Kostmann's Disease and HCLS1-Associated Protein X-1 (HAX1).

abstract：:Light chain amyloidosis (AL) is a protein folding disorder with an underlying B cell neoplasia where the monoclonal immunoglobulin light chains (LCs) produced from insoluble amyloid fibrils. The deposition of these fibrillar aggregates in vital organs causes severe organ dysfunction over time and is associated with hi...

journal_title：Journal of clinical immunology

authors： Abraham RS,Geyer SM,Ramírez-Alvarado M,Price-Troska TL,Gertz MA,Fonseca R

更新日期：2004-07-01 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

authors： Brown GR,Thiele DL

更新日期：2000-09-01 00:00:00

abstract：:Chronic Granulomatous Disease (CGD), caused by genetic defects in components of the phagocyte NADPH oxidase pathway, leads to recurrent life-threatening bacterial and invasive fungal infections. While a number of unique pathogens have been associated with this disease, the causative organisms may be difficult to ident...

journal_title：Journal of clinical immunology

authors： Ramesh M,Resnick E,Hui Y,Maglione PJ,Mehta H,Kattan J,Bouvier NM,LaBombardi V,Victor TR,Chaturvedi S,Cunningham-Rundles C

更新日期：2014-02-01 00:00:00

abstract：:Treatment decisions made in clinical practice, based on current guidelines, often conflict with decisions by third-party payors that restrict the ability of patients with primary immunodeficiency disease (PI) to adhere to appropriate treatment. This is seen by many physicians as potentially placing the health of patie...

journal_title：Journal of clinical immunology

authors： Ballow M

更新日期：2012-09-01 00:00:00

abstract：:The cellular and molecular basis of immune senescence is unclear. A number of mechanisms have been proposed. In this issue of the Journal of Clinical Immunology, some of the mechanisms for various immunologic abnormalities in aging are presented. In this article, various molecular steps of both death receptor and mito...

journal_title：Journal of clinical immunology

authors： Gupta S

更新日期：2000-07-01 00:00:00

abstract：:Antibodies to certain self-antigens are detected in normal individuals as well as in patients with autoimmune diseases. Natural autoantibodies found in normal human sera are thought to act as an immune regulator, a functional controller of specific proteins, or the first-line defense against pathogenic microorganisms....

journal_title：Journal of clinical immunology

authors： Suk K,Hwang DY,Lee MS

更新日期：1999-05-01 00:00:00

abstract：PURPOSE:Home-based subcutaneous immunoglobulin (SCIg) therapy is an alternative to hospital-based intravenous infusions (IVIg). However, SCIg requires patient training and long-term support to ensure proper adherence, optimal efficacy and safety. We evaluated if switching patients to home-based SCIg including an interp...

journal_title：Journal of clinical immunology

authors： Perraudin C,Bourdin A,Spertini F,Berger J,Bugnon O

更新日期：2016-07-01 00:00:00

abstract：:Hematopoietic stem cell transplantation (HSCT) is curative for severe combined immunodeficiency (SCID), but data on long-term impact of pre-HSCT chemotherapy, immune reconstitution and quality of life (QoL) of specific SCID genotypes are limited. We evaluated the long-term immune-reconstitution, health outcome and QoL...

journal_title：Journal of clinical immunology

authors： Abd Hamid IJ,Slatter MA,McKendrick F,Pearce MS,Gennery AR

更新日期：2018-08-01 00:00:00

abstract：:Atopic dermatitis in humans and dogs is a chronic relapsing allergic skin disease. Dogs show a spontaneous disease similar to the human counterpart and represent a model to improve our understanding of the immunological mechanisms, the pathogenesis of the disease, and new therapy development. The aim of the study was ...

journal_title：Journal of clinical immunology

authors： Ricklin ME,Roosje P,Summerfield A

更新日期：2010-11-01 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

authors： Buchbinder D,Baker R,Lee YN,Ravell J,Zhang Y,McElwee J,Nugent D,Coonrod EM,Durtschi JD,Augustine NH,Voelkerding KV,Csomos K,Rosen L,Browne S,Walter JE,Notarangelo LD,Hill HR,Kumánovics A

更新日期：2015-02-01 00:00:00

abstract：PURPOSE:Hemophagocytic lymphohistiocytosis (HLH) is a rare systematic immune disease manifested with excessive activation of lymphocytes and macrophages. This study was designed to explore the feasible prognostic factors of secondary HLH (sHLH). METHOD:We retrospectively analyzed 179 patients with newly diagnosed sHLH...

journal_title：Journal of clinical immunology

authors： Huang J,Yin G,Duan L,Tian T,Xu J,Wang J,Gao X,Cheng W,Liu L,Qiu H

更新日期：2020-07-01 00:00:00

abstract：:The use of exogenous serum to provide protection against infections began more than a century ago. Over time, this concept matured and led to the preparation of concentrated immunoglobulin (IgG) products that were safe and effective when delivered subcutaneously (SC) or intramuscularly (IM) but were not ideal for intr...

journal_title：Journal of clinical immunology

authors： Torgerson TR

更新日期：2013-01-01 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

authors： Arnold CR,Wolf J,Brunner S,Herndler-Brandstetter D,Grubeck-Loebenstein B

更新日期：2011-04-01 00:00:00

abstract：PURPOSE:Mendelian susceptibility to mycobacterial disease (MSMD) is a rare primary immunodeficiency, triggered by non-tuberculous mycobacteria or Bacillus Calmette-Guérin (BCG) vaccines and characterized by severe diseases. All known genetic etiologies are inborn errors of IFN-γ-mediated immunity. Here, we report the m...

journal_title：Journal of clinical immunology

authors： Sarrafzadeh SA,Nourizadeh M,Mahloojirad M,Fazlollahi MR,Shokouhi Shoormasti R,Badalzadeh M,Deswarte C,Casanova JL,Pourpak Z,Bustamante J,Moin M

更新日期：2019-04-01 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

authors： Hoffman RM,Jamieson BD,Bosch RJ,Currier J,Kitchen CM,Schmid I,Zhu Y,Bennett K,Mitsuyasu R

更新日期：2011-10-01 00:00:00

abstract：:Prior to the advent of cardiac bypass, most children with congenital cardiac anomalies and chromosome 22q11.2 deletion syndrome died. With improved technology, there is now a wave of young adults with chromosome 22q11.2 deletion syndrome requiring clinical care. Fifteen young children and 20 adults with chromosome 22q...

journal_title：Journal of clinical immunology

authors： Jawad AF,Prak EL,Boyer J,McDonald-McGinn DM,Zackai E,McDonald K,Sullivan KE

更新日期：2011-12-01 00:00:00

abstract：:The human monoclonal autoantibody 16/6 is a common anti-DNA idiotype found to have clinical relevance in patients with systemic lupus erythematosus (SLE). Therefore the ability of peripheral blood T cells of SLE patients and healthy controls to proliferate and to produce helper T-cell factors following stimulation wit...

journal_title：Journal of clinical immunology

authors： Mendlovic S,Shoenfeld Y,Bakimer R,Segal R,Dayan M,Mozes E

更新日期：1988-05-01 00:00:00

abstract：:Bias in cytokine responses has been proposed as a contributing mechanism to pathogenesis in persistent HIV or hepatitis C virus (HCV) infections. We investigated whether coinfection with HCV modifies the profile of antigen-specific cytokine secretion in women persistently infected with HIV compared to women with singl...

journal_title：Journal of clinical immunology

authors： Villacres MC,Literat O,Degiacomo M,Du W,La Rosa C,Diamond DJ,Kovacs A

更新日期：2005-03-01 00:00:00

abstract：:Various factors seem to be etiologic in the susceptibility to sinopulmonary infections in ataxia-telangiectasia (A-T) patients, i.e., low serum and salivary IgA, low serum IgG2, and even aspiration of saliva. S. pneumoniae is a common pathogen responsible from pulmonary infections and impaired antibody response to pol...

journal_title：Journal of clinical immunology

authors： Sanal O,Ersoy F,Yel L,Tezcan I,Metin A,Ozyürek H,Gariboglu S,Fikrig S,Berkel AI,Rijkers GT,Zegers BJ

更新日期：1999-09-01 00:00:00

abstract：:This phase III, open-label, multi-centre study investigated the efficacy, safety, pharmacokinetics and quality of life impact of Evogam(®), a new chromatographically fractionated 16% subcutaneous immunoglobulin, utilising a 1:1 dose transition ratio from previous immunoglobulin therapy. Thirty-five previously treated ...

journal_title：Journal of clinical immunology

authors： Empson MB,Tang ML,Pearce LK,Rozen L,Gold MS,Katelaris CH,Langton D,Smart J,Smith WB,Steele RH,Ziegler JB,Maher D

更新日期：2012-10-01 00:00:00

abstract：:Using fresh whole blood or isolated lymphocytes, the activity of in vivo generated cytotoxic T-lymphocytes (CTL) was measured as the OKT3-specific lysis of HL-60 targets, in a cross-sectional study of 53 HIV (+) patients. CTL activity in the entire HIV(+) group was two to three times higher than in HIV(-) controls, wi...

journal_title：Journal of clinical immunology

authors： Vanham G,Kestens L,Penne G,Goilav C,Gigase P,Colebunders R,Vandenbruaene M,Goeman J,van der Groen G,Ceuppens JL

更新日期：1991-11-01 00:00:00

abstract：INTRODUCTION:Systemic lupus erythematosus (SLE) is a systemic autoimmune disease associated with aberrant activation of T and B lymphocytes. Abnormal activation of intracellular signaling molecules in lymphocytes by inflammatory cytokines can instigate the inflammation in SLE. MATERIALS AND METHODS:The activation of e...

journal_title：Journal of clinical immunology

authors： Wong CK,Wong PT,Tam LS,Li EK,Chen DP,Lam CW

更新日期：2009-11-01 00:00:00

abstract：INTRODUCTION:Autoantibodies to granulocyte-macrophage colony-stimulating factor (GM-CSF) can cause acquired pulmonary alveolar proteinosis (PAP). Cases of acquired PAP susceptible to typical respiratory pathogens and opportunistic infections have been reported. Anti-GM-CSF autoantibodies have been reported in a few pat...

journal_title：Journal of clinical immunology

authors： Kuo CY,Wang SY,Shih HP,Tu KH,Huang WC,Ding JY,Lin CH,Yeh CF,Ho MW,Chang SC,He CY,Chen HK,Ho CH,Lee CH,Chi CY,Ku CL

更新日期：2017-02-01 00:00:00

abstract：:A monoclonal antibody produced by hybridomas obtained from a mouse immunized with drug-resistant cells recognizes certain hamster and human drug-resistant cell lines but not their drug-sensitive parental lines. Preliminary characterization of the antigen indicates that it is a molecule of approximately 180,000 daltons...

journal_title：Journal of clinical immunology

authors： O'Hara CJ,Grover J,Price GB

更新日期：1984-09-01 00:00:00

abstract：:CFSE dye dilution analysis and [3H] thymidine incorporation were used side by side to assess proliferative responses of peripheral blood mononuclear cells (PBMCs) after vaccination of renal cell carcinoma patients (n=6) with antigen-loaded dendritic cells. Immune responses against the control antigen keyhole limpet he...

journal_title：Journal of clinical immunology

authors： Putz T,Ramoner R,Gander H,Rahm A,Bartsch G,Höltl L,Thurnher M

更新日期：2004-11-01 00:00:00

abstract：:Oral tolerance is the immunologic mechanism by which the mucosal immune system maintains unresponsiveness to the myriad of antigens in the mucosal environment which might otherwise induce untoward immune responses. Recent studies have shown that it is mediated by several distinct, yet interacting mechanisms including ...

journal_title：Journal of clinical immunology

authors： Strober W,Kelsall B,Marth T

更新日期：1998-01-01 00:00:00

abstract：:An absolute requirement for monocytes was demonstrated in the T-cell proliferative response to tetanus toxoid (TT) antigen. Antigen-pulsed monocytes were shown to be effective in triggering T-cell proliferation. Using 125I-radiolabeled TT antigen, uptake by monocytes increased progressively over an 18-hr period, at wh...

journal_title：Journal of clinical immunology

authors： Alpert SD,Jonsen ME,Broff MD,Schneeberger E,Geha RS

更新日期：1981-01-01 00:00:00

abstract：:Despite multiple sexual exposures to HIV-1 virus, some individuals remain HIV-1 seronegative. Although several genetic factors have been related to HIV-1 resistance, the homozygosity for a mutation in CCR5 gene (the 32-bp deletion, i.e., CCR5-Delta32 allele) is presently considered the most relevant one. The C-type le...

journal_title：Journal of clinical immunology

authors： Rathore A,Chatterjee A,Sivarama P,Yamamoto N,Dhole TN

更新日期：2008-01-01 00:00:00

abstract：INTRODUCTION:Rheumatic fever (RF) is an autoimmune disease caused by the gram-positive bacteria Streptococcus pyogenes that follows a nontreated throat infection in susceptible children. The disease manifests as polyarthritis, carditis, chorea, erythema marginatum, and/or subcutaneous nodules. Carditis, the most seriou...

journal_title：Journal of clinical immunology

authors： Guilherme L,Kalil J

更新日期：2010-01-01 00:00:00

abstract：:During the last five decades, elegant mouse models of hematopoiesis have yielded most of the seminal insights into this complex biological system of self-renewal and lineage commitment. More recent advances in assays to measure human stem and progenitor cells as well as high resolution RNA profiling have revealed that...

journal_title：Journal of clinical immunology

authors： Parekh C,Crooks GM

更新日期：2013-05-01 00:00:00