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Focal and segmental glomerulosclerosis (FSGS) is a common histopathological lesion that can represent a primary podocytopathy, or occur as an adaptive phenomenon consequent to nephron mass reduction, a scar from a healing vasculitic lesion, direct drug toxicity or viral infection among other secondary causes. Thus, the presence of an FSGS lesion in a renal biopsy does not confer a disease diagnosis, but rather represents the beginning of an exploratory process, hopefully leading ultimately to identification of a specific etiology and its appropriate treatment. We define primary FSGS as a ‘primary’ podocytopathy characterized clinically by the presence of nephrotic syndrome in a patient with an FSGS lesion on light microscopy and widespread foot process effacement on electron microscopy (EM). Secondary FSGS is commonly characterized by the absence of nephrotic syndrome and the presence of segmental foot process effacement on EM. Failure to accurately differentiate between the primary and secondary forms of FSGS has resulted in many patients undergoing unnecessary immunosuppressive treatment. Here, we review some key points that may assist the practicing nephrologist to distinguish between primary and secondary FSGS. [ABSTRACT FROM AUTHOR]

Idiopathic focal segmental glomerulosclerosis (FSGS) is among the most common, morbid and treatment-resistant conditions faced by nephrologists. While glucocorticoids have traditionally been the mainstay of initial treatment, they induce remission in only a minority of patients. A variety of other immunosuppressants have been utilized against steroid-resistant FSGS, but few have been rigorously examined in well-controlled trials. Recently, the results were published from a National Institutes of Health (NIH)-sponsored multicenter randomized trial comparing cyclosporine (CSA) with a combination of mycophenolate mofetil (MMF) and pulse dexamethasone (DEX) for the treatment of steroid-resistant FSGS. No difference in treatment effectiveness was shown between the two groups, and adverse effects were comparable. This was the largest randomized trial ever undertaken in FSGS, but it was unfortunately underpowered to show clinically relevant differences in response rates. This shortcoming, along with particularities of the study population and outcome measures, makes it challenging to draw definitive conclusions from the trial results. Despite these limitations, the trial does provide valuable insights into treatment strategies for FSGS and offers important lessons for planning future research. [ABSTRACT FROM PUBLISHER]

Background. Nephrotic syndrome is caused by a variety of glomerulopathy. The current study investigated the renal histopathological spectrum of patients with nephrotic syndrome who received a renal biopsy in our department within the last 15 years.Methods. One thousand five hundred and twenty-three consecutive patients (≥14 years old at renal biopsy) with nephrotic syndrome were recruited. Patients were divided into four groups according to age at the time of renal biopsy. The renal histopathological spectrum was also compared between nephrotic-range proteinuria patients with and without hypoalbuminaemia.Results. Among the 1523 patients, the most common cause of nephrotic syndrome was idiopathic membranous nephropathy (IMN) (20.7%), followed by minimal change disease (MCD) (20.4%). Among the patients aged 14–24, 25–44, 45–59 and ≥60 years, the most common cause of nephrotic syndrome was MCD (33.0%), lupus nephritis (LN) (23.0%), IMN (37.9%) and IMN (42.3%), respectively. Among the female patients aged 14–24 and 25–44 years, LN was the leading cause of nephrotic syndrome (35.8 and 36.2%, respectively). The proportion of patients with renal amyloidosis increased in parallel with patient age. The comparison between nephrotic patients with and without hypoalbuminaemia suggests that patients with MCD, LN or renal amyloidosis were more likely to develop hypoalbuminaemia.Conclusions. The renal histopathological spectrum of nephrotic syndrome differs between ages. MCD, LN and IMN were the main cause of nephrotic syndrome among younger patients, and IMN was the main cause of nephrotic syndrome among older patients. The proportion of patients with renal amyloidosis increased in parallel with patient age. [ABSTRACT FROM PUBLISHER]

Background. Steroid-dependent, steroid-resistant or frequently relapsing nephrotic syndrome carries a poor prognosis, including progression to renal failure. There are a number of studies confirming the efficacy of FK506 in steroid-resistant or steroid-dependent nephrotic syndrome. Although the use of this medication is becoming more common, we know very little about the potential nephrotoxicity when used in nephrotic syndrome.Method. We retrospectively reviewed the characteristics and biopsy findings of 11 children with steroid-dependent or frequently relapsing nephrotic syndrome treated with FK506. Two sequential biopsies were evaluated for the change in interstitial fibrosis, measured by a quantitative stereological method, and the change in arteriolar hyaline thickening, tubular atrophy and interstitial fibrosis, graded according to Banff criteria.Results. There was an increase in interstitial fibrosis (P = 0.005), with a median absolute change in the per cent volume density between initial and follow-up biopsies of 1.8% [interquartile range (IQR) 3.9%]. Median percentage change in volume density of interstitial fibrosis, relative to volume density of interstitial fibrosis prior to initiating FK506, was 93% (IQR 138%). Banff scores for interstitial fibrosis and tubular atrophy also increased following tacrolimus therapy (P = 0.04 for both). Average FK506 trough level over the treatment period was significantly associated with change in fibrosis (Spearman's rho = 0.67 and P = 0.02).Conclusions. This is some of the first histological data concerning tacrolimus nephrotoxicity in childhood nephrotic syndrome. Although the role of the natural progression of the underlying disease in the observed change is not definitively clear, the changes seen are in keeping with the known nephrotoxic effects of FK506 demonstrated in renal transplant. This increase is small when presented as a median change. However, there were a number of children who had a larger change in fibrosis. The factors predictive of interstitial fibrosis while on FK506 are not well defined; the findings from this study suggest that FK506 level may be a factor. Given the observations and limitations of the few published studies, there is an obvious need for further study in a large multicenter prospective trial. [ABSTRACT FROM PUBLISHER]

Amyloidosis is a group of diseases categorized by precipitation of a group of protein aggregates (amyloid) in tissues, including the kidney, and proteinuria is usually the commonest, though not exclusive, hallmark of clinical presentation. AL and AA are the most commonly recognized forms of amyloidosis involving the kidney, but other forms have been described. We present a case of renal amyloidosis due to a novel amyloidogenic protein, leucocyte cellderived chemotaxin 2, without proteinuria at presentation or on subsequent follow-up. [ABSTRACT FROM AUTHOR]

Background. The pathogenesis of hepatitis B virus (HBV)-associated glomerulonephritis (HBVGN) is generally believed to be immune complex deposition. However, the presence of HBV-DNA and -RNA in HBVGN renal tissues suggested a direct virally induced injury. We previously showed that nuclear factor κB (NF-κB) was activated in HBVGN renal tissues, especially in tubular cells. We therefore investigated the role of NF-κB in tubular epithelial cells with HBV infection. [ABSTRACT FROM PUBLISHER]

Background. Little is known about the decline of kidney function in patients with normal kidney function at baseline. Our objectives were to (i) identify predictors of incident chronic kidney disease (CKD) and (ii) to estimate rate of decline in kidney function. [ABSTRACT FROM PUBLISHER]

Background. The study determined (i) whether or not quartan malaria nephropathy (QMN) is still a major cause of childhood nephrotic syndrome (CNS) in Nigeria, (ii) secondary causes other than QMN and their associated glomerular pathology and (iii) renal and patient outcome. [ABSTRACT FROM PUBLISHER]

The article presents information on a study related to sodium bicarbonate therapy to prevent contrast media-induced acute kidney injury (CI-AKI). The researchers had made a comprehensive meta-analysis including a total of 18 trials focusing on separate analysis of papers both published and unpublished. It is reported that sodium bicarbonate-based hydration was found to be superior to normal saline in the prevention of CI-AKI in all recent meta-analyses.

Background. Infections such as malaria, schistosomiasis, hepatitis B and HIV have been suggested as major causes of the nephrotic syndrome (NS) in African children. We retrospectively analysed the course of the NS in 32 children from Ghana and reviewed the literature on NS from 18 different African countries for the presence of ‘the tropical nephrotic syndrome’.Methods. Thirty-two children (22 boys, 10 girls, median age 12 years, range 1–18 years) with NS were treated from 2000–2003 at Battor Hospital, Ghana. Thirteen out of 32 children underwent a renal biopsy which was investigated by light, immune and electron microscopy. All 32 patients were initially treated with oral prednisone (PRED) therapy (29 with standard therapy for 8 weeks and three individually tailored), and steroid-resistant children received also intravenous methylprednisolone pulses (three children) or oral cyclophosphamide (two children).Results. All patients fulfilled the clinical and laboratory criteria of a NS. The initial median serum creatinine was 65 µmol/l (range 44–133 µmol/l). Renal biopsy was performed in 13/32 children and revealed focal and segmental glomerulosclerosis (FSGS) in 10 patients, minimal change disease (MCNs) in two and no conclusive result in one patient. Glomerular immune complex deposition was absent in all biopsies. After treatment with PRED, oedema disappeared in 24/32 patients; however, proteinuria normalized in 16/32 patients only. The NS relapsed in 9/16 steroid-sensitive patients after cessation of PRED therapy, and two children were frequent relapsers. The steroid-resistant NS did not respond to an intensified immunosuppression in 5/16 children receiving methylprednisolone or cyclophosphamide. Five out of 32 children died, all were steroid resistant.Conclusions. There was no evidence for a dominating role of steroid-resistant ‘tropical glomerulopathies’ in children with a NS in Ghana. Similar to South Africa, focal and segmental glomerulosclerosis (FSGS) and minimal change disease were the most frequent findings on histology. Contrary to Nigeria, membrano-proliferative glomerulonephritis was not found in these patients. We conclude from this data and from the literature that the histological pattern of NS may vary between different African countries. Concerning therapy of NS under tropical conditions, we emphasize that despite the limited therapeutic facilities half of these patients may benefit from corticosteroids; however, steroid resistance and FSGS resulted in a high mortality. [ABSTRACT FROM AUTHOR]

Human hepatitis B virus (HBV) is well known as a cause of membranous nephropathy (MN). While the association of HBV infection with MN is strong, data regarding its association with other glomerular diseases are conflicting. Here, we report a case of focal segmental glomerulosclerosis (FSGS) with HBV infection. In this case, we have found HBV-DNA in urinary podocytes by real-time PCR methods. After the administration of anti-viral therapy, FSGS improved, paralleling the decreased level of HBVDNA in podocytes. The refractory FSGS induced by HBV could be effectively treated with appropriate antiviral agents. [ABSTRACT FROM AUTHOR]

The article discusses the problems of nephrology practice in Sub-Saharan Africa. It says that lack of resources is a major problem on the renal care in many parts of Africa. Rationing of services is practiced in many parts of Africa, including South Africa, resulting in small numbers of patients selected for renal replacement therapy (RRT). Appropriate training and retention of healthcare workers is another major challenge.