Prednisone/cyclophosphamide treatment in adult-onset autosomal dominant familial focal segmental glomerulosclerosis (FSGS 1).
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Administration, Oral↗
Biomedical subjects
M Polenakovic
Publications and source records attributed to M Polenakovic.
Infection as a risk factor in the outcome of patients with acute renal failure assessed by SOFA score.
Acute renal failure (ARF) is a complex syndrome, frequently reported with mortality in 20-80% of the patients. Infection, as a cause or a complication of the syndrome, is a risk factor which unfavourably determines the outcome. A prospective 4-year study was performed of 112 ARF patients in the intensive care unit (ICU), with an evaluation of 68 clinical and laboratory parameters, as risk factors, at admission to the ICU. The scoring system used was SOFA (Sepsis-related Organ Failure Assessment). The outcome was associated with 27 risk factors among which infection as an acute ARF insult has shown a particularly strong correlation (p<0.0001). There was no association with infection as an intra-hospital complication. When divided according to the outcome (survivors vs. non-survivors), platelets, hematocrit, total protein and mean arterial pressure were significantly higher, while leucocytes and bilirubin significantly lower in the surviving group of patients. The parameters of the SOFA scoring system, such as the coagulation, liver and cardiovascular code, were statistically significant in relation to the outcome, and also the SOFA score taken as an independent variable (p<0.0001). The classification matrix of the analyzed SOFA scoring system was successful in the classification of 91.95% of the surviving and 48.00% of the non-surviving patients with ARF and the odds ratio was 41.0. Mortality rate in patients with ARF is 22.7%. Patients with ARF due to sepsis have a worse prognosis than those with non-septic ARF. Coagulation disorder, liver and cardiovascular dysfunction, as well as the SOFA score itself are independent variable in the outcome prediction. The SOFA score, derived from easily obtained data, is useful for judging survival prognosis in patients with ARF.
Acute Kidney Injury↗
Therapeutic apheresis in the Republic of Macedonia - our five years experience (2000-2004).
Membrane plasma exchange (PE) is a mode of extracorporeal blood purification. Since 1985 membrane PE has been in regular use at the Department of Nephrology, Medical Faculty of Skopje, R.Macedonia. In this paper we report on five years (2000-2004) of single centre plasma exchange activity. We performed 540 PE treatments (108 PE/per year) on 99 patients. The M/F ratio was 40/48. The patients underwent a median of 5.45 procedures (range, 1-16). The treated patients were from different Departments. Protocols for PE depend on the disease and its severity. PE were performed 2-4 times weekly using Gambro PF 2000 N filters with an adaptation of the Gambro AK10 dialysis machine or with the Gambro Prizma machine (2 cases). Blood access was achieved through femoral vein. Substitution was made with fresh frozen plasma and/or with 20% human albumin combined with Ringer's solution. An average amount of 2150 ml plasmafiltrate per treatment (respectively 30 to 40 ml plasmafiltrate/kg body weight) was eliminated. Most therapeutic procedures were performed on patients from the Department of Neurology. 63.6% of all patients were referred for Myasthenia gravis and the Guillian Barre syndrome. The total number of procedures per year has remained fairly stable, corresponding to a median of 5.4 treatments/100 000 inhabitants. We observed hypocalcaemia in 8% of the patients, urticarial reactions in 7.3%, pruritic reactions in 12%, and hypotension/headache in 6.8%. No major procedural complications were seen.
Guillain-Barre Syndrome↗
ANCA-GBM dot-blot test in diagnosis of patients with glomerulonephritis.
Patients with rapidly progressive glomerulonephritis who are positive for anti-neutrophil cytoplasmic antibody (ANCA) or anti-glomerular basement membrane (GBM) antibodies may develop chronic renal failure leading to end-stage renal disease (ESRD) within days or weeks. The early serologic detection of auto-antibodies associated with ANCA and anti-GBM diseases will be helpful in preventing ESRD. We evaluated the combined ANCA-GBM dot-blot strip assay (Biomedical Diagnostics, Brugge, Belgium) in 30 consecutive patients with biopsy proven glomerulonephritis (GN). MPO- and PR3-ANCA were detected in 5 and 2 samples, respectively. Three samples were positive for both MPO- and PR3-ANCA (all 3 had focal segmental necrotizing GN). One patient was diagnosed as having Goodpastures' syndrome (the only anti-GBM positive result) and two had Wegener's granulomatosis (the two PR3-ANCA positive results). Two additional samples were equivocal: positive for MPO-ANCA and PR3-ANCA, respectively. Patients positive only for MPO-ANCA had only limited extrarenal organ manifestations. Anti-PR3 positive patients with necrotizing glomerulonephritis had a more dramatic deterioration of their renal function at diagnosis. Radiographically, these patients had nodular or pneumonia-like lesions. Acute respiratory failure necessitating mechanical ventilation was developed in one GBM positive patient. In conclusion, the ANCA-GBM dot-blot is a useful screening tool in situations where conventional ANCA testing is not readily available.
Adult↗
Inflammation predicts all-cause and cardiovascular mortality in haemodialysis patients.
Among non-traditional cardiovascular risk factors both malnutrition and inflammation appear to be strong predictors of mortality and morbidity in haemodialysis (HD) patients. Our study objective was to determine predictors of all-cause and cardiovascular mortality, considering the nutritional and immunologic parameters, in a cohort of HD patients treated in a single haemodialysis centre. 216 patients on HD were analyzed for clinical, nutritional-serum albumen and BMI, immunologic-serum CRP (C-reactive protein) and fibrinogen and dialysis parameters -- ultrafiltration, length of dialysis in hours, HD dose (using spKt/V and eKt/V). Mortality was monitored prospectively over a two-year period. Fifty-five of the 216 HD patients died during the follow-up period and the main cause of death was cardiovascular disease (CVD) -- 33 patients out of 55 (60%), followed by infection/sepsis (13 pts, 24%). The patients who died were significantly older, had a significantly shorter duration of HD in hours, ultrafiltration was significantly less, HD doses were significantly lower, as were serum levels of albumin (36.06 +/- 4.17 vs. 39.74 +/- 3.31; p=0.000) and Hg (93.14 +/- 15.43 vs. 109,16 +/- 12,08; p=0.000), but they had significantly higher serum levels of CRP (40.26 +/- 34.75 vs. 8.71 +/- 7.68, p=0.000) and fibrinogen (5.28 +/- 1.28 vs. 4.42 +/- 0.97, p=0.000). Kaplan-Meier survival estimates showed that the group with the lowest levels of albumin (< 3.5 g/L), and with the greatest levels of CRP (>20 mg/l) and fibrinogen (>5 g/L) had the lowest survival (log-rank test p=0.0008, p=0.00000, p=0.0000). However, in the Cox proportional hazards model, a high CRP and low Hg level (chi-square=96.467, p=0.0000) were predictors of all-cause mortality, whereas serum level of albumin did not show to be predictive. When only cardiovascular mortality is entered into the Cox model, CRP and Hg levels are still more important in predicting mortality (chi-square=70.055, p=0.0000) and only if CRP is not taken into account in the multivariate analysis, serum albumin level remains, after Hg, the strongest predictor for both overall and cardiovascular mortality (chi-square=76,564, p=0.0000; chi-square 50.619 p=0.0000). It can be concluded that inflammation predicted all-cause and cardiovascular mortality in our study group, because high CRP, as a marker of inflammation and low haemoglobin, as a result of inflammation, remained powerful predictors of both overall and cardiovascular death.
Biomarkers↗
20 years after methylprednisolone/chlorambucil treatment in idiopathic membranous nephropathy stage II-III with nephrotic syndrome.
There is now controlled evidence that a 6-month course with methylprednisolone and chlorambucil may favour remission of the nephrotic syndrome and may significantly improve the 10-year kidney survival in patients with idiopathic membranous nephropathy. We analyzed the outcome of 15 nephrotic patients (proteinuria 7.06 +/- 1.07 g/d), stage II-III membranous nephropathy, aged 37.93 +/- 2.32, 8 males and 7 females, with normal serum creatinine (62.8 +/- 2.34 micromol/l), followed > 10 years after the treatment. It consisted of 1g i.v. methylprednisolone for three consecutive days, followed by oral steroids 0.4 mg/kg/d and chlorambucil 0.2 mg/kg/d monthly, alternatively. 10 patients, age and sex matched, who refused any treatment of any reason, represented the control group. Complete remission was defined as protein loss of 0.2 g/d, partial 0.2-2 g/d with normal creatinine and renal dysfunction as increase in plasma creatinine. The follow-up period was between 10 and 20 years. Complete remission after the treatment was noted in 9/15, partial in 4/15, and 2/15 patients did not respond. 10-year survival rate of the whole group was 100%, 15-year - 86.7%, i.e. two patients with persistent nephrotic syndrome developed end-stage renal failure after 12 years. 13/15 patients (complete, partial remission) were followed > 15 years without development of end-stage renal failure. One patient (female, 32) developed idiopathic thrombocytopenia after 8 years. 3 patients (complete remission) were followed > 20 years, they are still without proteinuria. 10-year survival rate of untreated patients was 40%. It is concluded that in nephrotic patients with stage II-III membranous nephropathy steroids/chlorambucil therapy may be effective in favoring remission and in preserving renal function.
Adult↗
IgA nephropathy: 23 years of follow-up.
IgA nephropathy (IgAN) is the most common primary glomerulonephritis. Some patients reach end-stage renal failure (ESRF), others experience an indolent course. We aimed the study to examine the association of risk factors with the progression to renal failure. Eighty patients diagnosed with IgAN by renal biopsy (RB) were studied. Baseline clinical and demographic data were reviewed. Severity of histological involvement was scored as H. S. Lee's grading system. The mean age of patients at biopsy was 36.65 +/- 8.83 years with predominance of men (male : female, 58 : 22). Patients were followed-up from 6 months to 23 years (276 months). An end-point was defined as the date when patient underwent their first haemodialysis or their last visit of follow-up. The differences in means between groups were compared by independent samples t-tests or one-way analysis of variance (ANOVA). Kaplan-Meier survival curves and Cox regression models were used to analyze the time course from renal biopsy to end points. The largest subclasses were grade I and II, with 31 patient each. Subclass III was observed in 12 patents. Subclass IV and V were found in 3 patients each. During the follow-up period, all patients with grade IV and V (after 6-48 months), five patients grade I (after 60-144 months), four patients grade II (after 48-84 months), and 7 patients from grade III (after 24-108 months) entered ESRD. Mean prioteinuria was 1.68 +/- 0.99 g/day. Macrohematuria had 32; microhematuria had 48 pts. The mean serum creatinine was 148.02 +/- 68.76 micromol/l. By multivariate analysis using the Cox regression model, grades, renal insufficiency and significant proteinuria were independent prognostic factors for progressive renal disease. At the end of follow-up, grades were significantly related to serum creatinine, proteinuria, hypertension and progressive renal disease. Renal biopsy in IgAN may be the most powerful predictor for renal outcome.
Adult↗
Early mortality rate in end-stage renal disease patients initiating hemodialysis.
Early referral of patients with chronic renal failure (CRF) to a nephrologist improves morbidity and delays mortality after start of renal replacement therapy (RRT). Late referral is associated with increased cardiovascular morbidity and short-term mortality. The aim of this study was to assess the factors associated with early mortality rate in ESRD patients, during the first month after initiating hemodialysis. One hundred and eighty nine 189 patients hospitalized at the Department of Nephrology in Skopje after starting RRT and followed up during the first month were included in this study. Early referrals were considered those who had been referred to a nephrologist for more than 3 months before initiating RRT. Female to male ratio was 93 to 96 (49/51%). Out of them, 110 were late referrals, and 79 early ones (58/42%). Out of 189, 20 patients died during the first month of follow up (10.6%). 85% of the patients who died were late referrals, not having time and opportunity to be treated for renal anemia, for malnutrition and prevention of cardiovascular and uraemic complications, and all of them lacked permanent vascular access. Early mortality was also associated with older age, renal anemia and malnutrition. Late referrals were also older, predominantly male and had higher serum potassium levels. The main causes of death were related to uraemic intoxication, malnutrition and inflammation as well as lack of permanent vascular access, all of it leading to sepsis, and cardiovascular complications.
Aged↗
Differences in renal registries between the Balkans and Western Europe.
The epidemiology data of renal replacement therapy are collected by national renal registries and extended to international registries as the European Renal Association Renal Registry. The extent and accuracy of data vary widely among countries. The aim of this study was to compare combined data from the national renal registries of Balkan countries contributing the ERA-EDTA Registry or from other sources, with combined data from renal registries of the Western and Northern European Countries and regions contributing the ERA-EDTA Registry. Data regarding incident and prevalent patients in 2003, mean age of incident and prevalent patients, incidence and prevalence by cause of renal failure and prevalence by established therapy were compared between the countries of the Balkan region and the countries of Western and Northern Europe contributing to the ERA-EDTA Registry. Data were obtained by the Annual Report 2003 of the ERA-EDTA Registry, and for Romania by the study of G. Mirescu published in NDT 2004. Some data were obtained by the questionarries sent to people responsible for the national registries. The results showed the following: the incident number of patients per million population (pmp) at day 1 of RRT, as well as at day 91, adjusted for age and gender, did not statistically differ between the Balkans and Western Europe: 119.2 +/-25.1 vs. 123.3 +/- 25.9 and 110.9 +/- 34.7 vs. 106.5 +/- 18.3, respectively. The mean age of incident patients at day 91 of RRT significantly differed between the Balkans and Western Europe, 57.7 +/- 4.49 vs. 63.3 +/- 2.2, p < 0.005. The percentage of incident patients by cause of renal failure at day 91 of RRT did not significantly differ between the Balkans and Europe. The mean incidence of the percentage of DM as a cause of renal failure between the Balkans and Western Europe did not differ, 23.05+/- 4.5 vs. 20.3 +/- 7.2. When adjusted for age and gender, the significant difference in prevalent number of ESRD patients between the Balkans and Western Europe disappeared. The mean age of prevalent patients between the Balkans and Europe did not significantly differ, 54.3 +/- 4.2 vs. 58.2 +/- 2.8. The percentage of primary renal disease in prevalent patients did not significantly differ, except for policystic kidney disease which is significantly more frequent in Western Europe compared to the Balkans, 9.2 +/- 1.9 vs. 6.8 +/- 1.8, p < 0.01. Diabetes mellitus and hypertensive nephropathy are much more frequent in incident patients compared to prevalent ones. Transplantation is significantly more frequent in Western Europe, predominantly cadaveric, whereas dialysis is more frequent in the Balkans. It can be concluded that no difference exists between the incidence and prevalence of diabetes mellitus as a primary renal disease in ESRD patients between the Balkan and European countries, indicating that the epidemic of diabetes is already present in the Balkans and imposing, perhaps, efforts to be undertaken for planning prevention strategies; kidney transplantation is significantly less represented as RRT in Balkan countries compared to Western and Northern Europe, and efforts should be made for its increase, particularly the cadaveric one, and moreover, because the population on RRT is significantly younger in the Balkans.
Europe↗
The evolution of untreated borderline and subclinical rejections at first month kidney allograft biopsy in comparison with histological changes at 6 months protocol biopsies.
Our study sought to identify the possible implications of histological findings of borderline and subclinical rejections as well as histological markers of chronic allograft nephropathy (CAN) in protocol biopsies at 1 and 6 months after living-related kidney transplantation. Twenty-eight paired allograft biopsies were blindly reviewed using Banff '97 criteria, among which only 10.7% (6/56) showed no histopathological lesions. BR was found in 9/28 (32.1%) and 6/28 (21.4%), and SR in 3/28 (10.7%) and 10/28 (35.7%) of the patients, in the 1 and 6 month biopsies, respectively. The mean CAN score (sum of histological markers for chronicity) increased significantly at 6 months biopsy, 1.57 +/- 1.36 vs. 4.36 +/- 2.32 (p < 0.01). When compared according to chronicity index (CI < 5 >), the high CI group had a mean CAN score of 2.36 +/- 1.15 at 1 month, which increased to 5.14 +/- 1.99 at 6 months biopsy (188.9%). The proportion of these changes in low CI group were also increased from 0.79 +/- 1.12 to 3.57 +/- 2.38 (451.9%). In conclusion, a protocol 1 month biopsy may uncover a high prevalence of BR or SR in stable allografts. The presence of an untreated BR or SR in biopsies with low chronicity index showed greater susceptibility to histological deterioration on the 6 month biopsy, associated with rapid impairment of graft function and chronic allograft nephropathy.
Adult↗
Reverse transcriptase/polymerase chain reaction analyses of hemodialysis ultrafiltrates and sera of hepatitis C virus positive patients.
The hepatitis C virus is a highly prevalent infection among chronic dialysis patients and represents one of the major problems of hemodialysis units. Hepatitis C virus transmission occurs either by blood transfusion or nosocomially. One of the proposed pathways of nosocomial transmission of the hepatitis C virus is cross-contamination through the dialysis procedure. In an effort to elucidate whether the hepatitis C virus may pass across the hemodialysis membrane, we have performed analyses of ultrafiltrates collected in different stages of hemodialysis treatments, using different types of hemodialysis membranes and different types of dialysis machines. Samples collected from the dialysis compartment during the rinsing of the blood compartment at the end of the hemodialysis treatment were also analyzed. The hepatitis C virus was found in 17 out of 58 ultrafiltrate samples taken at different times of the hemodialysis treatment. Moreover, the hepatitis C virus was present in 15 out of 17 samples collected from the dialysate compartment during the saline solution rinsing step of the blood compartment. The presence of the hepatitis C virus had no strict correlation with the type of dialysis membrane or with the type of dialysis machine. Although the results suggest that the passage of the hepatitis C virus during the hemodialysis treatment is multi-factorial and case- specific, the most critical point is when the blood is flushed out with physiological saline.
Cross Infection↗
Occurrence of subclinical post-streptococcal glomerulonephritis in family contacts.
OBJECTIVE: There are few data in the published literature on the occurrence of subclinical post-streptococcal glomerulonephritis. In order to estimate the incidence of subclinical disease, 75 families of index cases with sporadic clinical post-streptococcal glomerulonephritis were screened for the presence of subclinical disease. METHODS: Three hundred and seventeen family contacts were investigated 1-7 days after the admission of the index cases. The diagnosis of subclinical disease was based on the presence of abnormal urinalysis, transitory hypocomplementaemia and increased antistreptolysin O titre. RESULTS: No cases of clinical/subclinical disease were detected among 147 parents. Abnormal urinalyses were found in 22.3% of sibling contacts. The incidence of nephritis among 170 siblings was 9.4% and the calculated ratio subclinical/clinical disease was 0.11. There were 16 siblings (9.4%) whose abnormal urinalyses could not be explained by appropriate tests; 11 of them had dysmorphic microhaematuria and significantly elevated antistreptolysin O titre. CONCLUSIONS: Sibling contacts have increased risk for the development of clinical/subclinical post-streptococcal glomerulonephritis compared with their parents. Sibling contacts with unexplained urinary abnormalities might have subclinical nephritis in evolution; their complement levels normalized before occurrence of nephritis in index cases.
Acute Disease↗
Plasmaexchange in the treatment of myasthenia gravis associated with thymoma.
BACKGROUND: All patients with thymomatous Myasthenia Gravis (MG) should undergo early and total thymectomy, but controversy abounds in the choice of chronic immunosuppressive agents. The value of plasmaexchange (PE) in MG has been clearly established in preoperative preparation and treatment of myasthenic crisis. Whether PE may be used in the chronic long-term therapy of patients with thymomatous MG in addition to conventional immunosuppressive agents and cholinesterase inhibitors is yet to be answered. CASE HISTORY: We present a 40-year old woman with an 11 year history of MG. Thymectomy was done during the first year of the disease and the histopathologic finding was thymoma. To sustain clinical remission after thymectomy she continued with immunosuppression with methylprednisolone and cyclosporin A (or azathioprine) in addition to cholinesterase inhibitors. Despite the almost continuous immunosuppression, the disease course continued with fluctuating myasthenic weakness which few times progressed to myasthenic crisis requiring mechanical ventilation. During myasthenic crisis we performed 6-8 plasmapheresis at 2-3 day intervals in addition to conventional immunosuppressive therapy. The disease rapidly worsened in January 2000 and we started with intermittent plasmapheresis (3-6 procedures at 2-3 day intervals, every 6-8 weeks) in order to sustain remission. With this therapeutic protocol, during 20 months follow-up we managed to prevent myasthenic crisis and to avoid ventilatory support. CONCLUSIONS: Plasmaexchange could be used as a successful and safe therapeutic tool in chronic long-term therapy in addition to conventional immunosuppressive agents to sustain remission in patients with MG. This is particularly important in the treatment of patients with thymomatous MG because they have an increased frequency of myasthenic crisis and often respond poorly an to immunosuppression with steroids or other immunosuppressants.
Adult↗
Diabetic glomerulosclerosis without overt diabetes mellitus.
The duration of diabetes mellitus and the presence of hyperglycaemia were thought to be important in the development of nodular glomerulosclerosis. We report on 3 patients without overt diabetes, but with glucose intolerance, all male, aged 43-66 years, with histological features of diabetic glomerulosclerosis. The nodular form was present in 2 patients and the diffuse form in 1. These cases suggest that factor(s) other than hyperglycaemia are responsible for diabetic renal damage.
Adult↗
Epoetin omega for treatment of anemia in maintenance hemodialysis patients.
After the synthesis of epoetins alpha and -beta, a third molecule of recombinant human erythropoietin (rHuEPO) was synthesized and was named epoetin-omega. The molecule of epoetin-omega is a sialoglycoprotein with smaller amounts of O-bound sugars, less acidic and with different hydrophylity than the other 2 epoetins. The purpose of the study was to assess the efficacy, safety and clinical tolerance of epoetin-omega for treatment of renal anemia. In an open-label, uncontrolled prospective clinical study, 22 end-stage renal disease patients (9 male and 13 female) were followed for 6 months. They all had a hemoglobin (Hb) value below 85 g/l, and were on regular hemodialysis therapy 3 times a week, 4 hours per session. The initial weekly dose of epoetin-omega was 90 units per kg of body weight (b.w.) divided in 3 equal portions and administered subcutaneously after each dialysis session. After correction of the hemoglobin, the dose of rHuEPO was individualized to keep Hb within target limits of 100-120 g/l. To follow efficacy and safety, a number of clinical and laboratory parameters were monitored. All patients responded well to the therapy with corrected hemoglobin after the 10th week of the study. The mean dose of epoetin-omega during the correction period never exceeded 100 U/kg b.w. per week. The average maintenance dose of rHuEPO was 50-60 U/kg b.w. per week. Iron was, where needed, supplied intravenously. We noted no change in serum urea. creatinine, phosphorus, and heparin dose per dialysis session. The prothrombin time improved during the study. Serum albumin increased. No change was observed in urea reduction ratio (URR), body weight and mean arterial pressure. One serious adverse event was noted: worsening of hypertension in 1 patient, with the development of hypertensive encephalopathy and severe headache. rHuEPO treatment was stopped. The blood pressure was effectively controlled by reducting her body weight by 5%. Thereafter, rHuEPO therapy was resumed with good blood pressure control. We could conclude that recombinant human erythropoietin-omega was an efficient and safe therapeutic agent for the treatment of renal anemia.
Adolescent↗
Early histopathological changes in diabetic nephropathy.
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Adult↗
Lipids, protein intake, and progression of diabetic nephropathy.
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Diabetes Mellitus, Type 2↗
Therapeutic apheresis in nephrology and neurology.
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Adult↗