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Introduction: Monogenic urinary stone disease (MUSD) tends to be more severe with early onset of symptoms and a higher risk of chronic kidney disease than sporadic USD. The literature on the outcome after renal transplant in patients with certain MUSD is scarce. Materials and Methods: This is a retrospective single-center observational study conducted in a tertiary care renal transplant unit in North India between 2018 and 2021. The renal transplant recipients who developed an end-stage renal disease (ESRD) due to renal calculus disease/nephrocalcinosis were included in the study. All the patients presented to us in an anuric state, and hence, a 24-h urine metabolic profile could not be performed. Ear, nose, and throat and ophthalmological evaluations were done to rule out extrarenal manifestations. These patients were subjected to genetic analysis, i.e., clinical exome sequencing using next-generation sequencing. Results: Out of 283 live renal transplants, 11 patients developed ESRD due to nephrocalcinosis/renal calculus disease. Out of 11, only 4 had genetic mutations and the rest did not have any identifiable genetic mutations. The gene mutations were identified in ADCY10, CLDN16, CaSR, and SLC3A4. The patient with ADCY10 mutation had a strong family history. The clinical phenotype and in silico parameters analysis predicted the variant to be damaging except the one with CaSR mutation which causes Hypocalciuric hypercalcemia syndrome, type 1. Three of four underwent surgical intervention at younger age. All underwent successful live-related renal transplantation, with good graft function on follow-up, without any recurrence of calculus in the allograft. Conclusion: Renal transplantation can be safely proceeded in patients with the above monogenic mutations. Genetic analysis should be a part of pretransplant evaluation in young onset nephrolithiasis and end-stage kidney disease patients to look for a monogenic cause, to assess the risk of recurrence postrenal transplant. [ABSTRACT FROM AUTHOR]

Background: Posterior urethral valve (PUV) is obstructive uropathy that may lead to chronic kidney disease (CKD) and end-stage renal disease (ESRD) in children. Glomerular filtration rate (GFR) measurement remains the gold standard for renal function measurement. However, due to its less availability and cumbersome, it is not commonly used, and GFR is estimated utilizing various endogenous filtration markers. Objective: This study includes pediatric patients with PUV. We aimed to compare the measured GFR (mGFR) with various creatinine-based estimated GFR methods (eGFR). Materials and Methods: A single-center retrospective study included 62 treated cases of PUV, postvalve fulguration. The mGFR measured by 99mTc-diethylenetriaminepentaacetate in vitro method and compared with eight eGFR (Schwartz, Cockcroft-Gault [CG], Counahan-Barratt [CB], CKD Epidemiology Collaboration [CKD-EPI], full-age spectrum [FAS] age, FAS height (FAS Ht), Schwartz-Lyon [SL], and Ht independent). Patients were subdivided into different CKD grades and compared with various eGFR. Discussion: PUV is a common cause of CKD in children and needs special consideration as there is growth retardation associated with it. It decreases creatinine production and thus fallacies in eGFR measurement. There is a requisite to identify and closely monitor the subset of patients with baseline decreased renal function and therefore at risk of developing ESRD. Results: A total of 62 patients were included. Mean age and serum creatinine levels were 8.02 ± 5.53 years and 1.15 ± 0.95 mg/dl (range: 0.4-4.5), respectively. The mean mGFR was 61.6 ± 31.80 mL/min/1.73 m² and a positive variable correlation was 0.46-0.77 between mGFR and eGFR. Based on mGFR, there were 14 (22.6%), 21 (33.8%), 13 (20.9%), 9 (14.5%), and 5 (8.1%) patients in Grades I-V, respectively. The correct classification of the CKD grades was noted in 25 (40.3%), 16 (25.8%), 32 (51.6%), 16 (25.8%), 25 (40.3%), 27 (43.5%), 26 (41.9%), and 28 (45.2%) patients by Schwartz, CG, CB, CKD-EPI, FAS age, FAS Ht, SL, and Ht-independent equation. The eGFR overestimates GFR at the lower level and underestimates at higher levels. Conclusion: Our results confirm the considerable limitations of various creatinine-based clearance methods for estimating actual GFR. The creatinine clearance-based eGFR should not replace the measurement of the GFR. An initial measure of the mGFR followed by serial follow-up with the eGFR equation may be done. The most accurate eGFR equations are CB for Grade II, SL or Ht independent for Grade III, FAS age for Grade IV, and SL for Grade V CKD. [ABSTRACT FROM AUTHOR]

Introduction: The most common complication of percutaneous renal biopsy is bleeding, which can be seen in up to one-third of cases. The aim of this study was to evaluate the effect of prebiopsy administration of intranasal desmopressin acetate in reducing the incidence of biopsy-related bleeding complications in patients with significant renal dysfunction who underwent renal biopsy. Methods: This was a retrospective, observational study of percutaneous native renal biopsies performed at our center from July 2014 to June 2018. Bleeding complication rates of patients with renal failure (estimated glomerular filtration rate [eGFR] <30 mL/minute/1.73 m2 ) who received desmopressin and those who did not receive desmopressin were compared. Results: Desmopressin administration before renal biopsy in patients with eGFR <30 mL/minute/1.73 m2 was associated with a significant reduction of bleeding complications (major and minor together; P = 0.025) and no significant reduction in major complications (P = 0.616) or intervention rates (P = 0.251) when compared with a group that did not receive desmopressin. Conclusions: While prebiopsy intranasal desmopressin use was associated with a significant reduction of overall bleeding complications including major and minor complications, there was no reduction in the rate of other major complications and interventions. [ABSTRACT FROM AUTHOR]

Context: Role of endovascular management of vascular complications in renal transplant recipients. Aims: To present our experience in endovascular management of vascular complications in renal transplant recipients with a literature review. Settings andDesign: Aretrospective observational study. Materials andMethods: This is a retrospective analysis of postrenal transplant recipients referred to our department for the evaluation and endovascular management of vascular complications from January 2010 to December 2014. Cases of transplant renal artery stenosis (TRAS) and common iliac artery (CIA) stenosis were treated with percutaneous transluminal balloon angioplasty with or without stenting. Intraparenchymal pseudoaneurysms, arteriovenous fistula (AVF), and active contrast extravasation cases were managed with coil and/or glue embolization. Treated patients were followed up with serial blood pressure measurements, serum creatinine estimation, and Doppler evaluation in the postprocedure period and at 3‑month and 6‑month intervals. Results: Among a total of ten patients, there were five TRAS, two intraparenchymal pseudoaneurysms with active contrast extravasation, one AVF, one active renal bleed with peri‑graft hematoma, and one right CIA stenosis. Intraparenchymal pseudoaneurysms, AVF, and active extravasations were postbiopsy. All treated patients showed improved renal function, increased hematocrit, improved blood pressure control in the postprocedure period, and follow‑up. Conclusions: Vascular complications in renal transplant patients may lead to graft dysfunction, increased morbidity, and even graft loss if left untreated. Endovascular interventions are first‑line treatments with excellent short‑ and long‑term outcomes. [ABSTRACT FROM AUTHOR]

Background: While the majority of bleeding complications after a percutaneous kidney biopsy (PKB) occur early (≤24 h), delayed onset bleeding complications (>24 h) have been rarely reported and can be catastrophic for the patient. Purpose: To describe the incidence, risk factors, and outcomes of delayed bleeding complications after PKB. Material and Methods: We retrospectively studied native and graft kidney biopsies in patients who developed delayed bleeding complications (>24 h) after the biopsy performed in the Department of Nephrology and Renal Transplantation of a tertiary care medical institution in north India between January 2014 to December 2018. Results: Of the 4912 renal biopsies reviewed, 20 patients (16 men, 4 women; 0.40%) had a delayed biopsy bleeding complication. Of these patients, 95% had major bleeding complications requiring blood transfusions and 85% needed intervention like gelfoam/coil embolization. Despite intervention, one patient (5%) had mortality due to complications of bleeding and sepsis. When compared to a control group of patients with early biopsy bleed, patients with the delayed biopsy bleed had similar demographic and clinical profiles except for higher pre-biopsy hemoglobin and lower systolic and diastolic blood pressure. Conclusion: A post-PKB delayed onset bleed is not uncommon, and the vast majority of these patients had major bleeding complications requiring blood transfusions and/or intervention like embolization. They had a similar demographic and clinical profile presentation as early bleed patients. Meticulous outpatient monitoring and patient education after discharge may be useful to detect this complication promptly and to intervene early to have good patient outcome. [ABSTRACT FROM AUTHOR]

Introduction: The management of active antibody-mediated rejection (ABMR) is evolving, and optimal treatment remains uncertain due to incomplete understanding of pathogenesis. Bortezomib is found to be useful in the treatment of active ABMR. We studied the efficacy and safety of bortezomib in renal-transplant recipients with active ABMR. Materials and Methods: We retrospectively included renal-transplant recipients with active ABMR, who received bortezomib as main management. Results: Eighteen live-related renal-transplant recipients of active ABMR were included. C4d was positive in 14 patients and negative in eight patients. Patients with active ABMR had a mean improvement in glomerular filtration rate (GFR) of 7, 10.5, and 15 ml/min/1.73 m2 at 3, 6, and 12 months, respectively, from baseline GFR. Improvement was significant at 3 (P = 0.009) and 6 months (P = 0.018) of follow-up. Conclusion: Bortezomib may be a safe and effective therapy in patients with active ABMR in patients. [ABSTRACT FROM AUTHOR]

Background: Peritoneal dialysis (PD) is the preferred modality of dialysis among children with end-stage renal disease. Methods: To study the incidence of technique failure and survival among children with end-stage renal disease on continuous ambulatory peritoneal dialysis (CAPD), we included children younger than 18 years of age who commenced and continued PD for more than 3 months as their primary form of dialysis between 1st January 2005 and 31st December 2016. Kaplan–Meier survival analysis was applied to analyze the CAPD outcomes. Results: A total of 68 Tenckhoff (58 double cuffs, and ten single cuffs) catheters were inserted in 66 patients (mean age 12.3 ± 3.91 years) during the study period. Of the 66 children, 31 (47%) experienced 45 episodes of peritonitis. The total duration on CAPD was 107.58 years with a peritonitis rate of 0.42 episodes per year. Overall, the mean patient survival was 41 (95% confidence interval (CI) 29–54) months, with mean patient survival of 72% at 12 months, declining to 30% at 36 months and then remaining stable until the end of follow-up (106 months). The overall mean technique survival was 55 (95% CI 40–69) months, with mean technique survival of 69% at 12 months, declining to 44% at 36 months and then remaining stable until the end of follow-up (106 months). Conclusion: CAPD is a viable option for end-stage renal disease in children from developing countries with a lack of access to automated PD and pediatric hemodialysis centers. [ABSTRACT FROM AUTHOR]

Acute kidney injury (AKI) is an independent variable for poor outcome in critically ill patients. The pathophysiology of septic AKI is distinct from that of non-septic AKI. We studied the clinical profile and outcome of septic AKI since such data is sparse in Indian patients. In this single-center retrospective, observational, cohort study, septic AKI has been found with high incidence (31%) and overall mortality was 52%. Age, number of non-renal organ failure, and APACHE II score were found as significant predictors of outcome in this population. [ABSTRACT FROM AUTHOR]