Introduction: There is paucity of data of C3 glomerulopathy in Indian children. Methods: First Indian pediatric case series where consecutive renal biopsies done over a period of ten years were reviewed to identify those patients who had isolated or predominant C3 deposits on immunofluorescent microscopy, fulfilling the criteria for C-3 glomerulopathy. The clinical, biochemical, serological, histopathological profile, eGFR and the need for renal replacement therapy was analyzed. Results: Eighteen patients, comprising 5.3% (18/298) of all renal biopsies, had C3 glomerulopathy, four with Dense Deposit Disease (DDD) and fourteen with C3 Glomerulonephritis (C3GN) with a median follow-up of 38.2 months. Median age of presentation was 7.45±3.03 years (2.5yrs- 13.5yrs) with nine boys and nine girls. Presentation was nephrotic syndrome in seven (39%), acute nephritic syndrome in three (16.7%), hematuria in five (27.7%) and acute kidney injury in three (16.7%). Median eGFR was 69 ml/min/1.73m² (8.2-107 ml/min/1.73m²). Hematuria was seen in 16 (88%), proteinuria in 18 (100%) and low C3 in 16 (88%) at the time of presentation. Mesangioproliferative glomerulonephritis was the predominant pattern in DDD while C3GN showed a mix of mesangioproliferative, membranoproliferative, endocapillary and crescentic GN (p = 0.43).Complete or partial remission was seen in seven patients who received long term alternate day steroids alone or with added mycophenolate mofetil. The cumulative patient survival was 70.8%. Kaplan Meir analyses for renal survival without progression to ESRD was 60.2% at one year and 48.1% at five and ten years. Conclusion: Interstitial fibrosis and tubular atrophy on renal biopsy was an independent predictor of adverse renal outcome in the cohort (p = 0.013, HR8.1;95% CI -1.6-42).

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Background: Repeat renal biopsy is usually done for lupus nephritis (LN) flare or resistant disease. We analyzed the changes between first and repeat biopsy and the contribution of repeat biopsy on renal outcome in LN patients. Methods: This was a retrospective study carried out at a tertiary care center in India. Sixty-two LN patients who underwent repeat biopsy for clinical indications, between January 2012 to December 2016, were included. Clinical and histological parameters at first and second biopsies were compared. Logistic regression analysis was done to determine parameters on repeat biopsy predicting response at last visit. Results: Repeat biopsy was done for relapse in 56% and for resistant disease in 44% patients. Seven (13.7%) out of 51 patients with baseline proliferative histology converted to non-proliferative lesion on second biopsy, while 2 (18.2%) out of 11 with baseline non-proliferative lesion converted to proliferative lesion on second biopsy. On repeat biopsy, the presence of endocapillary proliferation decreased, whereas glomerulosclerosis, interstitial fibrosis/tubular atrophy (IFTA), and glomerular basement membrane thickening increased. At the last visit (median follow-up of 38.6 months after first biopsy and 13.8 months after second biopsy), 79% of patients were in remission and 6.5% needed renal replacement therapy. The presence of IFTA >30% and thrombotic microangiopathy (TMA) on second biopsy independently predicted response at last visit. Conclusion: In Indian patients with LN, chronicity markers and superimposed membranous pattern increased on repeat biopsy done for clinical indications. The presence of IFTA and TMA on second biopsy predicted response at last visit.

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Renal transplantation is the preferred form of renal replacement therapy in patients who develop end-stage kidney disease (ESKD). Among the diverse etiologies of ESKD, glomerulonephritis is the third most common cause, behind hypertensive and diabetic kidney disease. Although efforts to prolong graft survival have improved over time with the advent of novel immunosuppression, recurrent glomerulonephritis remains a major threat to renal allograft survival despite concomitant immunosuppression. As a result, clinical expertise, early diagnosis and intervention will help identify recurrent disease and facilitate prompt treatment, thus minimizing graft loss, resulting in improved outcomes. In this review, we highlight the clinicopathologcal characteristics of certain glomerular diseases that recur in the renal allograft.

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Background and Aims: Renal failure occurring in the setting of cirrhosis increases mortality by more than threefold. Serum creatinine, the conventional marker for renal dysfunction has inherent limitations in identifying and categorizing renal dysfunction in patients with chronic liver disease (CLD). Neutrophil gelatinase associated lipocalin (NGAL) is a novel biomarker which gets upregulated as early as 2-6 hours following the insult to renal tubules. In this study, we aim to check the utility of uNGAL to identify the different phenotypes of renal dysfunction in patients with CLD. We also intend to assess the utility of NGAL to predict 90-day transplant-free survival in patients with CLD. Methods: A total number of 120 adult patients, with cirrhosis of liver were recruited. Those with pre-existing renal parenchymal disease, receiving nephrotoxic medications, spontaneous bacterial peritonitis, septic shock, proteinuria, hematuria, urinary tract infection and anuria were excluded. Urine samples for NGAL was measured at admission and at 48 hours thereafter. Patients were followed up for 90 days post admission. Results: Among the study population, 16 patients (13.3%) had normal kidney function, 43 (35.8%) had prerenal azotemia and 54 (45%) had Hepatorenal Syndrome (HRS -AKI) and 7 (5.8%) had acute tubular necrosis (ATN). Urinary NGAL (uNGAL) levels were considerably lower in patients with normal kidney function and prerenal azotemia. An uNGAL level of 124 ng/ml on admission could distinguish severe forms of renal injury, with a sensitivity of 86% and specificity of 84%. The non survivors had higher uNGAL levels at admission [209.6 ng/ml (118.7-376.8) vs. 123 (33.6-344.3); P = 0.013].The receiver operated curves for uNGAL and serum creatinine at admission did not show any significant difference for predicting 90 day mortality (AUC for uNGAL: 0.632 vs 0.580 for serum creatinine; difference in AUC 0.053, P value 0.17). Conclusion: uNGAL levels are elevated in patients with HRS-AKI and ATN. A higher uNGAL level at admission was suggestive of severe renal dysfunction. An elevated uNGAL on admission is associated with inferior survival. However, uNGAL is not superior to serum creatinine in predicting 90-day mortality.

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Melioidosis is a tropical infection that is increasingly being reported from South India. It is frequently observed in patients with diabetes mellitus, chronic ethanol consumption and chronic kidney disease (CKD). It presents commonly with pneumonia, deep seated abscesses or osteoarticular infections. Cardiac complications are very rare with endocarditis being reported in very few patients. We report the first case of endocarditis in melioidosis in India. Although infections are common in patients with CKD, melioidosis at the time of diagnosis of CKD has never been reported in the past. Our patient had multiple liver abscesses and endocarditis, and responded well to a 6 week course of ceftazidime and doxycycline, with the latter being continued for 20 weeks.

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Introduction: Despite high rates of morbidity and mortality in patients with contrast-induced nephropathy (CIN), there is no consensus regarding prevention of this well-known complication of contrast media use. One agent that has been widely used in this regard is N-acetyl cysteine (NAC). Nevertheless, its efficacy is still controversial. The aim of this study was to assess the efficacy of NAC, both in the oral and intravenous forms, for the prevention of CIN. Methods: This study is a double-blind randomized placebo controlled clinical trial. We randomized 434 adult patients with chronic kidney disease (constant serum creatinine ≥1.5 mg/dL) who were candidates for coronary angiography/plasty. The patients were categorized into three groups. One group received 1,200 mg NAC intravenously half an hour before the procedure and oral placebo starting 3 days before angiography. The second group received oral NAC 600 mg twice daily for 3 days, starting the day before the intervention and intravenous placebo half an hour before intervention. The third group received both oral and intravenous placebo. CIN was defined as a 25% relative increase in serum creatinine from baseline value, 48 h after use of contrast medium. Results: Of the 434 patients, 149 received intravenous NAC, 145 received oral NAC, and the remaining 140 received placebo. The incidence of CIN in the three groups was 6.1%, 7.6%, and 10.8%, respectively (p = 0.34). Conclusion: In patients with chronic kidney disease, neither intravenous nor oral NAC is superior to placebo for preventing CIN.

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Introduction: Permanent vascular access is an essential intervention in patients with advanced chronic kidney disease (CKD) and its success depends on various non-modifiable and modifiable factors. Considering the element of unpredictability and failure, we attempted to analyze various factors responsible for primary arteriovenous fistula (AVF) failure in presumed high-risk groups. Materials and Methods: We conducted an observational study of newly created AVFs at a tertiary referral government hospital in Eastern India between January 2014 and June 2015. All adult CKD patients undergoing AVF creation were included. Primary AVF failure was assessed at 12 weeks and total follow-up was 24 weeks in presumed high-risk groups of females, patients aged ≥65 years and those with diabetes mellitus. Results: Female gender was at a higher risk of primary AVF failure if aged ≥65 years (P = 0.0026), second AVF creation (P = 0.03), loupe magnification not used (P = 0.03), arterial plaque (P = 0.028), absent immediate thrill, and with radiocephalic AVF (P = 0.02). Absent immediate thrill (<0.0001) and AVF size ≤5 mm (P = 0.002) were important independent risk factors for primary failure. Diabetes or elderly age did not have additional risk, except with uncontrolled hypertension and female gender. Conclusion: Female gender was at a higher risk of poor unassisted AVF patency if their age was ≥65 years, had second AVF creation, loupe magnification not used or if arterial plaque was present. An absence of thrill immediately or at 24 hours or an AVF diameter ≤5 mm were independent intraoperative factors for poor outcome. On the contrary, diabetics, elderly males and intimal thickness were essentially noncontributors for AVF failure, except in few subsets.

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Nutcracker phenomenon (NCP) refers to compression of the left renal vein (LRV), most frequently between the aorta and the superior mesenteric artery, with impaired blood outflow often accompanied by distention of the distal portion of the vein. The nutcracker syndrome (NCS) is the terminology used when the nutcracker phenomenon is accompanied by a complex of symptoms such as pain (abdominal, flank, and pelvic), hematuria and orthostatic proteinuria. Diagnosis can be made with Doppler ultrasound (DUS), computed tomography (CT), magnetic resonance imaging (MRI), and venography. We describe a case of a young female adult who was identified with NCP by DUS, after a first CT had found no abnormalities. She presented with flank pain and severe hematuria causing a drastic decrease in hemoglobin levels. The management of NCS depends upon the clinical presentation and the severity of the LRV hypertension. The treatment options range from conservative to nephrectomy. Treatment decision should be based on the age of patients, severity of symptoms, and their expected reversibility. This case describes an underreported disorder that presents with non-specific symptoms, demonstrating the difficulties of the diagnostic approach of NCS, as well as the challenges in the appropriate management, given the lack of standardized treatment.

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Background: Hypertension is prevalent in 35%–46% of the general population; 1% of them experience accelerated hypertension. Among patients with accelerated hypertension, acute worsening of renal functions occur in 22%-55%. Morbidity and mortality rates are high. Partial renal recovery is seen in some, while others rapidly progress to end-stage renal disease. Methods: Patients who presented with accelerated hypertension, renal dysfunction, and had undergone renal biopsy were evaluated and their clinical profile was analyzed. Those who became dialysis dependent were excluded from further follow-up. Study outcome were blood pressure control, renal functions, requirement of renal replacement and mortality. Results: Of the 30 patients evaluated, age at presentation was 41.2 ± 15.46 years and 26 (86.7%) were males, 10 (33%) had presented with nonspecific complaints. Mean duration of hypertension and blood pressure were 21.93 months and 196 ± 20.8/129 ± 12.4 mmHg, respectively. Glomerulonephritis and hypertensive nephrosclerosis had similar characteristics except proteinuria (P = 0.04). Average follow-up (n = 25) duration was 3.69 years (range: 0.05–9.6). At the end of study, 6 were dialysis dependent, while in others, mean e-GFR was 23.96 ml/min/1.73 m². Poor renal prognosis was predicted by glomerulonephritis (relative risk-4.6) and degree of interstitial fibrosis. Five-year patient and renal survival were 94.4% and 71.9%, respectively. Conclusion: Accelerated hypertension occurs among patients with both primary and secondary hypertension. It leaves permanent renal sequelae. Though some patients recover renal function partially, further progression is rapid, especially among those with chronic glomerulonephritis.

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In patients with end-stage renal disease (ESRD), functional vascular access is like a lifeline. Among the patients undergoing hemodialysis, arteriovenous fistulas are done in most of them. However, in approximately 15% of these patients with multiple access failures, the use of tunneled dialysis catheters (TDC) for hemodialysis has become an integral component of treatment plans. Unfortunately, in many cases of multiple access failure, it is difficult to get proper access especially if problems with peritoneal dialysis also exist. The problems related to tunneled dialysis catheters are infections, thrombosis, hemorrhage, migration, dislodgement, leakage, kinking, and chronic venous stenosis. The progressive loss of venous access sites requires a systematic approach to alternative sites as it is important for patient survival. Herein, we present a case of ESRD with multiple access failures who was effectively managed with the placement of a percutaneous transhepatic vein permcath. Till date, there is very little data onsuch interventions from India.

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Introduction: Visceral fat area (VFA) is known to increase after initiation of peritoneal dialysis (PD). However, the factors contributing to the increase in VFA in long-term PD patients have not been sufficiently elucidated. The present study investigated factors that affect VFA in patients who continue PD for ≥3 years. Methods: Twenty patients (63.1 ± 10.3 years, 9 men, 11 diabetic patients) between January 2008 and January 2015 were included. VFA, subcutaneous fat area (SFA) and waist circumference at initiation and follow-up were measured at the level of the umbilicus by computed tomography using an image analysis system. Change in VFA was defined as the value obtained by dividing VFA at the final follow-up by that at the initiation. The correlations between clinical parameters at initiation and changes in VFA were analyzed. Results: There was no significant change in body weight (57.6 ± 10.4 vs 58.3 ± 7.8 kg, P = 0.296) during the mean final follow-up period of 55 ± 13 months, although VFA increased significantly (103.6 ± 39.2 vs 122.6 ± 38.3 cm², P = 0.030). Although subcutaneous fat area (SFA) did not change (124.7 ± 52.3 vs 124.5 ± 49.2 cm², P = 0.989), waist circumference increased significantly (79.4 ± 8.4 vs 83.7 ± 6.9 cm, P = 0.010). SFA (r = −0.735, P < 0.001), waist circumference (r = − 0.644, P = 0.002), high-density lipoprotein cholesterol (HDL-C) (r = 0.487, P = 0.029), and age (r = 0.507, P = 0.023) correlated significantly with changes in VFA. Conclusions: VFA might increase with long-term PD in patients with end-stage kidney disease who have high HDL-C, small SFA, and small waist circumference at initiation.

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Significance of antiphospholipid antibodies in immune thrombocytopenic purpura is debatable and pose a diagnostic and therapeutic dilemma. Catastrophic antiphospholipid syndrome is a rare life-threatening entity, occurring in patients with antiphospholipid syndrome, usually after a triggering event. We describe an adult lady of chronic immune thrombocytopenic purpura (in remission) with antiphospholipid antibodies, who presented with rapidly progressive renal failure and had primary antiphospholipid syndrome nephropathy. The index manuscript titled exemplifies the fact that although the presence of APLA in ITP is known, however, management in the absence of clinical event remains debatable and may carry a future risk of thrombotic event/s mandating close monitoring with a high index of suspicion.

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A 56-year-old kidney transplant recipient presented with a progressive cauliflower-like growth on the plantar surface of the right foot and on workup found to have chromoblastomycosis, which was successfully treated by antifungal agent and surgical excision with no recurrence. Chromoblastomycosis belongs to the heterogeneous group of subcutaneous mycoses. It is caused by various pigmented (dematiaceous) fungi, which gain entry into the skin via traumatic implantation. The case is of interest because it has so far not been reported from the northwest arid zone of India in kidney transplant recipients.

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