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J Mátyus

Publications and source records attributed to J Mátyus.

At least 19 recordsLinked to original sources

[Use of erythropoietin in pregnancy: review of the literature in connection with 2 cases].

Recently a growing number of case reports has been published about successful pregnancy outcome of dialysed women on recombinant human erythropoietin therapy. During pregnancy the maternal demand for erythropoietin may undergo changes, with consideration of recombinant human erythropoietin therapy in the early stage of renal insufficiency, as is shown by our two reported cases. The use of recombinant human erythropoietin seems to be safe for the foetus: it does not cross the placental barrier, and therefore lacks any direct foetal effect. The treatment of anaemia with recombinant human erythropoietin carries benefits for both the mother and foetus. One of the most important preconditions for successful recombinant human erythropoietin therapy is adequate iron supplementation. Due to the increased risk of pregnancy induced hypertension or preeclampsia, there is a need for slow and gradual correction of anaemia, and an individually tailored target hematocrit. A close follow up of he patient by the obstetrical-nephrological team is essential, with the intensive monitoring of the fetuses. In some cases with normal renal function the stimulation of erythropoiesis with recombinant human erythropoietin may also be needed during the pregnancy.

Acute Kidney Injury

[Interferon therapy in cryoglobulinemic membranoproliferative glomerulonephritis associated with hepatitis C virus infection].

The authors report the case of a 38 year old man with horseshoe kidney who developed a severe nephroso-nephritis syndrome, caused by cryoglobulinemic membranoproliferative glomerulo-nephritis. A combination of steroid and cyclophosphamide treatment resulted in partial improvement, but was discontinued after 12 weeks due to adverse reactions, with a consequent early relapse. The 4 week course of cyclosporine monotherapy proved ineffective and signs of cryoglobulinemia appeared. The elevation of transaminase, manifested during the immunosuppressive therapy demonstrated the presence of underlying chronic C hepatitis. In the light of the liver biopsy result, interferon treatment was commenced at a dose of 3 million unit thrice weekly. After 4 months of interferon treatment the persistent nephrotic range proteinuria decreased to below 0.5 g/day. Four months later clinical signs of cryoglobulinemia disappeared, and after the 10th month of interferon treatment no cryoglobulin could be detected in the patient's sera. After one year, the interferon treatment was discontinued following a negative PCR result for HCV. However, one month later the proteinuria increased and the quantitative hepatitis C virus nucleic acid test in sera became positive again. Our case demonstrates that interferon therapy may be effective in the treatment of cryoglobulinemic glomerulonephritis responding poorly to the immunosuppressive therapy, though larger doses or longer periods of treatment may be required to prevent relapses.

Adult

[Amyloidosis associated with dialysis].

Recently dialysis related amyloidosis has become a major complication in patients treated with long-term dialysis therapy. The serum level of the amyloid precursor beta 2-microglobulin is significantly elevated in uraemia, mostly due to the retention. The bioincompatibility of dialysis membranes and the endotoxin content of the dialysate may contribute to the synthesis and tissue deposition of beta 2-microglobulin, but the details of pathogenesis are not yet cleared. At first periarticular and perineural structures are involved in the deposition of amyloid. The carpal tunnel syndrome is of great differential diagnostic value, it appears frequently together with the beginning of the joint pain. The main target of arthropathy are the large and medium-sized joints symmetrically. Deposition of the amyloid to the subchondral bone cysts might lead to pathological fractures, mainly in the hips and destructive spondylarthrophathy might involve severe neurologic complications. Visceral organs (gastrointestinal and urogeniteal tract, heart etc.) are involved rarely and later. Ultrasonography and isotope methods in addition to the conventional radiologic examinations are also used to differentiate the joint complaints nowadays. The definitive diagnosis is based on immunohistology. The alteration of dialysis strategy first of all the usage of high permeable, biocompatibile membranes and pure dialysis water has a role in the prevention of disease and decreasing its progression. In the case of developed lesions timely surgical-orthopedic interventions are required in addition to drug therapy. Todays' renal transplantation is a successful treatment, but the consequences of amyloid depositions already formed can't be left out of considerations even after transplantation.

Amyloidosis

[Pseudoporphyria].

A 46-year-old patient with chronic renal failure receiving maintenance haemodialysis for 3 years had a few months history of blister formation and skin fragility involving the face, arms and dorsa of the hands. In this case clinically mimicking porphyria cutanea tarda (PCT) no demonstrable abnormality in the porphyrin metabolism excretion was detected.

Diagnosis, Differential

[Recombinant human erythropoietin in the therapy of anemia in hemodialyzed patients].

The authors reported on a three month long EPREX (human recombinant erythropoietin) therapy of 5 hemodialysis patients for the treatment of their anemia. The drug was administered in bolus form 2 or 3 times a week after dialysis in a dose of 50 to 150 IU/bodyweight increased gradually in every (or every second) week. Hgb ad Htk values were determined once a week while erythrocyte, leukocyte, thrombocyte and reticulocyte count once a month. Serum iron, TIBC, serum ferritin, BUN, serum creatinine, urea, serum ions, liver function assays, serum lipids and amylase were also established. Hgb, Htk levels and reticulocyte count have significantly increased in the 4th week of treatment already, severe anemia ceased with improved appetite, general condition and physical strength. Serum urea and LDH levels significantly increased while SGOT decreased. No significant change in leukocyte and thrombocyte count, serum Na, K, Ca, P, Cl, BUN, creatinine, total protein level, serum albumin, bilirubin, alkaline phosphatase, GGT, GPT, amylase and blood sugar as well as serum lipid level were observed. No adverse reactions occurred during the treatment. After the three gradually decreased and within 6 weeks they had to be transfused again. In three patients the need for transfusion has significantly grown after the treatment. The authors consider EPREX a highly efficient drug in the treatment of anemia in dialysis patients.

Anemia

Use of tensiomin (captopril) in the antihypertensive treatment of haemodialysis patients.

The observations of a 16-week Tensiomin therapy of 10 hypertensive patients treated with hemodialysis have been discussed. The patients have been treated for about 5 years with hemodialysis, suffered from anuria and required besides systhematical ultrafiltration a combination antihypertensive therapy. Tensiomin was combined with Minipress, Trasicor, Depressan, Estulic and Corinfar by using three- or four-drug combinations. In the course of the administration of Tensiomin the doses of the other antihypertensive drugs could be decreased by 50% on average, while the blood pressure of the patients was normalized. By controlling the patients on weeks 1, 4, 12 and 16 of therapy toxic side-effects or notable pathological changes of the examined laboratory parameters (WBC, serum total protein, Na, K, Ca, P, bilirubin, blood sugar and SGOT values) were not seen. It has been concluded that Tensiomin is an effective drug in combination therapy applied for normalizing the hypertension of dialysed patients.

Adult

[Bone scintigraphy in uremic osteodystrophy].

99mTc-HEDP bone scan was carried out on 12 long-time haemodialysed patients, suffering from bone pains. X-ray examinations of the bone and laboratory tests (serum calcium, -phosphor, -alkaline phosphatase, -parathormone, -aluminium, -ferritin) were also performed. The scintigrams were evaluated by two semiquantitative scores. Based on diffuse, increased radiopharmacon uptake of the bones and more than five points in the Fogelman score 5 patients most likely had serious and 3 had moderate hyperparathyroidism. In two patients osteomalacy was presumed based on decreased radiopharmacon uptake of the bones, increased uptake of the soft tissues and zero Fogelman score. Mixed or other bone disease was suggested in two other patients. Good correlation was found between the results of bone scans, the parathormone values and the results of histology obtained after parathyreoidectomy of 4 patients and autopsy of two others. This non-invasive examination (ie. bone scan) is helpful in differential diagnosis of uraemic osteodystrophy and its wide use is proposed in domestic nephrological practice.

Adult

Ultrasonographic test of subcutaneous arteriovenous Cimino fistula.

A total of 168 chronic dialysed patients were subjected to echography; 48% of them presented aneurysmal alterations of the fistula, with a tendency to increase in proportion to the length of the dialysis period. Two examination methods, the audial and the visual, furnish information on the flow within the fistula, the quality and lumen of the created anastomosis, blood yield, formation and position of collateral circulation. The authors emphasize the advantage of the non-invasive technique as against examination with contrast medium, the early performance of anticoagulation, and the significance of thrombectomy to spare a reoperation of the fistula.

Adult

Serum cholesterol and triglyceride levels in progeria as a model of ageing.

Hutchinson-Gilford progeria was observed in two brothers. Their parents, sister and other relatives did not show any signs of this illness. Serum total cholesterol and total triglyceride levels were normal in the whole family. The serum high density lipoprotein cholesterol (HDL-C) level of parents was low and that of boys was extremely low. The serum HDL-C level of the healthy sister and other relatives was normal. These findings in homozygous children and heterozygous parents may explain the development of the very early fatal arteriosclerosis described in this disease. The connection between the disorder of the lipid metabolism and progeria can serve as a useful model in the study of the role of lipid metabolism in normal ageing.

Aging