Small vessel vasculitis with crescentic glomerulo-nephritis associated with IgA-lambda monoclonal gammapathy.
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Biomedical subjects
Publications and source records attributed to M Polenakovic.
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A case of acute poststreptococcal glomerulonephritis following circumcision is presented. An 11-year-old boy was subjected to ritual circumcision, which was complicated by the infection of the wound and development of oliguria, edema, hematuria and hypertensive encephalopathy 2 weeks later. The diagnosis of poststreptococcal glomerulonephritis was established upon the isolation of Streptococcus pyogenes, increased antistreptolysin O (ASTO) and antiDNAse B titers and hypocomplementemia. The clinical course was uneventful with resolution of the nephritic signs, normalization of the complement and clearance of the urinary abnormalities. To the best of our knowledge this is the first case of poststreptococcal glomerulonephritis following infection of the circumcision wound.
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The situation of end-stage renal disease (ESRD) patients in central and eastern Europe was very poor for many years during the so called socialistic era. Economical and political liberation resulted in the significant growth of renal replacement facilities in this region. The number of hemodialysis units increased significantly (56%) during the period 1990-1996, and the number of patients treated with this modality has risen by 75%. More dramatic progress was achieved in peritoneal dialysis. The number of units performing this method of renal replacement therapy (RRT) increased by 277% and the number of patients by more than 300%. Not only quantitative but also qualitative changes were observed. More modern hemodialysis machines installed in the vast majority of units allow for the performance of bicarbonate dialysis, controlled ultrafiltration, and sodium profile modeling. Also, a wider choice of biocompatible dialyzers has become available during the last few years. The number of centers performing renal transplantation has increased significantly, but the number of renal transplants has not followed this progress. Despite all the progress, further development of all RRT methods is necessary to achieve acceptance rates comparable to those observed in developed countries.
PURPOSE OF THE STUDY: The conditions of renal replacement therapy (RRT) were very poor in the countries located in Central and Eastern Europe (CEE) when they were members of the so-called 'socialist bloc'. The aim of the present analysis was to document the impact of the socioeconomic changes on dialysis therapy in the CEE countries. DESIGN: This was a special survey with the participation of 12 CEE countries, with data obtained through national registries (with the exception of Russia). RESULTS: During the period 1990-1996 the number of haemodialysis units increased by 56% and the number of centres performing peritoneal dialysis by 296%. The number of patients increased respectively by 78% (haemodialysis) and 306% (peritoneal dialysis). The percentage of patients with diabetic nephropathy and elderly patients rose dramatically during this period. One of the main reasons of such expansion was the rapid development of peritoneal dialysis programmes in the majority of the CEE countries. The introduction of modern haemodialysis machines and a wider choice of different dialysers and concentrates permitted individualization of dialysis procedures. These points and the wider use of erythropoietin had a positive influence on quality of life and treatment outcome. There was also a notable increase in the number of transplant centres, but less so of the number of transplanted patients. CONCLUSION: Renal replacement therapy experienced a major expansion in the CEE countries. Despite the progress achieved, the level of RRT is not yet completely satisfactory in most CEE countries.
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In a prospective study, 40 maintenance hemodialysis patients, randomized in two equal groups, were treated with recombinant human erythropoietin (rHuEPO) for their renal anemia, for a period of 2 years. One group was treated for 2 years, while the other was untreated control during the first year, but received rHuEPO during the second year of the study. Anemia was corrected in all treated patients and hematocrit maintained between 30 and 35 vol% by low-dose subcutaneous treatment with Recormon (Boehringer Mannheim GmbH, Germany), according to the study protocol. Bone marrow biopsy (BMB), from the posterior iliac crest, was taken by the method of Jamshidi from 32 patients. Fourteen patients from the control group were biopsied twice: once at baseline and the second time at 12 months of treatment, while 15 patients from the other group were biopsied only once, at 24 months of rHuEPO treatment. The biopsies were embedded in wax and in epoxy resin, and after staining for light and electron microscopy, they were semiquantitatively examined for several parameters: cellularity, myeloid:erythroid (M:E) ratio, megakaryocytes, fatty tissue, megaloblasts, and marrow iron. Cellularity of the bone marrow increased significantly at 12 months of treatment and it remained so at 24 months. M:E ratio was significantly reduced indicating expansion of the erythroid pool, both at 12 and 24 months of therapy. The number of megakaryocytes in the bone marrow increased significantly at 12 months and remained high at 24 months of treatment, while fatty tissue was significantly reduced at 12 and 24 months compared to the baseline values. There was no significant change in the percentage of megaloblasts in the bone marrow. Hemosiderin was reduced after treatment indicating mobilization of the bone marrow iron stores upon treatment with rHuEPO. We concluded that rHuEPO had a beneficial long-term effect on bone marrow.
We report on two families from the southern part of Former Yugoslav Republic of Macedonia (close to Mediterranean area) with autosomal dominant inherited nephrotic syndrome, documented with renal biopsy in three family members. Histopathological examination confirmed microcystic tubular changes and focal segmental glomerulosclerosis. In biopsied patients proteinuria was noted in the first decade, or second, but it was quantitatively documented as nephrotic in the second and third decade. The prognosis of the patients was poor: one patient died because of intracranial hemorrhage before developing end stage renal disease; the other two cases developed end stage chronic renal failure in the third and fourth decade.
Acquired renal cystic disease (ARCD) is a well documented complication of end-stage renal disease, and it has been related to the duration of dialysis therapy. The association of this condition with renal cell adenoma or carcinoma has already been established. There have also been studies on the concentration of some tumor markers in hemodialysis (HD) patients, clinically free from neoplastic disease, where it was concluded that some tumor markers could be elevated, despite the absence of malignant disease, suggesting their altered metabolism i.e. clearance by the hemodialysis membrane. We compared the pre-dialysis serum concentration of several tumor markers in three groups of chronic HD patients, all of whom had been on maintenance HD treatment for more than 5 years. Group 1 consisted of 16 patients without ARCD with a mean HD treatment duration of 97.06 +/- 28.25 months. Group 2 consisted of 32 patients with a mean HD treatment of 105.62 +/- 24.4 months, who had ARCD with less than 10 renal cysts detected by ultrasonography. Group 3 consisted of 14 patients with a mean HD duration of 109.92 +/- 37.72 months, with ARCD and more than 10 renal cysts. Concentration of the following tumor markers was determined by EIA or ELISA methods: carcinoembryonic antigen (CEA), mucin-like carcinoma-associated antigen (MCA), neuron-specific enolase (NSE), carbohydrate antigen 19-9 (CA 19-9), prostatic specific antigen (PSA), carbohydrate antigen 125 (CA 125), alpha fetoprotein (AFP), cytokeratin 19-fragments 21-1 (CYFRA 21-1). The concentration of all the tumor markers was comparable in all three patient groups, with no statistically significant difference between groups. The mean concentrations of MCA, PSA, CA 125 and AFP were within the normal range. CEA and CYFRA 21-1 had mean values in the upper limit of their normal values, while NSE and CA 19-9 were increased by more than twofold in all three patient groups. We concluded that (i) tumor markers should be used with caution when diagnosing neoplastic diseases in chronic HD patients, because of their altered metabolism, and (ii) that in the follow up of ARCD with possible neoplastic alteration, imaging techniques remain dominant diagnostic tools.
Ten patients with chronic renal failure from different genesis (serum creatinine levels 150-200 mumol/l), were evaluated from the aspect of the effect of the diuretic therapy. The effects of furosemide (FUR) and polythiazide (POL) were assessed after 3-month application. The mean values of the estimated parameters before treatment, after 3-month administration of FUR as a monotherapy and after the next 3 months simultaneously used (FUR + POL), presented a stable increase of the diuresis, without statistically significant changes of the global renal function, and triglyceride disorders. On the contrary, the improvement of calciuria through combined using of furosemide and polythiazide is statistically and clinically significant.