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Biomedical subjects

K Andrassy

Publications and source records attributed to K Andrassy.

At least 19 recordsLinked to original sources

[Systemic lupus erythematosus and silicosis].

A 60-year-old stonemason, suffering for many years from joint pains and exertional dyspnoea, developed a high fever with weight loss. Physical examination revealed reddening of light-exposed skin areas, fine rales and overly warm and reddened hand and knee joints. Abnormal laboratory findings were increased erythrocyte sedimentation rate of 66 mm/h, C-reactive protein concentration of 1 mg/dl, haemoglobin of 9.4 g/dl and white cell count of 3300/microliters. Urine contained albumin (100 mg/dl) and cylinders. Titres of both antinuclear and anti-ds-DNA antibodies were elevated (1:2560 and > 97 U/ml, respectively). The chest radiography showed enlarged hili, as well as reticular and nodular shadows which histologically showed silicosis. Systemic lupus erythematodes was diagnosed and the patient was treated with prednisone (2 mg/kg daily), the dosage being reduced to 12 mg daily within 3 months. When the joint pains recurred, azathioprine (50 mg daily) was added for 24 months. At present he is receiving prednisone (12 mg daily) and there has been no recurrence for 4 years.

Diagnosis, Differential

Hemolytic uremic syndrome complicating postinfectious glomerulonephritis in the adult.

Hemolytic uremic syndrome/thrombotic thrombocytopenic purpura is known to occur as a secondary complication of primary renal diseases, specifically of lupus nephritis, membranous glomerulonephritis, and focal segmental glomerulosclerosis. In a patient without a family history of hemolytic uremic syndrome/thrombotic thrombocytopenic purpura we observed biopsy-confirmed postinfectious glomerulonephritis with humps, with simultaneous subendothelial hyaline deposits and fibrinoid thrombi in arterioles. The patient had a history of febrile pharyngitis with diarrhea 10 days prior to renal biopsy. He presented with transient elevation of serum creatinine, a nephritic sediment, and hypertension not exceeding 160/100 mm Hg. The patient also had purpura, transient thrombocytopenia, and signs of intravascular hemolysis. Results of verocytotoxin serology were negative. With the exception of isolated microhematuria, all findings reverted to normal within 8 weeks without any specific treatment. This case illustrates that hemolytic uremic syndrome/thrombotic thrombocytopenic purpura may complicate primary postinfectious glomerulonephritis in adults.

Adult

Compassionate treatment of Wegener's granulomatosis with rabbit anti-thymocyte globulin.

UNLABELLED: The purpose of this study was to evaluate the effect of treatment with anti-thymocyte globulin in patients with Wegener's granulomatosis, untreatable with cyclophosphamide and steroids. MATERIALS AND METHODS: Five patients with active Wegener's granulomatosis, either not responsive to standard therapy, or who could not tolerate alkylating agents received a single course of rabbit anti-thymocyte globulin (ATG). RESULTS: Four patients showed a favourable response to treatment with partial or complete remission of disease activity, during a follow-up period of five to twelve months. One patient had progressive retroorbital granuloma, which resulted in enucleation of the eye. Side effects of the ATG treatment were mild with chills and fever during the first infusion, and development of serum sickness in two patients. Two other patients had labial herpes simplex shortly after the start of treatment, no other infectious complications were seen. CONCLUSIONS: We conclude that ATG treatment seems to be an effective treatment for patients with severe Wegener's granulomatosis.

Adult

[Panarteritis nodosa with kidney and spleen rupture: diagnostic and therapeutic possibilities].

Classic polyarteritis nodosa is a necrotizing vasculitis affecting medium- and small-sized arteries. Renal involvement from aneurysm formation is common and can result in perirenal or intrarenal bleeding. The gold standard of diagnostic procedures is the arteriography. Treatment of choice is a combination of steroids and cyclophosphamide, but in the case of severe hemorrhage surgery is mandatory. Patients with PAN need at least 2 years of long-term therapy; repeated arteriography will establish the efficacy of therapy.

Adult

[Acute myoglobinuric kidney failure as a consequence of autoaggressive behavior in mental retardation].

A 49-year-old female with mental retardation was admitted with suspected renal insufficiency with a raised creatinine (5.1 mg/dl), hyperkalaemia (5.6 mmol/l), and a 12-hour history of diffuse abdominal pain and persistent vomiting. On admission, she had a haematoma around the right shoulder and arm-pit, swelling of the right upper-arm, and severe limitation of movement of the right hand. These injuries were the result of trauma some 5 days previously. She was a long-term inpatient in a psychiatric clinic, with a history of autoaggressive behaviour, which had led to several fractures in the past as a result of falls. The creatinine kinase was elevated to 6680 U/l. The suspected diagnosis of acute oliguric renal failure due to rhabdomyolysis was confirmed by the presence of marked myoglobinuria (409 ng/ml). Because of the delay in diagnosis, acute renal failure developed, and the patient required haemodialysis for 20 days. Because of their many predisposing factors, psychiatric patients represent a special risk group for development of rhabdomyolysis, recognition of which is often delayed.

Acute Kidney Injury

Neutralization of the anticoagulant activity of low molecular weight heparin LU 47311 (Clivarin) in man by protamine chloride.

Bleeding induced by unfractionated heparin (UFH) can be antagonized by protamine as shown by normalization of thrombin time and aPTT. In order to learn about the neutralization capacity of protamine against the anticoagulant effects of LU 47311 a comparison study vs UFH was performed in 12 healthy male volunteers. Whereas the prolongation of aPTT and thrombin time induced by both heparins was reversed, inhibition of anti F Xa activity was not. A anti F Xa activity following injection of UFH was immediately antagonized by only 20-40% with LU 47311. A rebound phenomenon of LU 47311 after protamine chloride was not detected. The platelet system remained unchanged.

Adult

Pharmacokinetics of Clivarin a new low molecular weight heparin in healthy volunteers.

The antithrombotic activity and pharmacokinetics of Clivarin, a low molecular weight heparin was randomly studied in 10 healthy male volunteers. Doses of 20, 40, 60 and 80 anti F Xa U/kg BW were injected intravenously and subcutaneously in crossover fashion. The heparin concentrations were measured by inhibition of clotting assays (anti IIa and anti Xa activities using amidolytic assays and [dilute] thrombin time). The pharmacokinetic profile of Clivarin is characterized by a linear relationship between dose and absorption, relatively low clearance and a long elimination half-life, and a high anti Xa/anti IIa ratio of 5.3.

Adult

Nomenclature of systemic vasculitides. Proposal of an international consensus conference.

The following are some of the conclusions and proposals made at the Chapel Hill Consensus Conference on the Nomenclature of Systemic Vasculitis. 1. Although not a prerequisite component of the definitions, patient age is recognized as a useful discriminator between Takayasu arteritis and giant cell (temporal) arteritis. 2. The name "polyarteritis nodosa," or alternatively, the name "classic polyarteritis nodosa," is restricted to disease in which there is arteritis in medium-sized and small arteries without involvement of smaller vessels. Therefore, patients with vasculitis affecting arterioles, venules, or capillaries, including glomerular capillaries (i.e., with glomerulonephritis), are excluded from this diagnostic category. 3. The name "Wegener's granulomatosis" is restricted to patients with granulomatous inflammation. Patients with exclusively nongranulomatous small vessel vasculitis involving the upper or lower respiratory tract (e.g., alveolar capillaritis) fall into the category of microscopic polyangiitis (microscopic polyarteritis). 4. The term "hypersensitivity vasculitis" is not used. Most patients who would have been given this diagnosis fall into the category of microscopic polyangiitis (microscopic polyarteritis) or cutaneous leukocytoclastic angiitis. 5. The name "microscopic polyangiitis," or alternatively, "microscopic polyarteritis," connotes pauci-immune (i.e., few or no immune deposits) necrotizing vasculitis affecting small vessels, with or without involvement of medium-sized arteries. Cryoglobulinemic vasculitis, Henoch-Schönlein purpura, and other forms of immune complex-mediated small vessel vasculitis must be ruled out to make this diagnosis. 6. The name "cutaneous leukocytoclastic angiitis" is restricted to vasculitis in the skin without involvement of vessels in any other organ. 7. Mucocutaneous lymph node syndrome must be present to make a diagnosis of Kawasaki disease.(ABSTRACT TRUNCATED AT 250 WORDS)

Antibodies, Antineutrophil Cytoplasmic

A role for histones and ubiquitin in lupus nephritis?

Glomerulonephritis frequently develops in Systemic lupus erythematosus (SLE) but the pathogenesis is still poorly understood. Experimental evidence now suggests that histones can participate in immune complex formation in lupus nephritis. In a retrospective study, using samples from Northern and Southern Europe, Japan and South America, we searched for glomerular deposits of histones, both in native and ubiquitinated forms, in renal biopsy specimens from 48 patients with SLE and 70 cases of glomerulonephritis from patients without SLE. Positive glomerular immunofluorescent staining was revealed with rabbit antibodies to synthetic peptide 1-21 of histone H3, 22-45 of ubiquitin and to the branched region of ubiquitinated histone H2A (U-H2A) in 65% (31/48), 29% (14/48) and 54% (26/48) of the cases of SLE respectively. In total positive staining with at least one of the antibodies was seen in 36/48 (75%) cases. The staining was granular in nature and was present in capillary and mesangial areas. Only 3% (2/70) of non-SLE renal biopsies revealed positive staining with the above antibodies. None of the biopsy specimens from SLE patients were positive for ss- or ds-DNA, when tested with intercalating dyes. Serum samples were available from 15/48 SLE cases and were analysed with peptides and parent proteins by ELISA; epitopes in the N-terminal regions of core histones and in the C-terminus of histone H1 were often recognised by IgG antibodies in SLE sera, as was ubiquitin and the branched octapeptide of U-H2A. These results support the notion that the nuclear autoantigens histone and ubiquitin may be involved in the induction of glomerulonephritis in human SLE.

Animals

[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

[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

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