Podocytopathies are the most common group of glomerular disorder leading to proteinuria. On the basis of pathophysiology, light microscopic and ultrastructural evaluation, the podocytopathies include minimal change disease, diffuse mesangial sclerosis, focal segmental glomerulosclerosis and collapsing glomerulopathy. The present review summarizes the basic etiopathogenesis of podocytopthies, highlights the common genetic and acquired factors in its causation, puts forth various diagnostic modalities and discusses the role of emerging agents or treatment.

C3 glomerulopathy has recently been described as a distinct entity. The underlying mechanism is unregulated activation of the alternate pathway of the complement system. The most common presentation is with an acute nephritic syndrome. The diagnosis is made on immunofluoroscence by the presence of isolated or dominant C3 staining. In this retrospective study, renal biopsy data were collected from 2010 to 2013 patients with C3 glomerulopathy identified and their clinical and biochemical parameters analyzed. Out of 514 biopsies available for analysis, the incidence of C3 glomerulopathy was 1.16% (n = 6). The mean age of the presentation was 26 years and the average estimated glomerular filtration rate was 30.65 ml/min/1.73 m ² . The most common histopathological pattern was membranoproliferative glomerulonephritis (n = 4).

Pulse methyl prednisolone followed by oral prednisolone and abrupt switch to chlorambucil/cyclophosphamide (Ponticelli/modified Ponticelli regimen) is used in patients with idiopathic membranous nephropathy. This therapy where steroids are stopped abruptly is unphysiologic and expected to have hypothalamic pituitary adrenal (HPA) axis suppression; however, this has not been evaluated. A total of 13 consecutive adult patients with idiopathic membranous nephropathy who had completed modified Ponticelli regimen were studied. The regimen included administration of pulse methylprednisolone 1 g for 3 days followed by oral prednisolone 0.5 mg/kg/day for 27 days followed by oral cyclophosphamide at a dose of 2 mg/kg/day for the next month. This was repeated for three courses. Patients who had received corticosteroids prior to therapy were excluded. The HPA axis was evaluated after 1 month of completing the last course of steroid therapy. The evaluation was done using a low-dose adrenocorticotropic hormone stimulation test. A single intravenous bolus dose of synacthen (1 μg) was given at 9.00 am and the serum cortisol levels were estimated by radioimmunoassay at 0, 30, and 60 min. A peak cortisol level of 550 nmol/L or higher was considered as normal. Mean baseline cortisol levels was 662.3 ± 294.6 nmol/L and peak cortisol level was 767 ± 304.4 nmol/L. A total of 6 patients (46.2%) had low basal cortisol levels, only 3 (23%) had both basal and peak cortisol levels < 550 nmol/L suggestive of HPA axis suppression. To conclude, 23% of patients had suppression of HPA axis after modified Ponticelli regimen.

Vascular access (VA) complications are the leading cause of morbidity in the hemodialysis (HD) population and responsible for high health care costs. This study was designed to compare the profile of VA use for HD in Isfahan dialysis units in 2003 and 2013. A cross-sectional observational study was conducted between January and March 2013 on 536 HD patients in seven units. The patients data about VA type, number, survival, and demographic characteristics were collected and compared with the data collected in year 2003 on 320 patients in the same city units. The mean age of patients increased from 51 ± 17 to 58 ± 15 years (P < 0.001).The most common etiology of end-stage renal disease was diabetes mellitus, but the percentage increased in 2013 (P = 0.001).The use of an arteriovenous fistula (AVF) as a first dialysis access fell from 60.6% in 2003 to 35.4% in 2013 (P < 0.001). At the time of study, AVFs were used in 92.2% of patients in 2003 versus 56.5% in 2013 (P < 0.001). The 1, 2, 3 and 5 years AVF survival was 80%, 78%, 73%, and 69%, respectively in 2003 versus 79.4%, 61.2%, 47.3%, and 31.5% in 2013. The AVFs proportions decreased and the tunneled catheters proportions increased while the proportion of temporary catheters decreased in 2013 compared with 2003.

There is increased cardiovascular (CV) mortality in subjects with chronic kidney disease (CKD). Arterial stiffness in these subjects is increased when compared to a healthy population. Markers of arterial stiffness and intima media thickness (IMT) are predictors of CV mortality. The aim of this study was to investigate whether there is any difference in markers of arterial stiffness and IMT between subjects with normal renal function and those with mild renal disease. The arterial distension waveform, IMT, diameter, and brachial blood pressure were measured to calculate Young's modulus (E) and elastic modulus (Ep) in the common carotid arteries of subjects with normal kidney function (estimated glomerular filtration rate [eGFR] >90) and those mild CKD (stage 2, eGFR 89-60). Data were available for 15 patients with normal kidney function and 29 patients with mild CKD. The subjects with mild CKD were older, but other co-variables were not significantly different. Both arterial wall stiffness parameters (E and Ep), but not IMT were significantly higher in the mild CKD group. Logistic regression demonstrated that only the arterial wall stiffness parameters (Ep and E) were independently associated with mild renal disease compared with normal, in a model adjusting for sex, age and diabetes and history of cardiovascular disease (CVD). E and Ep may be early markers of CVD in subjects with mild CKD that may manifest change before other more recognized markers such as IMT and pulse pressure.

Killer immunoglobulin-like receptor (KIR) gene shows a high degree of polymorphism. Natural killer cell receptor gets activated once they bind to self-human leukocyte antigens (HLAs) with specific ligand. KIR gene and HLA ligand incompatibility due to the presence/absence of KIR in the recipient and the corresponding HLA ligand in the allograft may impact graft survival in solid organ transplantation. This study evaluates the effect of matches between KIR genes and known HLA ligands. KIR genotypes were determined using sequence specific primer polymerase chain reaction. Presence of certain KIR in a recipient, where the donor lacked the corresponding HLA ligand was considered a mismatch. The allograft was considered matched when both KIR receptor and HLA alloantigen reveald compatibility among recipient and donor. The data revealed better survival among individuals with matched inhibitory KIR receptors and their corresponding HLA ligands (KIR2DL2/DL3-HLAC2, KIR3DL1-HLABw4). On the contrary, no adverse effect was seen for matched activating KIR receptors and their corresponding HLA ligands. One of the activating gene KIR2DS4 showed risk (P = 0.0413, odds ratio = 1.91, 95% confidence interval = 1.02-3.57) association with renal allograft rejection. We conclude that the presence of inhibitory KIR gene leads to better survival; whereas activating motifs show no significant role in renal allograft survival.

We evaluated whether polymorphisms in interleukin (IL-1) gene cluster (IL-1 alpha [IL-1A], IL-1 beta [IL-1B], and IL-1 receptor antagonist [IL-1RN]) are associated with end stage renal disease (ESRD). A total of 258 ESRD patients and 569 ethnicity matched controls were examined for IL-1 gene cluster. These were genotyped for five single-nucleotide gene polymorphisms in the IL-1A, IL-1B and IL-1RN genes and a variable number of tandem repeats (VNTR) in the IL-1RN. The IL-1B − 3953 and IL-1RN + 8006 polymorphism frequencies were significantly different between the two groups. At IL-1B, the T allele of − 3953C/T was increased among ESRD (P = 0.0001). A logistic regression model demonstrated that two repeat (240 base pair [bp]) of the IL-1Ra VNTR polymorphism was associated with ESRD (P = 0.0001). The C/C/C/C/C/1 haplotype was more prevalent in ESRD = 0.007). No linkage disequilibrium (LD) was observed between six loci of IL-1 gene. We further conducted a meta-analysis of existing studies and found that there is a strong association of IL-1 RN VNTR 86 bp repeat polymorphism with susceptibility to ESRD (odds ratio = 2.04, 95% confidence interval = 1.48-2.82; P = 0.000). IL-1B − 5887, +8006 and the IL-1RN VNTR polymorphisms have been implicated as potential risk factors for ESRD. The meta-analysis showed a strong association of IL-1RN 86 bp VNTR polymorphism with susceptibility to ESRD.

A 60-year male was admitted with advanced renal failure and bilaterally enlarged kidneys. Kidney biopsy revealed diffuse interstitial infiltration by CD20 + lymphomatous cells suggestive of diffuse large B-cell, non-Hodgkin's lymphoma. Bone marrow examination was negative for malignant cells. Positron emission tomography-computed tomography showed uniformly diffuse and avid flurodeoxy glucose uptake in both kidneys, multiple hypodense areas of both lobes of liver, and axial and appendicular skeleton. Patient was treated with rituximab, cyclophosphamide, hydroxydaunorubicin, vincristine and prednisolone, became afebrile and serum creatinine normalized.

We report a 50-year-old female who presented with inflammatory arthritis, upper respiratory tract symptoms, and microscopic hematuria with nephrotic range proteinuria. Antineutrophil cytoplasmic antibodies (ANCA) were detectable and kidney biopsy showed pauci-immune focal necrotizing crescentic glomerulonephritis. She was treated with pulse intravenous cyclophosphamide (CYC) and prednisolone. Patient developed severe leucopenia after the first dose and subsequently had leucopenia to low dose CYC, mycophenolate mofetil and azathioprine were also tried. However, patient developed leukopenia with all the above agents. Initiation of tacrolimus (TAC) was followed by dramatic response: Proteinuria decreased, serum albumin normalized and C-ANCA and anti-PR3 ANCA assays became negative. This is the first successful case of TAC as an induction agent in a patient with GPA (ANCA associated vasculitis with renal involvement).

A 40-year-old male presented with nephrotic syndrome. Light microscopic analysis of the renal biopsy showed thickening of the glomerular capillary wall. Immunofluorescence examination revealed granular deposition of monoclonal immunoglobulin (Ig) G3-kappa and complement C3 along the glomerular basement membrane. Electron microscopy showed subepithelial electron dense deposits, thus confirming membranous glomerulonephritis (MGN) with monoclonal gammopathy. MGN with monoclonal gammopathy is an extremely rare but distinctive entity. This patient was treated with a combination of bortezomib, thalidomide and dexamethasone and showed partial remission of his nephrotic state and dysproteinemia.

Recent reports indicate that statins can cause nephrotoxicity. However, the mechanisms of nephrotoxicity remain unclear. We report a case of acute kidney injury (AKI) in a 54-year-old man following the administration of atorvastatin Renal biopsy showed acute interstitial nephritis. Atorvastatin was withdrawn and he was treated with corticosteroid following which renal function recovered. When he was rechallenged with rosuvastatin 6 months later following an episode of acute myocardial infarction, he developed AKI again indicating class effect of statin for nephrotoxicity.

Due to their ability to decrease the spread of infection, hand sanitizers are now ubiquitous in health care settings. We present the case of a 50-year-old woman who was admitted with acute alcohol intoxication and had near complete recovery in 12 hrs. Subsequently, she was found unresponsive on the floor of her hospital room on two separate occasions. Evaluations revealed repeatedly elevated levels of ethanol, acetone, and lactate as well as increased anion gap and hypotension, requiring intensive care unit evaluation and intubation for airway protection. During the second episode, she was found next to an empty bottle of ethanol-based hospital hand sanitizer. She confirmed ingesting hand sanitizer in order to become intoxicated.