Introduction: Hemodialysis is the most common mode of therapy worldwide for chronic kidney disease (CKD) and is used as a life-sustaining therapy for most of the patients. Studies proved that dialysis affects the Quality of life (QOL) of patients. Health-related quality of life has been increasingly recognized as an important medical outcome in patients with CKD. Kidney disease quality of life short form (KDQOL-SF™) is developed by RAND to assess the QOL in CKD patients. This study was conducted to translate and validate the questionnaire KDQOL-SF™ in Malayalam. Methods: A cross sectional study was conducted among the patients who were undergoing dialysis in Sree Gokulam Medical College and Research foundation, Trivandrum. The translation was done according the specifications given by RAND. A sample of 112 patients studied. Statistical analysis was done for evaluating item internal consistency, item discriminant validity, equality of item-scale correlations, scale level reliability, and validity. Scale level descriptive statistics were computed. Results: Item internal consistency was more than 0.4 for all scales except a few. Item-level discriminant validity was 100% for almost all scales. Scale level reliability and validity were examined; all scales met the required internal consistency criteria. The overall reliability of the tool was 0.81. Scale level reliability varies from 0.71 to 0.92, which support item homogeneity and internal consistency across scales. Overall mean health rating score was 53.43 ± 11.48.Conclusion: The Malayalam version of KDQOL-SF™ is reliable and valid which can be used for measuring the health-related quality of life of Malayalam speaking CKD patients.

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Background and Aims: Full-house immunofluorescence in a kidney biopsy is a common observation in lupus nephritis (LN) and was previously used synonymously with the diagnosis of LN. Though a minority of the patients will develop features suggestive of SLE during follow-up, a majority of the patients will continue without any clinical or serological evidence of systemic lupus erythematosus (SLE) over time. Our aim to conduct this study was to work up the clinicopathological spectrum of these “full-house” nephropathies (FHN) which were not due to lupus nephritis. Methods: A total of 6244 renal biopsies were evaluated at SGPGIMS Lucknow from January 2007 to December 2017 for full-house immunofluorescence. All those patients who had no clinical or serological evidence of SLE at the time of renal biopsy or at any time during follow up were included. Results: Among 498 patients with full house immunofluorescence, 81 patients had no clinical or serological evidence of SLE at the time of renal biopsy or at any time during follow up. The prevalence of non-lupus FHN in this study was 19.4%, and the major diagnoses were membranous nephropathy (25.9%), IgAN (22.2%), MPGN (14.8%), DPGN (12.3%), Crescentic GN (12.3%), Amyloidosis (8.6%), C1q nephropathy (3.7%). Conclusions: Full-house nephropathy (FHN), not otherwise suggestive of lupus nephritis, can also be found in a number of other conditions. Non-lupus full house nephropathy is an umbrella term for such cases which do not satisfy the standard criteria of SLE. This will prevent misclassifying these patients into SLE and further prevent them from unnecessary immunosuppression protocols.

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Introduction: Collapsing glomerulopathy (CG) is a distinct morphologic pattern of proliferative renal parenchymal injury. It differ from focal segmental glomerulosclerosis (FSGS) by clinicopathologic pattern and its adverse outcome. The clinical significance of CG in renal allograft biopsies is not yet clear due to scant data and less occurrence of CG in renal transplant recipients. We conducted this single-center retrospective study to evaluate the prevalence, clinicopathological features, and outcome of post renal transplant CG. Subjects and Methods: We studied 127 renal allograft biopsies performed over a period of 45 months (Jan 2015–Oct 2018). A diagnosis of CG was made if at least one glomerulus demonstrated global or segmental collapse of the glomerular capillary walls, associated marked hyperplasia, and hypertrophy of the overlying visceral epithelial cells. We analyzed clinical, biochemical, and pathological characteristics and its impact on renal allograft outcome. Statistical analysis was performed and continuous variables were expressed as means ± standard deviation (SD) or medians (interquartile range and noncontinuous data were expressed in percentage and numerical values. Results: The prevalence of CG was 5.3% (7/127) of allograft biopsies. Out of the seven patients, six patients had undergone live donor transplant and one patient had undergone deceased donor renal transplant. The native kidney disease was unknown in these patients except one (IgA nephropathy). The median duration of diagnosis for CG was 17 months after transplantation (range 5–132months). Presenting symptoms were pedal edema and hypertension in 71.4% (5) patients each. All patients had proteinuria of more than 1 gm and renal allograft dysfunction and median serum creatinine of 3.05 mg/dl (1.5–4.8 mg/dl). All patients received standard triple immunosuppression. Over a period of 2–20 months, 57.14% (4) patients developed a graft failure and 43% (3) of the other patients had functioning grafts with serum creatinine of 1.5–4.2 mg/dl. Conclusions: CG presents with moderate to severe proteinuria and may lead to rapid graft dysfunction and subsequent graft failure in most of the patients.

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Background and Aims: Non-diabetic renal diseases (NDRDs) form an important part of disease manifestations in patients with diabetes. Methods: This hospital-based prospective study was conducted to analyze incidence and spectrum of NDRDs in patients with diabetes with or without diabetic nephropathy (DN), effect of early specific interventions on outcome, and renal-retinal relationship in type 1 and type 2 diabetes mellitus with nephropathy. 44 Patients with T2DM with the clinical suspicion of NDRD were subjected to renal biopsy Renal biopsies were performed by using an automated biopsy gun. Tissue was processed for Light microscopy-LM and Immunofluorescence-IF. Electron Microscopy was done as and when required by reprocessing the tissue embedded in paraffin for LM. Biopsies were reported by one experienced renal pathologist. Results: Renal histopathology revealed that of 44 enrolled patients with clinically suspected NDRD, 61.4% had isolated NDRD, 13.6% had NDRD superimposed on DN, and 25% had isolated DN. The most common NDRDs were minimal change disease (19.2%) and DN + chronic pyelonephritis (33.3%) in patients with isolated NDRD, and NDRD superimposed on DN, respectively. In the DN group, no patient had proliferative diabetic retinopathy (PDR) or hypertensive retinopathy, 45.5% had nonproliferative diabetic retinopathy (NPDR) and 54.5% had no microangiopathy in retina. In the NDRD group, 9.1% each had PDR and hypertensive retinopathy, 36.4% had NPDR and 45.4% had no microangiopathy in retina. No patient in the DN group and 72.7% in the NDRD group received specific treatment. In hospital, dialysis support was provided to 27.3% and 21.2% of patients in the DN and NDRD groups, respectively. In the DN group, 72.7% of patients improved with conservative therapy, 18.2% were dependent on dialysis when discharged. One patient died during treatment. In the NDRD group, 78.8% showed recovery in the renal function and clinical improvement, 15.1% were dialysis dependent when discharged. Two patients died during treatment. Conclusion: Accurate diagnosis of underlying NDRD by kidney biopsy facilitates initiation of specific therapy, which may lead to clinical improvement in significant number of patients.

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Drug induced acute interstitial nephritis is an idiosyncratic reaction following a drug exposure. The commonest drugs implicated are nonsteroidal anti-inflammatory drugs (NSAIDs), antibiotics and proton pump inhibitors. Renal cortical necrosis is a rare cause of acute kidney injury caused by severe and sustained vasoconstriction of small renal vessels. There is a change in the epidemiology of acute kidney injury especially in developing countries where drug induced acute kidney injury is becoming increasingly common. Naproxen is known to cause renal failure by renal papillary necrosis, tubular damage and acute interstitial nephritis. We present a case of Naproxen induced acute interstitial nephritis with acute cortical necrosis. To the best of our knowledge this is the first documented case of Naproxen induced renal cortical necrosis.

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Introduction: The goal of arterio-venous fistula (AVF) creation is to achieve a well-functioning access that can be cannulated repetitively and can provide adequate flow for the dialysis. The objective of this study was to assess the role of far infrared (FIR) therapy in the unassisted maturation of newly created AVF in patients with chronic kidney disease (CKD). Materials and Methods: In this prospective open labeled randomised control trial, 107 patients were randomized. Participants in the control arm received oral clopidogrel 75 mg once daily for 30 days along with isometric hand exercise, whereas those in the test arm received FIR therapy twice weekly, 40 min session each, for 4 weeks. A biopsy from venous end was taken during fistula surgery. Doppler study of AVF was done at the end of the 4^th and 12^th week to assess AVF. Vascular access guidelines proposed by National Kidney Foundation –Kidney Disease Outcomes Quality Initiative (NKF- KDOQI) in 2006 were adapted to define the maturation of AVF. Results: Out of 107 patients, 51 were randomized to the test arm and 56 to the control arm. During follow-up, the blood flow rate through AVF (Qa) and the diameter of the cephalic vein draining (CVd) the AVF were measured. At the end of 3 months, Qa in Radio-Cephalic Fistula (RCF) was high in the test arm (p–0.003). The AVF failures were 5 (10.2%) and 14 (28%) in the test and control arms, respectively (p: 0.025). However, when adjusted for AVF failure within 6 h of surgery (may be related to surgical technique) this difference in AVF patency was statistically insignificant (p: 0.121). The mean Qa was high in patients with an arterial intimal medial thickness (AIMT) <0.5 mm. The IMT of the anastomosed artery had statistically significant correlation with the primary failure rate of AVF (P < 0.001). Conclusion: In patients with CKD, FIR therapy was effective in increasing the AVF blood flow rate at the end of 3 months, though the difference in primary failure rate was statistically insignificant.

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Central venous stenosis (CVS) refers to a significant stenosis of a large intrathoracic vein, such as the subclavian, brachiocephalic, or the superior vena cava (hemodialysis, HD). Percutaneous transluminal angioplasty (PTA) with or without stent placement has been the recommended as the preferred approach to CVS. A total of 10 consecutive HD patients with documented CVS over a 2-year time period from April 2017-April 2019 underwent percutaneous angioplasty and stent insertions under sedation. The procedure was performed by the interventional cardiologist in the institute. One patient underwent only PTA, whereas nine (90%) had PTA with primary stent insertion. Primary patency was 90% at 3 months, 80% at 6 months while at 12 months, it was 70% and remained at 70% at 24 months. We did not find any association between age, gender, diabetic status, dialysis vintage, or previous catheter infection with procedural patency. Central venous stenosis can be treated successfully with percutaneous angioplasty and primary stenting. Despite advances, prevention of CVS should be the primary approach.

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Atypical hemolytic uremic syndrome is a rare form of thrombotic microangiopathy caused by complement pathogenic variants. We describe a case of a 33-year-old woman who presented as rapidly progressing renal failure requiring dialysis and had anemia, microhematuria, low C3, normal C4 levels, and normal platelet count. Renal biopsy revealed arteriolar thrombotic microangiopathy and acute tubular injury. Patient was treated with plasma exchange and hemodialysis as required. This resulted in partial recovery at 1 month. Genetic workup by multiplex ligation-dependent probe amplification revealed a 1.5 times higher signal intensity on downstream region of CFH gene and 50% reduced intensity of exon 6 of CFHR1 gene, suggesting a gene conversion event, similar to those previously reported from Spain and Portugal.

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We present this rare case of hyperhomocysteinemia due to a mutation in methylene-tetrahydrofolate-reductase (MTFHR) combined with plasminogen activator inhibitor deficiency, causing bilateral renal artery thrombosis. This case highlights the importance of genetic screening in individuals with a family history of thrombotic diseases. There seems to be a role of intervention, even in the setting of renal infarction.

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Invasive fungal infections are a significant cause of morbidity and mortality in patients systemic lupus erythematosus. The case illustrates the autopsy findings in a patient with systemic lupus erythematosus complicated by multiple fungal infections. Rare, uncommon manifestations of SLE such as mesenteric panniculitis and rhabdomyolysis were also present. High index of suspicion with timely intervention with aggressive antifungal was life-saving.

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