Search PubMed⌕ Search

Biomedical subjects

K Andrassy

Publications and source records attributed to K Andrassy.

At least 73 records · Page 4Linked to original sources
PubMed1994

[Diagnosis and therapy in systemic vasculitis with renal involvement].

The diagnosis of Wegener's granulomatosis, microscopic polyarteritis and idiopathic rapidly progressive glomerulonephritis as the main cause of renal involvement in systemic necrotizing vasculitis was revolutionized following the introduction of the ANCA test. The different clinical manifestations of these diseases are described. By means of the ANCA test it could be demonstrated that the pulmonary-renal syndrome was mainly caused by these vasculitides and not by Goodpasture's syndrome. Cyclophosphamide and steroids remain the drugs of choice in the treatment. They can be administered in different ways depending upon the clinical manifestation of the diseases. As therapeutic alternatives cyclosporin, trimethoprim/sulfamethoxazole, intravenous immunglobulins and monoclonal antibodies are discussed.

Anti-Glomerular Basement Membrane Disease↗
PubMed1994

[Different autoimmunity in Wegener's granulomatosis and microscopic polyangiitis].

Wegener's granulomatosis (WG) and microscopic polyangiitis (MP) are "small vessel" vasculitides which can serologically be discriminated measuring autoantibodies directed towards different target antigens (ANCA). In the present study we can demonstrate increased autoimmunity in patients with MP, as evaluated by measurement of thyroid microsomal and cardiolipin antibodies. Neither thyroid microsomal- nor myeloperoxidase-, nor cardiolipin antibodies were correlated to each other.

Adult↗
PubMed1994

Postinfectious glomerulonephritis--is there a link to alcoholism?

From January 1984 to May 1993, we observed 30 cases of postinfectious glomerulonephritis (GN)--endocapillary, exudative GN with humps (23 males, 7 females; median age 49 years; range 17-77). They represented 4.5% of all renal biopsies. Crescents were present in 9/26 who had renal biopsies (35%) and there was a mesangioproliferative pattern in 14 (54%). Seventeen of the 30 patients (57%) were alcoholics by history and biochemistry. Cirrhosis was present in 8/17 (47%), but alcoholic hepatitis in none. Nine of the 17 alcoholic (53%) but none of the non-alcoholic patients developed chronic renal failure. Adverse renal prognosis was significantly correlated to alcoholism. We conclude that (i) alcoholism is common in patients with postinfectious GN, and, (ii) alcoholism adversely affects renal prognosis in patients with postinfectious GN.

Adolescent↗
PubMed1993

[Successful lysis therapy in acute unilateral renal vein thrombosis].

A nephrotic syndrome developed in a 50-year-old man who, because of rheumatoid arthritis for the last three years, had been receiving gold therapy (30-50 mg sodium aurothiomalate weekly for 10 months). Treatment for the nephrotic syndrome was initiated with 100 mg prednisone daily. Ten days later he complained of severe pain in his right flank and haematuria was noted. Serum creatinine concentration increased from 1.0 to 1.8 mg/dl, while creatinine clearance fell to 62 ml/min. Computed tomography demonstrated significant enlargement of the right kidney and a thrombus in the right renal vein which extended cranial into the inferior vena cava. High dosage infusion of urokinase (4.5-7.5 mill. IU daily for nine days) achieved complete lysis of the thrombus. The creatinine concentration fell to 1.1 mg/dl, while creatinine clearance rose to 104 ml/min. On the 5th day the right kidney had 25% of total function, several days later 40%.--This case illustrates that, as long as there are no contraindications, adequately high doses of urokinase can be appropriate treatment of acute renal vein thrombosis associated with the nephrotic syndrome.

Acute Disease↗
PubMed1993

The value of indirect immunofluorescence and solid phase techniques for ANCA detection. A report on the first phase of an international cooperative study on the standardization of ANCA assays. EEC/BCR Group for ANCA Assay Standardization.

This study describes the results of phase I of an international effort to develop and standardize assays for the detection of anti-neutrophil cytoplasmic antibodies (ANCA). 12 sera, four of which were selected for their potential to cause problems in the detection of various ANCA specificities, were analyzed in the standard indirect immunofluorescence (IIF) test and in ELISAs for ANCA routinely performed in the seven participating laboratories. The IIF methodology differed with respect to the dilution of the serum being screened and the concentration of the conjugate used. Results from sera with high ANCA titers were similar, although the quantitative values could not be compared. In sera containing rheumatoid factor and anti-nuclear antibodies (ANA), ANCA-unrelated staining patterns were observed. Six antigen preparations were used in ELISA for the detection of cANCA. In ELISA with purified proteinase-3 all three cANCA sera were positive, but not anti-myeloperoxidase (MPO) or anti-lactoferrin (LF) positive sera. The other assays were less sensitive or gave inconsistent results. Various preparations of purified MPO and LF used in ELISA were readily recognized by anti-MPO and anti-LF positive sera. From this study it can be concluded that the IIF test, although performed with different methods, shows comparable results using strongly positive sera. In general solid phase assays for cANCA detection are not well standardized and need improvement although the purified proteinase-3 ELISA is possibly an exception. MPO and LF can be used in ELISA procedures for the detection of pANCA-related antibodies.

Antibodies, Antineutrophil Cytoplasmic↗
PubMed1993

Presence of anticardiolipin antibodies discriminates between Wegener's granulomatosis and microscopic polyarteritis.

ANCA-tests are not always useful in evaluation of disease activity of patients with Wegener's granulomatosis (WG) and microscopic polyarteritis(MP). If ANCA-tests are failing in patients with Wegener's granulomatosis or microscopic polyarteritis, markers of activated coagulation are helpful in evaluation of disease activity. The measurement of anticardiolipin antibodies(ACA) may allow to discriminate between MP and WG, since ACA are only significantly elevated in patients with MP. Anticardiolipin antibodies (ACA) are acquired autoantibodies that are found in patients with a wide spectrum of clinical conditions, mainly systemic lupus erythematosus and related disorders. They are associated with increased thrombotic tendency. We investigated in patients with ANCA-positive vasculitis whether membrane phospholipids exposed in injured endothelial cells might stimulate the formation of ACA, thus contributing to the vascular thrombotic occlusion besides the activation of coagulation.

Adult↗
PubMed1993

Prevalence of ANCAs in patients on maintenance haemodialysis.

ANCA analysis as evaluated by concomitant determination of ANCA-IF, PR3-AB and MPO-AB in a larger cohort of dialysis patients disclosed that the prevalence of Wegener's granulomatosis and microscopic polyarteritis in Germany is 4 fold higher than hitherto believed. Low ANCA-IF titers without measurable PR3-AB or MPO-AB are not pathological- they resemble an increased autoimmune tendency of the dialysis patients. According to the european dialysis and transplantation association (EDTA)-registry the incidence of Wegener's granulomatosis (WG) and microscopic polyarteritis (MP) on haemodialysis (HD) in Germany is 0.5% and the prevalence is in the same range (1). In contrast to Germany the incidence in France and Great Britain is above 2%. This is mainly based on histologically proven diseases, which can identify only 1/3 of all affected cases, corresponding to our own experience. With the advent of ANCA serology, WG and MP can be diagnosed more easily. C-ANCA with proteinase 3-antibodies (PR3-AB) is typical of WG, and p-ANCA with myeloperoxidase-antibodies (MPO-AB) is typical of MP in our country. Since a considerable number of patients are on chronic HD without knowledge of their underlying renal disease we studied the prevalence of ANCAs in a larger cohort of HD patients.

Adult↗
PubMed1993

p-ANCA with myeloperoxidase antibodies and c-ANCA with proteinase 3 antibodies define a different vasculitis entity in patients with renal involvement.

In a 3 year period 359/3685 patients with suspected vasculitis had a positive ANCA-IF. C-ANCA were found in 146/359 and p-ANCA in 213/359 patients. P-ANCA and MPO-antibodies were more frequently associated with female gender and preglomerular vasculitis, whereas c-ANCA (WG) were more frequent in males without renal vasculitis. Detection of anti-neutrophil cytoplasmic antibodies (ANCA) has facilitated the diagnosis of Wegener's granulomatosis (WG) and microscopic polyarteritis (MP), important representatives of small vessel vasculitides (SVV). C-ANCA with proteinase 3 antibodies (PR 3-AB) are typical of WG and p-ANCA with myeloperoxidase antibodies (MPO-AB) are typical of MP in our country. We reported recently about an association between crescentic glomerulonephritis, renal vasculitis, p-ANCA, myeloperoxidase-AB and female gender in a small cohort of patients with rapidly progressive glomerulonephritis (RPGN). In the following we analyzed whether in a larger cohort of patients with SVV this preliminary statements could be confirmed.

Antibodies, Antineutrophil Cytoplasmic↗
PubMed1993

Scleroderma renal crisis as a presenting feature in the absence of skin involvement.

A 56-year-old man was admitted to the nephrology unit with a short (6-week) history of severe hypertension that necessitated renal replacement therapy within 7 days after admission. Renal biopsy showed features of thrombotic microangiopathy in arterioles and small arteries with occluding thrombi. The skin was unremarkable at the time of admission. Progressive skin lesions with scleroderma, telangiectasia, sclerodactyly, and generalized cutaneous sclerosis developed within 4 weeks and the specific skin changes were found on skin biopsy. On admission antinuclear antibody titers were high (1:10, 240) with a nucleolar pattern, and PM-Scl antibodies (1:5, 120) were present. In the present case the diagnosis of scleroderma renal crisis was made in vivo by renal biopsy. Renal and skin biopsies documented that renal lesions may precede the clinically manifest skin lesions of progressive systemic sclerosis.

Biopsy↗
PubMed1993

In situ production of TNF-alpha, IL-1 beta and IL-2R in ANCA-positive glomerulonephritis.

Humoral and cellular immune mechanisms are thought to be involved in various forms of vasculitis and glomerulonephritis. Recent clinical and experimental results point to a role of cytokines in ANCA-positive vasculitides. We analyzed tumor necrosis factor-alpha (TNF-alpha), interleukin-1 beta (IL-1 beta) and interleukin-2 receptors (IL-2R) in renal biopsies and in plasma from 22 patients with Wegener's granulomatosis and microscopic polyangiitis. Kidney biopsies were examined by immunocytochemistry, polymerase chain reaction and in situ hybridization. Immunoreactive TNF-alpha, IL-1 beta and/or IL-2R positive infiltrating cells were observed in 21 of 22 biopsies. TNF-alpha, IL-1 beta and IL-2R staining was evident in the interstitium and at periglomerular and perivascular sites. The number of positive cells was markedly increased in biopsies with active lesions. Positive cells were also present in cellular and fibrocellular crescents, surrounding tuft necrosis and in the walls of arteries and arterioles with acute vasculitic lesion. Some tubular epithelial cells stained for TNF-alpha and IL-1 beta. TNF-alpha, IL-1 beta and IL-2R positive infiltrating cells correlated with the presence of histologically active renal lesions. The evaluation of TNF-alpha and IL-1 beta expression at the mRNA level assessed by the polymerase chain reaction demonstrated specific transcripts for TNF-alpha and IL-1 beta in all six cases analyzed. In situ hybridization studies showed an increased expression of mRNA for TNF-alpha and IL-1 beta in infiltrating mononuclear cells, in epithelial cells of Bowman's capsule and in some tubules, predominantly of patients with active renal lesions. The results at the mRNA level correlated with the immunocytochemical findings. Compared to healthy individuals higher TNF-alpha plasma levels were observed in patients with vasculitis (34.4 +/- 16.6 pg/ml (SEM) vs. 1.9 +/- 0.7 pg/ml in controls; P < 0.01). All patients presented a marked increase in sIL-2R plasma levels (3512 +/- 485 U/ml vs. 397 +/- 21 U/ml in healthy controls; P < 0.001). IL-1 beta was not detected in most plasma samples. Elevated TNF-alpha and sIL-2R plasma levels were related to active renal lesions. Our study clearly demonstrates that in ANCA-positive vasculitis TNF-alpha and IL-1 beta are produced in situ by activated infiltrating mononuclear cells and resident renal cells. Intrarenal localization of cytokine producing cells and the correlation between cytokine production and histological signs of activity suggest that TNF-alpha and IL-1 beta are important locally acting mediators in the vasculitic/glomerulonephritic process.

Adolescent↗
PubMed1993

Low molecular weight heparin and haemodialysis: neutralization by protaminchloride.

Unfractionated heparin (UFH) is the most widely used agent in preventing clot formation in the extracorporeal circuit. The dose of heparin varies and is dependent on biocompatibility of membranes, the construction of the dialyser and the roller pump segment besides the individual patient's sensitivity. The risk of bleeding might be increased in patients with acute renal failure, particularly with multiple organ failure. Alternative strategies were elaborated, one of which is the use of low molecular weight heparin (LMWH). LMWH has a similar antithrombotic activity as UFH but a lower haemorrhagic tendency and a longer plasma half-life in patients with renal insufficiency. Various studies with LMWH--which are only comparable if the same dialyser and equipment are used--clearly show that the anti-factor Xa level must exceed 0.5 U/ml to prevent clot formation. With these levels LMWH is as effective and safe as UFH in inhibiting coagulation during chronic dialysis. Anti-factor Xa levels > 0.5 U/ml will, however, simultaneously increase bleeding tendency, in patients at risk. Therefore, lower doses of LMWH have to be administered to patients with acute renal failure and risk of bleeding. This haemorrhagic tendency may be neutralized by protamine. The present investigation with protamine chloride and different concentrations of LMWH (Clivarin) shows that clotting tests (thrombin time, aPTT) were immediately antagonized. Anti-factor Xa activity was neutralized by only 20-40%. The gradual decline of anti-factor Xa activity thereafter corresponds to the biological half-life of Clivarin. Whether the remaining anti-factor Xa activity is associated with any increased bleeding risk remains to be seen.

Biocompatible Materials↗
PubMed1993

ANCA in haemodialysis patients.

The prevalence of positive ANCA as well as the prevalence of PR-3 and MPO antibodies were examined in a cross-sectional sample of 1277 haemodialysis patients from 16 German haemodialysis centres. We found 32 patients positive for c-ANCA (median titre 1:40; range 1:20-1:320) and 65 for p-ANCA (1:80; 1:20-1:1280). Twenty-two percent of the c-ANCA-positive and 31% of the p-ANCA-positive patients had PR-3 and MPO antibodies by ELISA respectively. Clinical evidence of vasculitis was found in 11 of 32 c-ANCA-positive and 19 of 65 p-ANCA-positive patients. Of the 11 c-ANCA-positive, four had a known diagnosis of Wegener's granulomatosis (WG); WG was recognized after the test in a further five patients and two had renal limited RPGN. Of the 19 p-ANCA-positive patients, three had a clinical diagnosis of microscopic polyarteritis (MP), MP was newly diagnosed in a further 12, WG in one and renal limited RPGN in three. The patients had not received cyclophosphamide (the diagnosis had been non-specified 'systemic disease'). Thus false-positive ANCA, as defined by absence of vasculitis, was found in 5% of dialysis patients versus 0% in patients with preterminal renal failure (n = 152) or blood donors (n = 150). Patients with vasculitis tended to have higher c-ANCA and p-ANCA titres respectively, but there was a considerable overlap. Titres were not higher in patients symptomatic at the time of examination (6 of 11 c-ANCA and 10 of 19 p-ANCA), but PR-3 and MPO ELISA were positive in all but two.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1993

[Silicosis and systemic diseases].

We present case histories of 5 patients with silicosis who developed systemic lupus erythematosus and microscopic polyarteritis in two cases each, and rapidly progressive glomerulonephritis (limited Wegener's granulomatosis) in one case. An association between silica exposure and autoimmune disease is emphasized.

Aged↗