Audit of sweat chloride testing reveals analytical errors.

Objectives: Sweat chloride testing (SCT) is the mainstay for the diagnosis of cystic fibrosis (CF) and biomarker in the evaluation of CFTR-modifying drugs. To be a reliable and valid tool, analytical variance (CVA) must be minimized. However, external quality assessments have revealed significant deviations in routine clinical practice.

Stable production of cyanophycinase in Nicotiana benthamiana and its functionality to hydrolyse cyanophycin in the murine intestine.

Food supplementation with the conditionally essential amino acid arginine (Arg) has been shown to have nutritional benefits. Degradation of cyanophycin (CGP), a peptide polymer used for nitrogen storage by cyanobacteria, requires cyanophycinase (CGPase) and results in the release of β-aspartic acid (Asp)-Arg dipeptides. The simultaneous production of CGP and CGPase in plants could be a convenient source of Arg dipeptides.

Attenuation of murine antigen-induced arthritis by treatment with a decoy oligodeoxynucleotide inhibiting signal transducer and activator of transcription-1 (STAT-1).

The transcription factor STAT-1 (signal transducer and activator of transcription-1) plays a pivotal role in the expression of inflammatory gene products involved in the pathogenesis of arthritis such as various cytokines and the CD40/CD40 ligand (CD40/CD40L) receptor-ligand dyad. The therapeutic efficacy of a synthetic decoy oligodeoxynucleotide (ODN) binding and neutralizing STAT-1 was tested in murine antigen-induced arthritis (AIA) as a model for human rheumatoid arthritis (RA). The STAT-1 decoy ODN was injected intra-articularly in methylated bovine serum albumin (mBSA)-immunized mice 4 h before arthritis induction.

Osteoprotegerin reduces the loss of periarticular bone mass in primary and secondary spongiosa but does not influence inflammation in rat antigen-induced arthritis.

Objective: To assess the effect of osteoprotegerin (OPG) on joint swelling, synovial inflammation and cartilage destruction, periarticular and axial bone volume, and bone turnover in rat antigen-induced arthritis (AIA).

Design: Rats were treated with OPG (3 mg/kg/day) at regular intervals from day 1 to day 20 of AIA. Disease activity was evaluated by measurement of joint swelling as well as, joint inflammation and destruction by histology.

Chronic graft-versus-host-disease-like dermopathy in a child with CD4+ cell microchimerism.

We report the case of an 11-year-old boy suffering from a severe progressive chronic skin disease with clinical features of progressive systemic scleroderma, systemic lupus erythematosus and dermatomyositis. Skin biopsies revealed fibrosis and lichenoid changes and muscle biopsy a myositis. Immunohistology of the skin showed a lichen-ruber-like pattern.

Amelioration of murine antigen-induced arthritis by dehydroepiandrosterone (DHEA).

Objective And Design: Sex hormones have immunomodulatory properties and may play an important role in the pathogenesis of autoimmune diseases like rheumatoid arthritis (RA). This study sought to examine the effects of the natural weak androgen dehydroepiandrosterone (DHEA) and its metabolite androstenediol (AED) on the development of murine antigen-induced arthritis (AIA).

Methods: DHEA and AED were administered orally, approximately 10 mg/day, from the time of AIA induction (i.

High-dose clodronate therapy prevents joint destruction in chronic antigen-induced arthritis of the rat but inhibits bone formation at the axial skeleton.

Objective: To investigate the effects of clodronate on clinical disease activity, inflammatory alterations and cartilage destruction, periarticular and axial bone volume and bone turnover in chronic antigen-induced arthritis (AIA; day 28).

Methods: Rats with AIA were treated with clodronate (5 mg/kg/day continuously; 20 mg/kg/day intermittently or high-dose with 300 mg/kg 3 hours after arthritis induction +20 mg/kg/day continuously, respectively). Joint pathology was examined by histology.

Periarticular bone alterations in chronic antigen-induced arthritis: free and liposome-encapsulated clodronate prevent loss of bone mass in the secondary spongiosa.

The long-term effects of acutely administered clodronate (free or liposome-encapsulated) on periarticular bone mass and bone turnover were investigated in chronic antigen-induced arthritis (AIA; day 28). Wistar rats were treated intraperitoneally at 3 h and on days 1, 2, and 7 of AIA, with phosphate-buffered saline (PBS; sham), PBS-containing liposomes, free clodronate, or liposome-encapsulated clodronate (cumulative dose, 3.64 mg/animal).

Limiting dilution analysis of the frequency of autoreactive lymph node cells isolated from mice with antigen-induced arthritis.

Antigen-induced arthritis (AIA) in mice occurs after the single injection of methylated bovine serum albumin (mBSA) into the knee joint of animals preimmunized with the same antigen in complete Freund's adjuvant. A short acute reaction is followed by a chronic inflammation which shows similar histological features to human rheumatoid arthritis. The mechanisms leading to the chronicity of arthritis are not yet clear.

Adoptive transfer of susceptibility to antigen-induced arthritis into severe combined immunodeficient (SCID) mice: role of CD4+ and CD8+ T cells.

Antigen-induced arthritis (AIA) in mice occurs after immunization and a subsequent intra-articular injection with methylated bovine serum albumin (mBSA). The role of T lymphocytes in the adoptive transfer of susceptibility to AIA into SCID mice was investigated. Pooled spleen and lymph node cells from immunized syngeneic or allogeneic donor mice, isolated either before or after the induction of arthritis, could transfer the capacity both to develop arthritis and to produce antibodies to mBSA, collagen type II and cartilage proteoglycans into SCID mice.

Degradation of articular cartilage during the progression of antigen-induced arthritis in mice. A scanning and transmission electron microscopic study.

Ultrastructural changes of knee articular cartilage in C57B1/6 mice were studied in the course of antigen-induced arthritis by means of scanning and transmission electron microscopy. First damages of the cartilage surface were seen one hour after arthritis induction. The earliest signs were the loss of the superficial electron-dense layer as well as a progressive loss of proteoglycans in the cartilaginous matrix on the surface.

Immunomodulation of rat antigen-induced arthritis by leflunomide alone and in combination with cyclosporin A.

The effects of the new immunomodulating isoxazol derivative leflunomide, in comparison with cyclosporin A, on established antigen-induced arthritis in rats as well as serum antibody levels were determined. When treatment with leflunomide, at concentrations from 2.5 to 10 mg/kg/d, was started on day 3 of arthritis, the acute and chronic phases of arthritis were effectively inhibited.

Therapeutic effects of antibodies against adhesion molecules in murine collagen type II-induced arthritis.

Adhesion molecules play important roles in immune reactions and inflammatory processes and may constitute attractive targets for immunomodulatory approaches. In this study, blocking mAbs against a series of adhesion molecules were tested for their therapeutic effect on developing arthritis in a mouse model. MAbs were given for a period of 4 weeks at the time of exspected incidence of visible disease symptoms, i.

Different immunological mechanisms contribute to cartilage destruction in antigen-induced arthritis.

Antigen-induced arthritis in guinea pigs was used as a model to investigate the pathogenic mechanisms responsible for cartilage destruction in chronic joint inflammation. The activation of macrophages, their effects on cartilage metabolism, and the development of autoimmunity to cartilage constituents were studied during the progression of arthritis. The results show that in arthritic animals the macrophages are systemically activated, with a peak in the early phase of inflammation.

Influence of cyclosporin A on cytokine levels in synovial fluid and serum of rats with antigen-induced arthritis.

The effects of the T-cell-directed immunosuppressant cyclosporin A (CsA) on the development of antigen-induced arthritis (AIA) in rats as well as on cytokine levels in synovial fluid and serum were determined. The treatment with CsA effectively inhibited the chronic phase of arthritis as demonstrated by decreased joint swelling and reduced histological arthritic score. In animals with AIA the level of IL-6 in the synovial fluid and serum is increased, showing good correlation with the severity of the disease.

Humoral and cell-mediated sensitivity to cartilage constituents in mice with antigen-induced arthritis.

Serum antibodies against native collagen type II and cartilage proteoglycans as well as proliferative responses of spleen lymphocytes to these antigens were evidenced during the progression of antigen-induced arthritis in mice. Because there were some correlations to the severity of arthritis, it is suggested that autoimmunity to cartilage constituents play a role for the persistence of the joint inflammation and cartilage destruction in this experimental model of chronic arthritis.

The effects of immunomodulatory thymic and splenic peptides and cyclosporin A on antigen-induced arthritis in the rat.

Long-term treatment with natural and synthetic thymic and splenic peptides as well as cyclosporin A inhibited the development of antigen-induced arthritis in rats. This was demonstrated by decreased joint swelling and reduced degree of macroscopically and histologically evaluated severity of synovitis. The drug treatment also decreased serum levels of antibodies against the specific antigen methylated bovine serum albumin (mBSA) and against cartilage proteoglycans and collagens type I and II.

Beta-2-microglobulin-derived amyloidosis: onset, distribution and clinical features in 13 hemodialysed patients.

Postmortem examinations were carried out in 13 patients (6 males, 7 females, age 58 +/- 9 years) who had been on regular hemodialysis treatment for 10-90 months using disposable regenerated cellulose membrane dialyzers. The prevalence of beta 2-microglobulin (beta 2m)-derived AB-amyloid deposits in different sites was determined. At autopsy, specimens were obtained from different joints, paravertebral tissue, intervertebral discs and from visceral organs.

Effects of the immunomodulator diacetyl-splenopentin on antigen-induced arthritis in rabbits.

Long-term treatment with the immunomodulator diacetyl-splenopentin reduces the severity of chronic joint inflammation and cartilage destruction in rabbits with antigen-induced arthritis. The level of specific antibodies as well as specific and non-specific cell-mediated immune reactivities including the proliferative response of spleen lymphocytes to cartilage proteoglycans in treated animals are lower than in untreated arthritic rabbits. Moreover, suppressor cell activity, which normally decreases during the early phase of inflammation, is enhanced and hyperreactive helper cell potential is reduced.

Beta 2-microglobulin serum concentration and associated amyloidosis in dialysis patients.

Using radioimmunological estimation of beta 2-microglobulin (beta 2M), significantly greater serum values were found in 36 dialysis patients (44.4 +/- 20.3 mg/l) in comparison to healthy probands (1.

Changes of immunoregulatory properties and induction of autoimmune reactivity to cartilage in rabbits with antigen-induced arthritis.

The chronicity of the antigen-induced arthritis is characterized as dependent on the development of cell-mediated immunity to the antigen, but the exact mechanisms underlying are unclear. We have evidenced decreased suppressor and increased helper cell potential in the early phase of arthritis as result of the immunization procedure. In the late phase of arthritis proliferative responses of spleen lymphocytes to cartilage proteoglycans were revealed which were neither present in immunized animals without arthritis induction nor in the early phase of arthritis.

Significance of cell-mediated and humoral immunity in the acute and chronic phase of antigen-induced arthritis in rabbits.

In the course of antigen-induced arthritis of rabbit cell-mediated and humoral immune responses were repeatedly tested in order to prove their significance for the acute and chronic phase of inflammation. The arthritis was monitored during the progression of the inflammation by means of the joint swelling and at the end of experiments by histological evaluation of synovitis and cartilage degradation. Following the arthritis induction a strong increase of specific antibodies and of circulating immune complexes was evident.

Myocardial bioptic findings in correlation with TL-201 imaging and hemodynamic parameters in patients with latent cardiomyopathies suspected of having myocarditis.

Sixty-eight patients (24 males, 44 females, mean age 37.2 years) belonging to NYHA classes I and II were investigated. All patients had a nondilated ventricle as well as hemodynamic criteria of "latent cardiomyopathy.

[Origin and significance of a thermostable granulocyte antigen].

In 1972 the thermostabile antigen of granulocytes was for the first time isolated by Thoss and Abendroth from punctates of the joint of patients with rheumatoid arthritis. Its origin from mature neutrophil granulocytes was ascertained by fluorescence-microscopic investigations. In the present paper the existence of thermostabile antigen of granulocytes in neutrophil granulocytes could be confirmed.