The outcome of kidney transplant from living donors with pelvi-ureteric junction dysfunction.

PURPOSE: To assess the effect of receiving a kidney with PUJ dysfunction on the recipient renal graft function. METHODOLOGY: 198 patients, who underwent renal transplantation from 1st January 2004 to 31st December 2014 in a single Center in the North West of England, were retrospectively reviewed using a computerized database. Split kidney function and the PUJ dysfunction for the donors were assessed using Tc-99 m MAG3 renogram. Each recipient with PUJ dysfunction was matched with a control recipient by age, gender, and number of days after transplantation. Both groups were followed up for 3.5 years post-transplantation. RESULTS: Of the 198 recipients included in the study, 19 recipients received kidneys from donors with PUJ dysfunction. Prevalence of PUJ dysfunction was 9.5% and it was more common in males than females. There was no difference between the case group and the control group in terms of age, gender, and follow-up time post-transplantation. There was also no difference between the case group and the control group in mean creatinine (130 µmol/l and 138 µmol/l respectively, p = 0.305) or the mean eGFR (48.6 ml/min and 47.5 ml/min respectively, p = 0.054) at 3.5 year post-kidney transplantation. CONCLUSION: This study showed that PUJ dysfunction of renal allograft has a negligible effect on graft function over 3.5 years period post-transplantation. A prospective randomized trial is needed to test these findings. In the presence of widened gap between demand and supply in renal transplantation, PUJ dysfunction in potential donors should not preclude them from donation.

IgM as a novel predictor of disease progression in secondary focal segmental glomerulosclerosis.

AIM: To determine the role of immunoglobulin M (IgM) deposits in clinical manifestations, disease outcome, and treatment response of idiopathic and secondary focal segmental glomerulosclerosis (FSGS). METHODS: Kidney biopsy specimens of 171 patients diagnosed with FSGS (primary and secondary) and 50 control patients were retrospectively included in the study. For each patient, clinical and outcome data were obtained and compared to morphological parameters, including immunofluorescence analysis of mesangial IgM and complement 3 (C3) deposits analyzed on kidney biopsy samples. RESULTS: There were significant positive correlations between IgM and C3 deposition in secondary FSGS (P<0.001) and between IgM and mesangial deposits detected by electron microscopy in secondary FSGS (P=0.015), which indicated that higher IgM deposition correlated with higher C3 deposition and mesangial deposits only in secondary FSGS. Patients with secondary FSGS and the deposition of IgM showed inferior renal outcomes at earlier time points in comparison with patients with negative IgM expression (P=0.022). CONCLUSIONS: We detected a positive correlation between IgM and C3 in secondary FSGS. The association between IgM deposition and worse renal outcome in secondary FSGS indicates that IgM may play a role in the progression of this disease.

Clinical, serological and histological determinants of patient and renal outcome in ANCA-associated vasculitis with renal involvement: an analysis from a referral centre.

PURPOSE: To evaluate significance of clinical and histopathological prognostic factors for renal and patient outcome in AAV patient cohort. METHODS: Retrospective study included consecutive patients diagnosed with pauci-immune crescentic glomerulonephritis from January 2003 to December 2013. Primary outcome was combined endpoint patient death or progression to end-stage renal disease (ESRD). Secondary outcomes were patient survival and progression to ESRD (renal survival) singularly and disease relapse. Kaplan-Meyer survival analysis and multivariate Cox proportional hazard regression analysis were used to explore difference between phenotypes and finding significant predictors regarding outcomes. RESULTS: Out of 81 patients, 40.7% patients reached primary endpoint, 22.2% died, 29.6% reached ESRD and 16% relapsed during follow-up. Multivariate Cox proportional hazards regression-adjusted analysis found higher BVAS (HR 1.08, 95% CI 1.01-1.17, p = 0.042), higher baseline maximal serum creatinine (HR 1.02, 95% CI 1.01-1.03, p = 0.04) and lower haemoglobin (HR 0.97, 95% CI 0.95-0.99, p = 0.011) significantly associated with primary endpoint. Higher BVAS (HR 1.25, 95% CI 1.01-1.43, p = 0.001) and lower haemoglobin (HR 0.95, 95% CI 0.91-0.99, p = 0.008) were significantly associated with patient survival, while for renal survival, lower haemoglobin (HR 0.97, 95% CI 0.94-0.99, p = 0.041) and the need for acute haemodialysis (HR 3.15, 95% CI 1.20-8.26, p = 0.02) were significant predictors. On multivariate-adjusted analysis, no significant predictors for disease relapse were found. Kaplan-Meier survival analysis found no difference between clinical, serological and pathohistological phenotypes for all of the endpoints. CONCLUSIONS: Renal function at presentation, anaemia and BVAS should be included in prediction models for the outcomes for the AAV patients.

Non-diabetic renal disease in Croatian patients with type 2 diabetes mellitus.

AIM: Our study aimed to examine the prevalence of non-diabetic renal disease in selected patients with type 2 diabetes mellitus and to determine important risk factors for non-diabetic renal disease. METHODS: We conducted retrospective analysis of clinical, laboratory and pathohistological data of type 2 diabetes mellitus patients in whom renal biopsies were performed from January 2004 to February 2013 at Dubrava University Hospital Zagreb Croatia (n=80). RESULTS: According to renal biopsy findings, isolated diabetic nephropathy was found in 46.25%, non-diabetic renal disease superimposed on diabetic nephropathy in 17.5% and isolated non-diabetic renal disease in 36.25% of the patients. The most common non-diabetic renal diseases found were: membranous nephropathy, followed by IgA nephropathy and focal segmental glomerulosclerosis. In univariate analysis shorter duration of diabetes, independence of insulin therapy, lower levels of HbA1c and absence of diabetic retinopathy were found to be significant clinical predictors of non-diabetic renal disease. In multivariate analysis only independence of insulin therapy (OR 4.418, 95%CI=1.477-13.216) and absence of diabetic retinopathy (OR 5.579, 95%CI=1.788-17.404) were independent predictors of non-diabetic renal disease. CONCLUSIONS: This study confirmed usefulness of renal biopsy in patients with type 2 diabetes mellitus, due to the high prevalence of non-diabetic renal disease found. Since non-diabetic renal disease are potentially curable, we should consider renal biopsy in selected type 2 diabetes mellitus patients with renal involvement, especially in those with absence of diabetic retinopathy and independence of insulin therapy.

Epidemiologic data of adult native biopsy-proven renal diseases in Croatia.

PURPOSE: There is a paucity of epidemiological data on biopsy-proven renal disease in Croatia. The purpose of this report is a review of clinical and histological data, over a period of 15 years, from the single biggest adult native renal biopsy center in Croatia. METHODS: This report includes data from 922 adult native renal biopsies in patients referred from the whole country and performed in our center from 1996 till February 2012. Data on age, gender, serum creatinine, urine sediment, 24-h proteinuria, clinical syndrome and histological diagnosis were collected and analyzed retrospectively. In all patients, light, immunofluorescence and electron microscopic analysis was performed. RESULTS: The median age of the patients was 48 years (interquartile range 36-59 years), and the majority of patients were men (57.8 %). The most common indication for renal biopsy was nephrotic syndrome (40.3 %) followed by asymptomatic urinary abnormalities (31.7 %). The most common biopsy-proven renal disease in total was IgA glomerulonephritis (19.3 %), followed by focal segmental glomerulosclerosis (FSGS) (15.8 %) and membranous glomerulonephritis (9.2 %). In men, similar results were found, while in women, the most common were hereditary nephritis (13.4 %), FSGS (12.9 %) and connective tissue disease-related glomerular disorders (11.6 %). CONCLUSION: The presented data are an important contribution to the better understanding of the epidemiology of biopsy-proven renal disease in Croatia and Europe throughout comparison with other registry data. This data should be the basis for the formation of Croatian Registry of Renal Biopsies.

Prognostic significance of glomerular and tubulointerstitial morphometry in idiopathic membranous nephropathy.

The purpose of our study was to investigate the prognostic value of clinical and pathological, in particular glomerular and tubulointerstitial morphometric variables in idiopathic membranous nephropathy (IMN). We prospectively followed 60 Caucasian patients diagnosed with idiopathic membranous nephropathy for at least 2 years or until primary outcome (≥50% permanent decrease in estimated glomerular filtration rate or death). Glomerular and tubulointerstitial morphometric variables at the time of renal biopsy were analyzed with respect to this outcome. Univariate analysis revealed that significant negative prognostic factors for this outcome were higher cholesterol and smaller albumin concentrations, higher creatinine and maximal 24-h proteinuria, higher grade of nephroangiosclerosis, higher glomerular basement membrane thickness and glomerulopathy index, higher interstitial fibrosis and tubular atrophy percentage and higher injury score. In multivariate analysis, only the maximal 24-h proteinuria and interstitial fibrosis and tubular atrophy percentage were independent predictors of this outcome. The results suggest that morphometric analysis, mainly quantitative measurement of interstitial fibrosis and tubular atrophy percentage, injury score, glomerular basement membrane thickness and glomerulopathy index could be used as an additional method for risk stratification of patients with idiopathic membranous nephropathy.

[Fibrillary glomerulonephritis and immunotactoid glomerulopathy: case reports]. / Fibrilarni glomerulonefritis i imunotaktoidna glomerulopatija: prikaz bolesnika.

Fibrillary glomerulonephritis and immunotactoid glomerulopathy belong to the rare renal disorders characterized by formation of the organized glomerular deposits. Pathogenesis of these disorders is still not fully clarified but they could appear as a primary condition or be regarded as a part of the various systemic mainly lymphoproliferative disorders. Clinical presentation includes proteinuria, hematuria, arterial hypertension and progressive renal insufficiency during several years. In this work we presented a male patient with fibrillary glomerulonephritis and a female patient with immunotactoid glomerulopathy as a part of a non-Hodgkin lymphoma. The aim of this presentation is to show the features of the fibrillary glomerulonephritis and immunotactoid glomerulopathy as well as emphasize the significance of the electron microscopy in the identification of these uncommon entities.

[Drug induced allergic interstitial nephritis]. / Alergijski intersticijski nefritis uzrokovan lijekovima.

The allergic interstitial nephritis (AIN) is a rare renal disorder which is commonly clinically presented with an acute renal failure. AIN is the most frequent result of the hypersensitivity related to drugs (most often antibiotics). In the patients with clinical suspicion to a drug related AIN, kidney biopsy is the most important diagnostic procedure. Except of causative drug discontinuation, AIN therapy is based on high dose glucocorticoids 1 mg/kg/day with dose tapering during consecutive 3 months. In the present work, we have shown 10 patients with drug induced AIN. We identified 4 causative drug groups among which most frequent were antibiotics. In clinical presentation of our patients acute renal failure was dominant and median of baseline serum creatinine was 497.5 micromol/L. In all patients the kidney biopsy was performed and nine patients (90%) have been treated with recommended glucocorticoid regimen, additionally, in 3 patients hemodialysis was introduced. In all patients, reduction in serum creatinine value was achieved with serum creatinine median of 152 micromol/L after a three-month glucocorticoid treatment (p=0.018).

[Renal involvement in patients with rheumatoid arthritis]. / Bubrezne promjene u bolesnika s reumatoidnim artritisom.

In rheumatoid arthritis (RA) kidney is commonly affected organ with clinical presentation characterised by proteinuria (often nephrotic range) and microhematuria followed by chronic renal failure. This condition is well recognized as a rheumatoid nephropathy (rheumatoid glomerulonephritis), which is mediated by an immunological inflammation and by nephrotoxic effects of numerous drugs usually used in rheumatoid arthiritis treatment, such as NSAID, DMARD. In the patohistological examination various kinds of associated renal lesions could be seen. The most often are amyloidosis, glomerulonephritis, interstitial nephritis. In this study, we presented 15 patients, 10 women and 5 men, mean age of 60.2 with average rheumatoid arthritis duration of 19.4 years and signs of rheumatoid nephropathy. In all patients renal biopsy was performed with frequency of histopathological findings as follows: amyloidosis in 5 patients, IgA nephropathy in 3 patients, FSGS in 3 patients, mesangial proliferative glomerulonephritis in 3 patients, minimal change disease, pauci-immune glomerulonephritis and thin membrane disease in 1 patient. In all patients (except patient with thin membrane nephropathy) we started immunossuppresive therapy with glucocorticoids in combination with cyclophosphamide or cyclosporin or azatioprine. In conclusion, in all patients with rheumatoid arthritis, parameters of renal function should be monitored and in the case of patologic results, renal biopsy should be be performed. In the treatment of RA patients with related renal disorder, suspected causal drug should be removed from the treatment and specific immunosuppressive therapy initiated.