Abstract:
:Natural killer (NK) cell deficiency (NKD) is a subset of primary immunodeficiency disorders (PID) in which an abnormality of NK cells represents a major immunological defect resulting in the patient's clinical immunodeficiency. This is distinct from a much larger group of PIDs that include an NK cell abnormality as a minor component of the immunodeficiency. Patients with NKD most frequently have atypical consequences of herpesviral infections. There are now 6 genes that have been ascribed to causing NKD, some exclusively and others that also cause other known immunodeficiencies. This list has grown in recent years and as such the mechanistic and molecular clarity around what defines an NKD is an emerging and important field of research. Continued increased clarity will allow for more rational approaches to the patients themselves from a therapeutic standpoint. Having evaluated numerous individuals for NKD, I share my perspective on approaching the diagnosis and managing these patients.
journal_name
J Clin Immunoljournal_title
Journal of clinical immunologyauthors
Orange JSdoi
10.1007/s10875-019-00711-7subject
Has Abstractpub_date
2020-01-01 00:00:00pages
13-23issue
1eissn
0271-9142issn
1573-2592pii
10.1007/s10875-019-00711-7journal_volume
40pub_type
杂志文章abstract:INTRODUCTION:Rheumatoid arthritis (RA) is a chronic inflammation disease that may involve extra-articular organs in addition to joints. Many proinflammatory cytokines are involved in the inflammatory process of RA. IkappaBalpha conjugates with NF-kappaB and is a key player in regulation of the inflammatory process. We ...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-010-9439-9
更新日期:2010-09-01 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::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:PURPOSE:Colitis is a common and serious complication of chronic granulomatous disorder (CGD) and requires assessment. Colonoscopy is invasive and carries risks of serious complication. We therefore assessed non-invasive monitoring via magnetic resonance imaging (MRI). We also evaluated fecal calprotectin (FCP), the Har...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-019-00651-2
更新日期:2019-07-01 00:00:00
abstract::The immune system is affected by the aging process and undergoes significant age-related changes, termed immunosenescence. Different T cell subsets are affected by this process. Alterations within the bone marrow and thymus lead to a shift in the composition of the T cell repertoire from naïve to antigen-experienced T...
journal_title:Journal of clinical immunology
pub_type: 杂志文章,评审
doi:10.1007/s10875-010-9499-x
更新日期:2011-04-01 00:00:00
abstract:PURPOSE:Combined immunodeficiency (CID) presents a unique challenge to clinicians. Two patients presented with the prior clinical diagnosis of common variable immunodeficiency (CVID) disorder marked by an early age of presentation, opportunistic infections, and persistent lymphopenia. Due to the presence of atypical cl...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-014-0121-5
更新日期:2015-02-01 00:00:00
abstract::Increased levels of serum IgD can be found in single patients with a variety of clinical syndromes and in the disease entity designated hyper-IgD syndrome which is associated with periodic fever and lymphadenopathy. We investigated 17 patients, both children and adults, with high serum IgD levels ranging from 220 to 5...
journal_title:Journal of clinical immunology
pub_type: 临床试验,杂志文章,多中心研究
doi:10.1007/BF00917104
更新日期:1989-09-01 00:00:00
abstract:PURPOSE:The dysregulated expression of miRNAs in the immune system may be critical for immune responses to pathogens and evolve into the inflammation seen in sepsis. The aim of this study is to explore the important role of miRNAs in the regulation of the immune response during neonatal sepsis. METHODS:Using a microar...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-014-0004-9
更新日期:2014-04-01 00:00:00
abstract:INTRODUCTION:The molecular and cellular interactions between the immune system and bone tissue have been established. Sex hormone deficiency after menopause has multifunctional role by influencing growth, differentiation, and metabolism of the skeletal and the immune system. DISCUSSION:We have used nonparametric and m...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-009-9321-9
更新日期:2009-11-01 00:00:00
abstract::This review describes a series of studies performed in our laboratory which have focused on the activation and subsequent proliferation and differentiation of human B lymphocytes. Utilizing polyclonal signals which activate B cells by interacting with their surface membrane Ig, we have examined the events in the trans...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/BF00917136
更新日期:1984-09-01 00:00:00
abstract::The biological differences between lymphocytes separated from thymoma patients with myasthenia gravis (MG) and those separated from thymoma patients without MG was examined. We investigated whether lymphocytes in thymoma (Th-L) of patients with and without MG could react to interleukin-2 (IL-2) to develop cytolytic ac...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/BF01541224
更新日期:1996-07-01 00:00:00
abstract:PURPOSE:Multiple sclerosis (MS) is an autoimmune disease of the central nervous system mediated by T cells. B7-H3 plays a diverse role in regulating T cell responses. However, its expression and clinical significance in MS are not well known. This study analyzed the expression of membrane B7-H3 (mB7-H3) and levels of s...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-012-9800-2
更新日期:2013-01-01 00:00:00
abstract:OBJECTIVE:The purpose of the study was to determine associations between pre-antiretroviral therapy (ART) senescent CD8+ T lymphocytes and naïve versus non-naive CD8+ and CD4+ T lymphocyte subpopulations and CD4+ responses after initiation of ART in younger versus older individuals. METHODS:Retrospective analysis of 1...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-011-9550-6
更新日期:2011-10-01 00:00:00
abstract:INTRODUCTION:Treating recipient mice with palifermin (recombinant human keratinocyte growth factor) prevents the development of acute, lethal, graft-versus-host disease (GVHD). This is due, at least in part, to the ability of palifermin to protect epithelial cells from injury. Using the C57BL/6-->(C57BL/6 x DBA/2)F(1)-...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-008-9216-1
更新日期:2008-09-01 00:00:00
abstract:PURPOSE:DNA Ligase 4 (LIG4) is a key factor in the non-homologous end-joining (NHEJ) DNA double-strand break repair pathway needed for V(D)J recombination and the generation of the T cell receptor and immunoglobulin molecules. Defects in LIG4 result in a variable syndrome of growth retardation, pancytopenia, combined i...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-016-0266-5
更新日期:2016-05-01 00:00:00
abstract:PURPOSE:T-helper (Th) cells abnormalities are considered to be associated with the pathogenesis of Systemic lupus erythematosus (SLE). Recently, The Th22 cells have been identified and implicated in the pathogenesis of autoimmune diseases such as Rheumatoid arthritis (RA), although therir role in Systemic lupus erythem...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-013-9878-1
更新日期:2013-05-01 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::Deficient DNA mismatch repair results in microsatellite instability and might induce shifts of translational reading frames of genes encompassing coding microsatellites. These may be translated in truncated proteins, including neo-peptide tails functioning as tumor rejection antigens, when presented in the context of ...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1023/a:1025329819121
更新日期:2003-09-01 00:00:00
abstract:BACKGROUND:Although some previous studies have reported that genetic and immunological factors play important roles in the pathogenesis of Kawasaki disease (KD), the etiological factors of this enigmatical pediatric disease are still poorly understood. PURPOSE:This study aims to investigate whether polymorphisms of th...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-008-9203-6
更新日期:2008-09-01 00:00:00
abstract::We report the long-term follow-up of 12 pediatric-aged patients with chronic granulomatous disease (CGD). The mean age at the onset of infections was 5 months with a median delay in diagnosis of 2.5 years. Bacille Calmette-Guérin lymphadenitis was the most common presenting infection (6) followed by suppurative lympha...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1023/a:1021952315651
更新日期:2003-01-01 00:00:00
abstract:PURPOSE:Severe combined immunodeficiency (SCID) is a syndrome of diverse genetic cause characterized by profound deficiencies of T, B and sometimes NK cell function. Non-ablative HLA-identical or rigorously T cell-depleted haploidentical parental bone marrow transplantation (BMT) results in thymus-dependent genetically...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-012-9797-6
更新日期:2013-01-01 00:00:00
abstract:BACKGROUND:Several autoimmune disorders have been linked to adverse pregnancy outcome. IgA deficiency shares many autoimmune traits, but its association with pregnancy outcome is unknown. METHODS:Prospective population-based cohort study in Sweden of 613 mothers with IgA deficiency (IgA levels < .07 g/L) diagnosed in ...
journal_title:Journal of clinical immunology
pub_type: 杂志文章,多中心研究
doi:10.1007/s10875-014-0069-5
更新日期:2014-10-01 00:00:00
abstract::The prevalence of lymphoma in primary immunodeficiency cases and autoimmune diseases, as well as on a background of immunodeficiency following organ transplants, is increasing. The lymphoma treatment success rate is known to be a low prognosis. Our study aimed to emphasize the low survival rates in immunodeficient vs....
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-016-0324-z
更新日期:2016-10-01 00:00:00
abstract::Anesthetic drugs can influence the immune system, particularly granulocyte function. The goal of the present study was to evaluate if lidocaine used for epidural anesthesia during cesarean section can influence neonatal neutrophil chemotaxis. We measured chemotaxis and plasma cord lidocaine and cortisol levels in (A) ...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1023/a:1020583022614
更新日期:1998-05-01 00:00:00
abstract:INTRODUCTION:This study was done to further reveal the role of the innate immune system in celiac disease. METHODS:Dendritic cells were matured from venous blood of patients with active or treated celiac disease and DQ2-DQ8-positive or negative controls. Dendritic cells were treated with a peptic-tryptic digest of gli...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-008-9228-x
更新日期:2009-01-01 00:00:00
abstract::We have examined the primary immune responses, the numbers of total T (T11+) cells, T-helper (T4+) cells, T-suppressor (T8+) cells, and natural killer (NK) (Leu7+) cells, in 118 healthy control subjects and compared the data to those obtained from 20 patients with clinically diagnosed malignant mesothelioma and 375 lo...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/BF00918702
更新日期:1986-05-01 00:00:00
abstract::Human peripheral B lymphocytes were transformed with Epstein-Barr virus and probed with an antiidiotypic antibody made against a human monoclonal autoantibody. Six cell lines were isolated that produced antibodies bearing a common idiotope. Despite the presence of this common idiotope, the antibodies showed antigen-bi...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/BF00916951
更新日期:1988-11-01 00:00:00
abstract::Thymomas can present with a variety of paraneoplastic manifestations, mostly of autoimmune origin, including Good's syndrome when there is associated hypogammaglobulinemia. Although pure red cell aplasia is a recognised complication of thymoma, selective white cell aplasia is very rare, particularly in Good's syndrome...
journal_title:Journal of clinical immunology
pub_type: 杂志文章,评审
doi:10.1007/s10875-014-0014-7
更新日期:2014-04-01 00:00:00
abstract::Routine T cells phenotyping occasionally reveals a CD4+CD8dim T cell subset with an apparently homogeneous dot plot. The aim of this study was to elucidate their immunological significance from analysis of 31 healthy donors, 21 elderly and 220 immune deficient patients. CD4+CD8dim T cells expressed reduced levels of C...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-005-5257-x
更新日期:2005-09-01 00:00:00
abstract:INTRODUCTION:Experimental allergic encephalomyelitis (EAE) is a T cell-mediated autoimmune disease model for multiple sclerosis (MS). We have shown earlier that 15-deoxy-Delta(12,14)-prostaglandin J(2) (15d-PGJ(2)) and curcumin ameliorate EAE by modulating inflammatory signaling pathways in T lymphocytes. Toll-like rec...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-008-9202-7
更新日期:2008-09-01 00:00:00