Abstract:
:Lysinuric protein intolerance (LPI) is characterized by defective cellular transport of the dibasic amino acids, secondary dysfunction of the urea cycle, aversion to dietary protein, failure to thrive, hepatosplenomegaly and osteoporosis. Because several patients have suffered from recurrent respiratory infections and/or severe generalized varicella, and a few have developed systemic lupus, vasculitis or other autoimmune diseases, we have now evaluated the function of patients' immune systems. Serum concentrations of one to three IgG subclasses were decreased in 10 of the 12 patients studied. Antibody titres against diphtheria, tetanus and Haemophilus influenzae (Hib) were below the detection limit of the assay in four, three and eight of the 11 patients examined, respectively. (Re)vaccination of these 11 patients led to satisfactory responses against tetanus, but two patients still failed to develop measurable antibodies against diphtheria, two against Hib and six against one or more of the three serotypes of 23-valent pneumococcus vaccine. The proportions of T cells of all lymphocytes and the proliferative responses of the peripheral blood mononuclear cells were normal. In conclusion, humoral immune responses in some patients with LPI are defective and these patients may benefit from intravenous immunoglobulin therapy.
journal_name
Clin Exp Immunoljournal_title
Clinical and experimental immunologyauthors
Lukkarinen M,Parto K,Ruuskanen O,Vainio O,Käyhty H,Olander RM,Simell Odoi
10.1046/j.1365-2249.1999.00868.xsubject
Has Abstractpub_date
1999-06-01 00:00:00pages
430-4issue
3eissn
0009-9104issn
1365-2249journal_volume
116pub_type
杂志文章abstract::Immunoblotting of platelets that have been subjected to SDS-PAGE has revealed that sera from normal individuals contain IgG which binds to many platelet components. This binding was seen with autologous and heterologous platelets using serum of males and of nulliparous females who had not received blood transfusions. ...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:10.1111/j.1365-2249.1990.tb08097.x
更新日期:1990-03-01 00:00:00
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:
更新日期:1980-12-01 00:00:00
abstract::Histamine (10(-3)-10(-8) M) inhibits PHA-induced proliferation of human peripheral blood lymphocytes (HPBL). Inhibition is detected at low concentrations of PHA but is rarely observed at high PHA concentrations. The histamine type II (H2) receptor agonists dimaprit, impromidine and 4-methylhistamine (4MH) inhibit HPBL...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:
更新日期:1983-01-01 00:00:00
abstract::Peripheral blood leucocytes from multiple sclerosis (MS) patients and from normal individuals were tested for their interferon (IFN) producing capacity after stimulation in vitro with various lectins and viruses. The lectins, Con A, PHA and PWM, induced IFN-gamma. In a kinetic study, the response to Con A revealed its...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:
更新日期:1984-10-01 00:00:00
abstract::Mesenchymal stromal cells (MSC) have emerged as promising cell therapies for multiple conditions based on demonstrations of their potent immunomodulatory and regenerative capacities in models of inflammatory disease. Understanding the effects of MSC on T cells has dominated the majority of work carried out in this fie...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章,评审
doi:10.1111/cei.12929
更新日期:2017-04-01 00:00:00
abstract::Striking variations of serum immunoglobulin class and IgG subclass levels were observed in five patients with common variable immunodeficiency. They occurred mainly in untreated patients or, in those patients who received substitutive therapy, could not be merely due to replacement. They result in major changes in the...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:10.1111/j.1365-2249.1991.tb08118.x
更新日期:1991-04-01 00:00:00
abstract::Cells from the peripheral blood of patients with B cell chronic lymphocytic leukaemia (B-CLL) and acute lymphoblastic leukaemia (ALL) were examined for the expression of MHC class II antigens, using a number of monoclonal antibodies (MoAb) including L243 (anti-DR) and TU22 (anti-DQ). There was wide variation in expres...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:
更新日期:1986-02-01 00:00:00
abstract::Muscle is an attractive target for gene therapy and for immunization with DNA vaccines and is also the target of immunological injury in myositis. It is important therefore to understand the immunologic capabilities of muscle cells themselves. In this study, we show that proinflammatory stimuli induce the expression o...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:10.1046/j.1365-2249.1998.00664.x
更新日期:1998-09-01 00:00:00
abstract::The immunopathology of patch test reactions to aeroallergens in patients with atopic dermatitis (AD) has been compared to that of contact sensitivity reactions to nickel in non-atopic individuals. Both reactions were found to exhibit equivalent erythema and induration on gross examination at 48 h. Four millimetre punc...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:10.1111/j.1365-2249.1993.tb05909.x
更新日期:1993-03-01 00:00:00
abstract::Ye-3 is an HLA-B27-specific murine monoclonal antibody recognizing the heat-modifiable protein of Enterobacteriaceae. Here we used recombinant hybrid molecules between the HLA-B7 and HLA-B27 antigens to delineate the epitope recognized by Ye-3. Results of these experiments indicated that the segment of HLA-B*2705 span...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:10.1111/j.1365-2249.1990.tb05397.x
更新日期:1990-10-01 00:00:00
abstract::Previous studies demonstrated that NK resistant Epstein-Barr virus (EBV) carrying human lymphoblastoid cell lines become sensitive to NK cell-mediated destruction following induction of the viral cycle by superinfection with the P3HR-1 substrain of EBV or chemicals. In the present report we analysed the cellular membr...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:
更新日期:1983-10-01 00:00:00
abstract::T cells are thought to play an important regulatory role in atopic asthma. We hypothesized that human blood and BAL T cell subsets bearing various TCR-Vbeta genes might show selective differences in their cytokine profile. Peripheral blood (PB) and bronchoalveolar lavage (BAL) T cells from seven atopic asthmatic and s...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:10.1046/j.1365-2249.2002.01847.x
更新日期:2002-05-01 00:00:00
abstract::Mixed leucocyte culture blocking factor activity (MLC-BFA) in the plasma of haemodialysis patients appears as a result of recent blood transfusions and is concentrated in IgG fractions of the blocking serum. Four patients neither developed lymphocytotoxins nor MLC-BFA in spite of having received multiple blood transfu...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:
更新日期:1975-05-01 00:00:00
abstract::Progesterone, estradiol, testosterone, cortisol, and 11-desoxycortisol (compound S) were added to cultures of human lymphocytes stimulated with phytohaemagglutinin (PHA) and purified protein derivative (PPD). The immunosuppressive effect of cortisol was verified and the three sex-steroid hormones also were found to in...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:
更新日期:1977-03-01 00:00:00
abstract::Bacillus Calmette-Guérin (BCG) remains the only available and most widely administered vaccine against Mycobacterium tuberculosis (Mtb), yet it fails to protect vaccinated individuals either from primary infection or reactivation of latent tuberculosis (TB). Despite BCG's variable efficacy against TB, the fact remains...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:10.1111/cei.12634
更新日期:2015-08-01 00:00:00
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journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:10.1111/j.1365-2249.1995.tb02273.x
更新日期:1995-07-01 00:00:00
abstract::To elucidate the mechanisms of anti-DNA production, we assessed the binding of sera of normal human subjects (NHS) and patients with SLE to a panel of bacterial and mammalian DNA. Using single-stranded DNA as antigens in an ELISA, NHS showed significant binding to some but not all bacterial DNA, while lacking reactivi...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:10.1046/j.1365-2249.1997.4301328.x
更新日期:1997-07-01 00:00:00
abstract::Booster immunization of normal individuals with soluble tetanus toxoid (TT) produced significant levels of plasma anti-tetanus IgG and IgM detectable by an enzyme linked immunosorbent assay (ELISA). Similar changes in circulating anti-TT antibody were found in mice following primary immunization. There was. however, n...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:
更新日期:1982-09-01 00:00:00
abstract::An adult twin with recurrent bacterial infections was found to have a partial functional deficiency of complement factor D. Full restoration of alternative pathway activity and zymosan- or cobra venom factor-induced consumption of C3 and B was found after reconstitution of patient's serum with purified D. Family studi...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:
更新日期:1984-12-01 00:00:00
abstract::Transforming growth factor-beta (TGF-beta) and prostaglandins (PG) regulate the cell-mediated immune response, so it has been proposed that they affect the progression of pulmonary tuberculosis. Here we report that the administration of soluble betaglycan, a potent TGF-beta antagonist, and niflumic acid, a PG synthesi...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:10.1111/j.1365-2249.2006.03049.x
更新日期:2006-05-01 00:00:00
abstract::PHA activated human peripheral blood mononuclear cells (MNC) were incubated with human interleukin 2 (IL-2) in the absence or in the presence of 10(-6)-10(-9) M hydrocortisone (HC). HC suppressed the proliferative response of the activated MNC to highly purified human leucocyte IL-2 in a dose dependent manner. This su...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:
更新日期:1987-04-01 00:00:00
abstract::Soluble histocompatibility antigens of the class II region have been detected in synovial fluids obtained from patients with rheumatoid arthritis. A capture immunoassay involving two monoclonal antibodies was used; interference by rheumatoid factor, which is a feature of such assays, was overcome by mild pretreatment ...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:10.1111/j.1365-2249.1990.tb06437.x
更新日期:1990-04-01 00:00:00
abstract::Using an autoimmune hepatitis model of A/J mice which was prepared with immunization by syngeneic crude liver proteins, various influences of neonatal thymectomy were studied by observations of histological liver changes, autoantibody to liver-specific membrane lipoprotein (LSP), delayed-type hypersensitivity (DTH) to...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:
更新日期:1987-01-01 00:00:00
abstract::Benznidazole (BZL) is a nitroheterocyclic drug employed in the chemotherapy of Chagas' disease, a protozoan disease caused by Trypanosoma cruzi. Because this parasite mostly replicates in macrophages, we investigated whether BZL was likely to modify the synthesis of macrophage mediators such as nitrite, tumour necrosi...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:10.1046/j.1365-2249.1999.01053.x
更新日期:1999-11-01 00:00:00
abstract::Natural Killer (NK) cell activity against K562 target cells was measured in 21 female patients with primary Sjögren's syndrome (primary SS) and in 20 female normal controls matched for age. The in vitro effect of alpha-interferon (IF) and interleukin 2 (IL-2) on NK cell activity was examined and the percentage of larg...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:
更新日期:1986-01-01 00:00:00
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
更新日期:2016-11-01 00:00:00
abstract::We have produced monoclonal antibodies (MoAb) to the Rb core and lipid A regions of Salmonella lipopolysaccharide (LPS) and have assessed their ability to inhibit LPS-mediated mitogenic responses in vitro, and to protect against LPS toxicity and lethal Salmonella infection in vivo. Monoclonal antibodies RC-8 and RC-16...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:
更新日期:1988-04-01 00:00:00
abstract::An eosinophil stimulating material (ESM) is described in the blister fluids (BF) of six patients with bullous pemphigoid (BP). This ESM is similar in function to a subspecies of human colony stimulating factor (CSF), CSF-alpha, since (i) it stimulated the production from bone marrow cells of day 14 colonies (31 +/- 15...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:
更新日期:1982-12-01 00:00:00
abstract::A new, specific haemolytic assay for C2 is described using EAC43 (Antrypol) cells and serum deficient in C4 and C2 from a patient with hereditary angio-oedema. The method is simple and the results in pathological and normal sera correlate well with those obtained using the EA and C2-deficient serum method. ...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:
更新日期:1976-09-01 00:00:00
abstract::The hydrodynamic (gel filtration and sedimentation) properties of an isolated monoclonal IgM-K cryoglobulin (McE.) and five non-cryoglobulin cold-soluble proteins, as well as their constituent monomeric subunits and (Fc)5mu and Fabmu fragments, are compared under both native and partially denaturing conditions. It is ...
journal_title:Clinical and experimental immunology
pub_type: 杂志文章
doi:
更新日期:1978-01-01 00:00:00