder characterized by deficits in communication and social interactions as well as specific stereotypical behaviors. Both genetic and environmental factors appear to contribute to the pathogenesis of autism. Accumulating data including changes in immune responses, l...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-010-9402-9
更新日期:2010-05-01 00:00:00
abstract::Recombinant human erythropoietin (rhEPO) treatment of hemodialyzed (HD) patients normalizes the altered phenotype of CD4(+) lymphocytes and restores the balance of Th1/Th2 cytokines. We decided to test how the presence of rhEPO in cell culture modulates cytokine production of CD4(+) lymphocytes in HD patients with sta...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-012-9835-4
更新日期:2013-04-01 00:00:00
abstract::Early treatment intervention during human immunodeficiency virus (HIV) infection is a strategy aimed to preserve and/or enhance the developing anti-HIV immune responses. We report the effect of highly active antiretroviral therapy (HAART) combined with intermittent subcutaneous doses of Interleukin 2 (IL-2) on CD8(+) ...
journal_title:Journal of clinical immunology
pub_type: 临床试验,杂志文章
doi:10.1023/B:JOCI.0000019778.96564.26
更新日期:2004-03-01 00:00:00
abstract::We studied immunological functions of peripheral blood lymphocytes (PBL) from human T-cell leukemia virus type I (HTLV-I)-seropositive healthy carriers in vitro. Proliferative responses of PBL to T-cell and B-cell mitogens such as concanavalin A (Con A), pokeweed mitogen (PWM), and Staphylococcus aureus Cowan I (SAC) ...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/BF00918017
更新日期:1989-11-01 00:00:00
abstract:BACKGROUND:Chronic granulomatous disease (CGD) is characterized by mutation in any one of the five genes coding NADPH oxidase components that leads to functional abnormality preventing the killing of phagocytosed microbes by affecting the progression of a respiratory burst. CGD patients have an increased susceptibility...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-018-0567-y
更新日期:2018-11-01 00:00:00
abstract::Murine hematopoietic tissues contain cells which, upon injection into lethally irradiated mice, produce nodules on the surface of their spleen (colony-forming unit--spleen; CFU-S). The exact hierarchical level of the hematopoietic progenitors which give rise to CFU-S is not fully established; however, cell populations...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1023/a:1027302310505
更新日期:1997-05-01 00:00:00
abstract::Lymphocytes expressing gammadelta T cell receptors (TCR) constitute an entire system of functionally specialized subsets that have been implicated in the regulation of immune responses, including responses to pathogens and allergens, and in tissue repair. The gammadelta TCRs share structural features with adaptive rec...
journal_title:Journal of clinical immunology
pub_type: 杂志文章,评审
doi:10.1007/s10875-007-9077-z
更新日期:2007-03-01 00:00:00
abstract::Although CpG methylation is thought to be a negative regulator of gene transcription, its relationship with cytokine expression remains unclear. Interleukin (IL)-4 and interferon (IFN)-gamma are major cytokines that affect the differentiation of naïve CD4+ T lymphocytes into the Th1 and Th2 lineage. We used bisulfite ...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-007-9148-1
更新日期:2008-03-01 00:00:00