Immunodeficiencies.

Abstract:

:Primary immunodeficiencies (PIDs) are uncommon, chronic and severe disorders of the immune system in which patients cannot mount a sufficiently protective immune response, leading to an increased susceptibility to infections. The treatment of choice for PID patients with predominant antibody deficiency is intravenous immunoglobulin (Ig) replacement therapy. Despite major advances over the last 20 years in the molecular characterization of PIDs, many patients remain undiagnosed or are diagnosed too late, with severe consequences. Various strategies to ensure timely diagnosis of PIDs are in place, and novel approaches are being developed. In recent years, several patient registries have been established. Such registries shed light on the pathology and natural history of these varied disorders. Analyses of the registry data may also reveal which patients are likely to respond well to higher Ig infusion rates and may help to determine the optimal dosing of Ig products. Faster infusion rates may lead to improved convenience for patients and thus increase patient compliance, and may reduce nursing time and the need for hospital resources. Data from two recent studies suggest that Gamunex and Privigen are well tolerated at high infusion rates. Nevertheless, careful selection of patients for high infusion rates, based on co-morbid conditions and tolerance of the current infusion rate, is advisable. Based on the available data, intravenous Ig offers broad protection against encapsulated organisms. As vaccine trends change, careful monitoring of specific antibody levels in the general population, such as those against pneumococcal and meningococcal bacteria, should be implemented.

journal_name

Clin Exp Immunol

authors

Ballow M,Notarangelo L,Grimbacher B,Cunningham-Rundles C,Stein M,Helbert M,Gathmann B,Kindle G,Knight AK,Ochs HD,Sullivan K,Franco JL

doi

10.1111/j.1365-2249.2009.04023.x

subject

Has Abstract

pub_date

2009-12-01 00:00:00

pages

14-22

eissn

0009-9104

issn

1365-2249

pii

CEI4023

journal_volume

158 Suppl 1

pub_type

杂志文章,评审
  • The avidity of allospecific cytotoxic T lymphocytes (CTL) determines their cytokine production profile.

    abstract::Donor-specific CTL present within the cardiac allograft during a rejection episode are distinct from those that populate the cardiac allograft in the absence of rejection. Whereas the former generally have a high avidity for donor cells, the latter mainly have a low avidity for donor cells. This observation made us re...

    journal_title:Clinical and experimental immunology

    pub_type: 杂志文章

    doi:10.1046/j.1365-2249.1997.4441458.x

    authors: Van Emmerik NE,Daane CR,Knoop CJ,Hesse C,Vaessen LM,Balk AH,Mochtar B,Claas FH,Weimar W

    更新日期:1997-12-01 00:00:00

  • Antibody to severe acute respiratory syndrome (SARS)-associated coronavirus spike protein domain 2 cross-reacts with lung epithelial cells and causes cytotoxicity.

    abstract::Both viral effect and immune-mediated mechanism are involved in the pathogenesis of severe acute respiratory syndrome-associated coronavirus (SARS-CoV) infection. In this study, we showed that in SARS patient sera there were autoantibodies (autoAbs) that reacted with A549 cells, the type-2 pneumocytes, and that these ...

    journal_title:Clinical and experimental immunology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2249.2005.02864.x

    authors: Lin YS,Lin CF,Fang YT,Kuo YM,Liao PC,Yeh TM,Hwa KY,Shieh CC,Yen JH,Wang HJ,Su IJ,Lei HY

    更新日期:2005-09-01 00:00:00

  • Polymorphisms in STAT-4, IL-10, PSORS1C1, PTPN2 and MIR146A genes are associated differently with prognostic factors in Italian patients affected by rheumatoid arthritis.

    abstract::Rheumatoid arthritis (RA) is a systemic autoimmune disease resulting in chronic inflammation of the synovium and consequent cartilage and bone erosion. RA is associated strongly with the presence of rheumatoid factor (RF), and consists of clinical subsets of anti-citrullinated protein antibody (ACPA)-positive and -neg...

    journal_title:Clinical and experimental immunology

    pub_type: 杂志文章

    doi:10.1111/cei.12831

    authors: Ciccacci C,Conigliaro P,Perricone C,Rufini S,Triggianese P,Politi C,Novelli G,Perricone R,Borgiani P

    更新日期:2016-11-01 00:00:00

  • Nippostrongylus brasiliensis in young rats. Lymphocytes expel larval infections but not adult worms.

    abstract::Young rats were not able to expel adult N. brasiliensis infections even when the worms were damaged by antibodies and the young rats were given all the cellular components (sensitized lymphocytes and bone marrow cells) shown to be necessary for the expulsion of antibody-damaged worms from adult rats. In contrast, most...

    journal_title:Clinical and experimental immunology

    pub_type: 杂志文章

    doi:

    authors: Love RJ,Ogilvie BM

    更新日期:1975-07-01 00:00:00

  • Intravenous immunoglobulin (IVIG) preparations induce apoptosis in TNF-alpha-stimulated endothelial cells via a mitochondria-dependent pathway.

    abstract::Endothelial cells (ECs) are a target in inflammation, and the death of EC is regulated by various factors. Although intravenous immunoglobulin (IVIG) preparations are known to be beneficial therapeutic agents for the treatment of autoimmune diseases and systemic inflammatory disorders, their mechanism of action have n...

    journal_title:Clinical and experimental immunology

    pub_type: 杂志文章

    doi:10.1046/j.1365-2249.2002.01769.x

    authors: Nakatani K,Takeshita S,Tsujimoto H,Sekine I

    更新日期:2002-03-01 00:00:00

  • Regulation of IgA secretion in polyclonally induced in vitro human lymphocyte cultures: the function of T and B cells from mesenteric lymph nodes and peripheral blood.

    abstract::Human gut-associated immunoregulatory events were studied in a pokeweed mitogen (PWM)-stimulated culture system using lymphocytes obtained from the mesenteric lymph nodes (MLN) of female subjects undergoing gastroplasty for obesity. Compared with peripheral blood lymphocytes, lymphocytes obtained from MLN secreted IgG...

    journal_title:Clinical and experimental immunology

    pub_type: 杂志文章

    doi:

    authors: Pang G,Yeung S,Clancy RL,Cripps AW,Hennessy EJ,Santhanam AN

    更新日期:1986-04-01 00:00:00

  • Anti-golgi complex autoantibodies in a patient with Sjögren syndrome and lymphoma.

    abstract::During routine immunofluorescence studies of the serum of a patient with Sjögren's syndrome and lymphoma we detected antibodies giving a cytoplasmic pattern which did not correspond to previously described patterns found for autoantibodies. Using different cells and tissues as substrates for indirect immunofluorescenc...

    journal_title:Clinical and experimental immunology

    pub_type: 杂志文章

    doi:

    authors: Rodríguez JL,Gelpi C,Thomson TM,Real FJ,Fernández J

    更新日期:1982-09-01 00:00:00

  • Induction and increase of HLA-DR antigen expression by immune interferon on ML-3 cell line enhances the anti-HLA-DR immunotoxin activity.

    abstract::In order to evaluate the impact of induction and increase target antigen expression on immunotoxin potency, we measured the potentiating effect of recombinant immune interferon-gamma (rIFN-gamma) on the cytotoxicity of an anti HLA-DR ricin A-chain immunotoxin (2G5 RTA-IT) on the myeloid cell line ML-3. After 48 h of i...

    journal_title:Clinical and experimental immunology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2249.1990.tb05429.x

    authors: Chiron M,Jaffrezou JP,Carayon P,Bordier C,Roubinet F,Xavier C,Brandely M,Laurent G

    更新日期:1990-11-01 00:00:00

  • Mucosal and systemic immune responses to plasmid protein pgp3 in patients with genital and ocular Chlamydia trachomatis infection.

    abstract::The circulating and cervical B cell responses to Chlamydia trachomatis plasmid protein pgp3 were characterized in children and adults with ocular or genital chlamydial infection using the enzyme-linked immunospot assay (ELISPOT) and ELISA. No pgp3-specific ASCs were detected in healthy controls, but predominantly IgA ...

    journal_title:Clinical and experimental immunology

    pub_type: 杂志文章

    doi:10.1046/j.1365-2249.2003.02163.x

    authors: Ghaem-Maghami S,Ratti G,Ghaem-Maghami M,Comanducci M,Hay PE,Bailey RL,Mabey DC,Whittle HC,Ward ME,Lewis DJ

    更新日期:2003-06-01 00:00:00

  • In vitro exposure of human fibroblasts to local anaesthetics impairs cell growth.

    abstract::Lidocaine, bupivacaine or ropivacaine are used routinely to manage perioperative pain. Sparse data exist evaluating the effects of local anaesthetics (LA) on fibroblasts, which are involved actively in wound healing. Therefore, we investigated the effects of the three LA to assess the survival, viability and prolifera...

    journal_title:Clinical and experimental immunology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2249.2010.04252.x

    authors: Fedder C,Beck-Schimmer B,Aguirre J,Hasler M,Roth-Z'graggen B,Urner M,Kalberer S,Schlicker A,Votta-Velis G,Bonvini JM,Graetz K,Borgeat A

    更新日期:2010-11-01 00:00:00

  • Correlation between chemical structure and biological activities of Porphyromonas gingivalis synthetic lipopeptide derivatives.

    abstract::We recently separated a PG1828-encoded triacylated lipoprotein (Pg-LP), composed of two palmitoyl and one pentadecanoyl groups at the N-terminal of glycerocysteine from Porphyromonas gingivalis, a periodontopathic bacteria, and found that Pg-LP exhibited definite biological activities through Toll-like receptor (TLR) ...

    journal_title:Clinical and experimental immunology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2249.2006.03182.x

    authors: Makimura Y,Asai Y,Taiji Y,Sugiyama A,Tamai R,Ogawa T

    更新日期:2006-10-01 00:00:00

  • High IgE in rheumatoid arthritis (RA) patients is complexed with anti-IgE autoantibodies.

    abstract::This study presents data on more than 300 RA and allergic patients analysed for their serum levels of anti-immunoglobulin isotype autoantibodies and IgE. We observed high levels of IgE in sera of RA and allergic patients. Interestingly, we measured significantly higher specific IgE levels against Alternaria but not ag...

    journal_title:Clinical and experimental immunology

    pub_type: 杂志文章

    doi:10.1046/j.1365-2249.1999.00787.x

    authors: Millauer N,Zuercher AW,Miescher SM,Gerber HA,Seitz M,Stadler BM

    更新日期:1999-01-01 00:00:00

  • Reduced blood BDCA-2+ (lymphoid) and CD11c+ (myeloid) dendritic cells in systemic lupus erythematosus.

    abstract::Type 1 IFN is thought to be implicated in the autoimmune process of SLE. Plasmacytoid dendric cells (DC), which are natural IFN-alpha producing cells, play a pivotal epipathogenic role in SLE. The present study was undertaken to investigate the phenotypic characteristics of peripheral blood DC in SLE patients in compa...

    journal_title:Clinical and experimental immunology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2249.2005.02897.x

    authors: Migita K,Miyashita T,Maeda Y,Kimura H,Nakamura M,Yatsuhashi H,Ishibashi H,Eguchi K

    更新日期:2005-10-01 00:00:00

  • Expression of the B cell repertoire in lpr mice; abnormal expansion of a few VHJ558 germ-line genes.

    abstract::Analysis of the VH gene repertoire of the J558 family was done in lipopolysaccharide (LPS)-stimulated resting cells and in vivo activated cells derived from C57Bl/6-lpr mice (IghCb). Using a restriction fragment length polymorphism (RFLP) based on digestion with the restriction enzyme Pstl, the expression of the subfa...

    journal_title:Clinical and experimental immunology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2249.1995.tb05543.x

    authors: Alarcón-Riquelme ME,Fernández C

    更新日期:1995-02-01 00:00:00

  • Analysis of T cell subsets by monoclonal antibodies in patients with tuberculosis after in vitro stimulation with purified protein derivative of tuberculin.

    abstract::By using OKT monoclonal antibodies; OKT3(pan T), OKT4(inducer/helper), OKT8 (suppressor/cytotoxic) and OKIa1, T lymphocyte subsets were examined in lymphocytes of patients with tuberculosis both before and after in vitro stimulation with purified protein derivative of tuberculin (PPD). In freshly obtained lymphocytes ...

    journal_title:Clinical and experimental immunology

    pub_type: 杂志文章

    doi:

    authors: Shiratsuchi H,Tsuyuguchi I

    更新日期:1984-08-01 00:00:00

  • Incidence of selective IgG2 deficiency in patients with vasculitis.

    abstract::IgG2 deficiency has been recently associated with the appearance of vasculitis. None of the 42 patients with IgG2 deficiency whom we studied has a history of vasculitis. We have also studied 56 patients with vasculitis, and found four of them with IgG2 levels below the normal range for their age. As a group, the vascu...

    journal_title:Clinical and experimental immunology

    pub_type: 杂志文章

    doi:

    authors: Jiménez A,López-Trascasa M,Fontán G

    更新日期:1989-11-01 00:00:00

  • Regulation of experimental autoimmune uveitis in rats--separation of MHC and non-MHC gene effects.

    abstract::Experimental autoimmune uveitis (EAU) is an organ-specific autoimmune disease and has served as a model of certain ocular inflammatory conditions in man. The present study was aimed at separating the effects of MHC and non-MHC genes on the development of EAU in the rat. EAU-susceptible LEW (RT1l), EAU-resistant WKAH (...

    journal_title:Clinical and experimental immunology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2249.1991.tb02947.x

    authors: Hirose S,Ogasawara K,Natori T,Sasamoto Y,Ohno S,Matsuda H,Onoé K

    更新日期:1991-12-01 00:00:00

  • Predominance of IgA deposits in glomeruli of Schistosoma mansoni infected mice.

    abstract::We found that IgA is predominant among the immune deposits in the renal glomeruli of mice infected with Schistosoma mansoni, and thus conducted an analysis of the deposition of different immunoglobulin isotypes in the glomeruli throughout the course of infection in mice. Both immunofluorescent and immunoperoxidase met...

    journal_title:Clinical and experimental immunology

    pub_type: 杂志文章

    doi:

    authors: el-Sherif AK,Befus D

    更新日期:1988-01-01 00:00:00

  • An approach to the routine estimation of circulating carcinoembryonic antigen immune complexes in patients with carcinomata of the gastrointestinal tract.

    abstract::The carcinoembryonic antigen (CEA) distribution obtained after fractionation of thirty sera of patients with gastrointestinal tumours by gel filtration in Sephadex G200 was compared with that resulting from extraction of the sera with perchloric acid. In all cases gel filtration yielded a fraction of free CEA and in t...

    journal_title:Clinical and experimental immunology

    pub_type: 杂志文章

    doi:

    authors: Kapsopoulou-Dominos K,Anderer FA

    更新日期:1979-07-01 00:00:00

  • Antibody penetration of viable human cells. I. Increased penetration of human lymphocytes by anti-RNP IgG.

    abstract::Antibody penetration of viable cells and interaction with intracellular antigens may have major consequences for immunopathological processes in connective tissue diseases. We have reported previously that antibody can penetrate viable human lymphocytes. To assess further the role of antinuclear antibodies in this pro...

    journal_title:Clinical and experimental immunology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2249.1991.tb08128.x

    authors: Ma J,Chapman GV,Chen SL,Melick G,Penny R,Breit SN

    更新日期:1991-04-01 00:00:00

  • The immune response to the cell wall of Mycobacterium bovis BCG.

    abstract::Mice were immunized with the cell wall of BCG suspended in an oil-in-saline emulsion, and examined against time for the emergence of T cell-mediated acquired immunity. Evidence is presented that shows that levels of acquired resistance expressed in these animals over the first month following inoculation, and which en...

    journal_title:Clinical and experimental immunology

    pub_type: 杂志文章

    doi:

    authors: Orme IM

    更新日期:1988-03-01 00:00:00

  • Expression and functional role of 1F7 (CD26) antigen on peripheral blood and synovial fluid T cells in rheumatoid arthritis patients.

    abstract::The expression and the functional role of the CD26 (1F7) T cell surface molecule, an ectoenzyme which seems to represent a functional collagen receptor of T lymphocytes and to have a role in T cell activation, were analysed in both peripheral blood (PB) and synovial fluid (SF) T cell samples from patients with active ...

    journal_title:Clinical and experimental immunology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2249.1994.tb06134.x

    authors: Muscat C,Bertotto A,Agea E,Bistoni O,Ercolani R,Tognellini R,Spinozzi F,Cesarotti M,Gerli R

    更新日期:1994-11-01 00:00:00

  • Effects of tunicamycin and various monosaccharides on phytohaemagglutinin-induced autorosette formation.

    abstract::The structural characteristics of autologous red blood cell (ARBC) receptors on human peripheral blood lymphocytes (PBL) induced by phytohaemagglutinin (PHA) were examined. Tunicamycin, which is known to be a blocker of the protein glycosylation of N-glycosidic type glycoprotein, significantly inhibited the production...

    journal_title:Clinical and experimental immunology

    pub_type: 杂志文章

    doi:

    authors: Hyodo S,Kishi T,Kashiwa H,Kittaka E,Suzawa T,Tanaka Y,Sakano T,Usui T

    更新日期:1984-07-01 00:00:00

  • Busulfan and chloramphenicol induced T cell lymphoma: cell surface characteristics and functional properties.

    abstract::We report the immunological studies on three transplantable lymphoma lines that developed when CAF1 mice were injected with busulfan and chloramphenicol. The lymphoma cells displayed Thy-1.2, brain associated antigen, and H-2d alloantigen. They were negative for surface IgM and Ia antigens. Expression of T cell differ...

    journal_title:Clinical and experimental immunology

    pub_type: 杂志文章

    doi:

    authors: Bhoopalam N,Price K,Norgello H,Barone-Varelas J,Fried W

    更新日期:1986-06-01 00:00:00

  • Circulating soluble selectins in Kawasaki disease.

    abstract::To investigate the significance of circulating adhesion molecules associated with leucocyte-endothelial cell interaction in Kawasaki disease (KD), serum levels of soluble E-, P-, L-selectin (sE-, sP-, sL-selectin), and vascular cell adhesion molecule-1 (VCAM-1) were measured in 16 patients with KD, eight with other fe...

    journal_title:Clinical and experimental immunology

    pub_type: 杂志文章

    doi:10.1046/j.1365-2249.1997.3852128.x

    authors: Takeshita S,Dobashi H,Nakatani K,Koike Y,Tsujimoto H,Hirayama K,Kawamura Y,Mori K,Sekine I,Yoshioka S

    更新日期:1997-06-01 00:00:00

  • Studies on the origin of the precursor cells in multiple myeloma, Waldenström's macroglobulinaemia and benign monoclonal gammopathy. I. Cytoplasmic isotype and idiotype distribution in peripheral blood and bone marrow.

    abstract::Lymphocytes and plasma cells in the peripheral blood and bone marrow of patients with multiple myeloma, benign monoclonal gammopathy and Waldenström's macroglobulinaemia were investigated for their cytoplasmic immunoglobulin distribution. Anti-idiotypic sera were used as markers for monoclonality. Double-wavelength fl...

    journal_title:Clinical and experimental immunology

    pub_type: 杂志文章

    doi:

    authors: Van Camp B,Reynaert P,Broodtaerts L

    更新日期:1981-04-01 00:00:00

  • The potential role for infections in the pathogenesis of autoimmune Addison's disease.

    abstract::Autoimmune Addison's disease (AAD), or primary adrenocortical insufficiency, is a classical organ-specific autoimmune disease with 160 years of history. AAD is remarkably homogeneous with one major dominant self-antigen, the cytochrome P450 21-hydroxylase enzyme, which is targeted by both autoantibodies and autoreacti...

    journal_title:Clinical and experimental immunology

    pub_type: 杂志文章,评审

    doi:10.1111/cei.13207

    authors: Hellesen A,Bratland E

    更新日期:2019-01-01 00:00:00

  • HLA class II glycoprotein expression by monocytes from HIV-infected individuals: differences in response to interferon-gamma.

    abstract::The expression of HLA class II glycoproteins by monocytes from HIV-infected individuals was evaluated. We found no differences in HLA-DR, HLA-DQ, or HLA-DP expression by freshly isolated monocytes from patients with clinical AIDS, HIV-infected patients without AIDS or from uninfected individuals. However, monocytes fr...

    journal_title:Clinical and experimental immunology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2249.1990.tb05489.x

    authors: Zwilling BS,Darrow R,Mcpeek M,Laufman H,Pearl D

    更新日期:1990-12-01 00:00:00

  • Cellular immune responses against hepatitis C virus: the evidence base 2002.

    abstract::Hepatitis C virus (HCV) is an RNA virus which is estimated to persistently infect about 170 million people worldwide. After acute infection, there is an initial period during which long-term outcome is decided. There is strong evidence that the cellular immune responses, involving both CD4+ and CD8+ T lymphocytes, are...

    journal_title:Clinical and experimental immunology

    pub_type: 杂志文章,评审

    doi:10.1046/j.1365-2249.2002.01840.x

    authors: Ward S,Lauer G,Isba R,Walker B,Klenerman P

    更新日期:2002-05-01 00:00:00

  • Immune response to rabbit liver-specific lipoprotein in acute viral hepatitis.

    abstract::A serial prospective study of cell-mediated immunity to rabbit liver-specific lipoprotein (RLSP) has been done in 26 patients with acute viral hepatitis (AH) (18 HBsAg+ and eight HBsAg-) using a lymphocyte transformation test. An increased stimulation index was recorded in 56% of HBsAg+ cases and in 63% of the HBsAg- ...

    journal_title:Clinical and experimental immunology

    pub_type: 杂志文章

    doi:

    authors: Ortona L,Laghi V,Cauda R,Nervo P

    更新日期:1980-12-01 00:00:00