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Objective: The ketogenic diet has been the mainstay of treatment of drug-resistant epilepsy (DRE). No comparative trials have been conducted to assess the efficacy of the two less strict ketogenic diets: modified Atkins diet (MAD) and low glycemic index treatment (LGIT). This study assesses the non-inferiority of LGIT compared with MAD., Methods: This was an open-label randomized non-inferiority trial. Children with DRE were randomized to receive either MAD or LGIT as an add-on to anti-seizure medications. The primary endpoint was percentage seizure reduction at the end of 24 weeks of therapy compared to the baseline. The non-inferiority margin of -15% was predefined to calculate the sample size., Results: Ninety-one children were enrolled and randomized to receive either MAD (n = 45) or LGIT (n = 46). Intention-to-treat analysis done at the end of 24 weeks of therapy showed a mean (±standard deviation [SD]) percentage seizure reduction of 60.7% (±41.3) in the MAD sub-group and 57% (±39.4) in the LGIT sub-group (p = 0.664). The absolute difference between the means of percentage seizure reduction was -3.7 (-20.5 to 13.2) and crossed the non-inferiority margin. Ten children in the MAD group and nine children in the LGIT group did not complete 24 weeks of therapy. Adverse effects were comparable between the arms (MAD, 66.6%; LGIT, 50%), although serious adverse effects were higher in the MAD arm. The most common adverse effect was decreased acceptance (24.2%) followed by decreased satiety (9.9%), vomiting (9.9%), weight loss (5.5%), constipation (5.5%), and diarrhea (3.3%). Dyslipidemia was more commonly seen in the MAD group (MAD, six; LGIT, one). One death in the LGIT arm was unrelated to therapy. Although there was no statistically significant difference in improvement in cognition, behavior, and quality of life scales, improvement was noted from baseline scores., Significance: LGIT may be non-inferior to MAD in the treatment of children with DRE with the advantage of increased acceptance and fewer adverse effects., (© 2025 International League Against Epilepsy.)

Competing Interests: There are no conflicts of interest.

Background and Objectives: We compared experiences of patients who reported usually being seen by a resident with those usually seen by a staff physician., Methods: We analyzed responses to a patient experience survey distributed at 13 family medicine teaching practices affiliated with the University of Toronto between May and June 2020. We analyzed responses to seven questions pertaining to timely access, continuity, and patient-centeredness. We compared responses between two types of usual primary care clinicians and calculated odds ratios before and after adjustment for patient characteristics., Results: We analyzed data from 6,545 unique surveys; 18.6% reported their usual clinician was a resident physician. Resident patients were more likely to be older, born outside of Canada, report a high school education or less, and report having difficulty making ends meet. Compared to patients of staff physicians, patients of resident physicians had lower odds of being able to see their preferred primary care clinician and lower odds of getting nonurgent care in a reasonable time. They also had lower odds of reporting patient-centered care, but we found no significant differences in whether the time for an urgent appointment was about right or whether accessing care after hours was easy., Conclusions: In our setting, patients who reported usually seeing resident physicians had worse continuity of care and timeliness for nonurgent care than patients who reported usually seeing staff physicians despite resident patients being older, sicker, and having a lower socioeconomic position. Postgraduate training programs need to test models to support access and continuity for resident patient panels.

Objectives: Respiratory tract infections are life-threatening infections in solid-organ transplant recipients that pose risk to the graft and to the patient. This study was undertaken to examine the clinical and microbiological spectrum of pneumonia in renal transplant recipients., Materials and Methods: Of 400 consecutive renal transplant recipients, 87 recipients (21.8%) were hospitalized between November 2014 and October 2016 with pneumonia. We examined demographic profiles and clinical investigations., Results: The median age of patients was 38 years (range, 19-72 y). The mean time of presentation after renal transplant was 18 months (range, 1-174 mo). Most patients (80.5%) were on maintenance immunosuppression with tacrolimus, mycophenolate mofetil, and steroids; 34% of patients had an induction agent. Chronic hepatitis C and hepatitis B infections were found in 12.6% and 2.2% of patients, respectively, and new-onset diabetes in 19.5% of patients. Fever (88%), cough (87%), shortness of breath (68%), and hypotension (33%) were common presenting symptoms. Diarrhea was the most frequent accompanying symptom, found in 9.2% of patients. Cytopenia and graft dysfunction were present in 38.7% and 80.4% of patients. Among infections, fungal infections were the most frequent (30%) followed by mixed infections (20.7%), tuberculosis (12.6%), bacterial (12.6%), and viral (3.5%) infections. Etiology could not be found in 27.6% patients. Mortality rate was 24.1%, with the highest rates for fungal infections (44%), followed by bacterial (25%) and mixed infections (18%). Presence of hypoxia and hypotension at presentation was associated with increased risk of death, whereas use of induction agents, new-onset diabetes posttransplant, diabetes mellitus, and acute kidney injury were not correlated with death or increased duration of hospital stay., Conclusions: Pneumonia carries high risk of mortality in renal transplant recipients. Fungal and bacterial infections carry high risk of mortality. Despite invasive investigations, a substantial number of patients had unidentified etiology.

We investigated the effect of two dosing regimens of oral iron on iron status and hematological parameters in patients with CKD. In this single center, open label, randomized, active controlled clinical trial, stable adult patients with CKD stage G3-4 with percentage transferrin saturation (%TSAT) ≤ 30% and serum ferritin ≤ 500 ng/ml were eligible. Participants were randomized to receive either 100 mg of ferrous ascorbate once daily (OD group) or 100 mg of ferrous ascorbate twice daily (BD group, total daily dose 200 mg). The primary outcome was change in %TSAT between groups over 12 weeks. The secondary outcomes were changes in other iron status and hematological parameters, serum interleukin-6 (IL-6) and hepcidin. 80 participants were enrolled out of which 76 completed the study. Change in %TSAT was not significantly different between groups (β = - 1.43, 95% CI - 3.99 to 1.12, BD group as reference). The rise in serum ferritin was less in the OD group as compared to BD group (β = - 0.36, 95% CI - 0.61 to - 0.10) whereas MCHC increased in the OD group as compared to decrease in the BD group (β = 0.37, 95% CI 0.067-0.67). These observations need exploration to ascertain the impact of different oral iron dosing strategies in CKD., (© 2023. The Author(s).)

The preferential use of convective modes of hemodialysis (HD) for targeting hyper-cytokinemia state in sepsis-related acute kidney injury (AKI) has been questioned for its efficacy. Several studies have used predilution hemodiafiltration (HDF) in critically ill AKI patients with mixed results. In this study, we compared intermittent online postdilution HDF with the standard high-flux (HF) intermittent HD in non-critically ill patients with community-acquired (CA) AKI. In this pilot study, stable patients with CA AKI and systemic inflammatory response syndrome were included and given either postdilution online-HDF (OL-HDF) or standard HF HD outside intensive care units. The primary objectives were to assess the feasibility of conducting the study at a larger scale and to detect the differential impact of convective clearance on the rates of independence from dialysis at discharge or after 30 days. Plasma cytokine clearance was assessed as a secondary objective. Eighty consecutive AKI patients were randomized to receive dialysis in one of the treatment arms after fulfilling the eligibility criteria. The baseline parameters of clinical severity, etiology, and indications of dialysis, plus the baseline plasma cytokine profiles, were comparable. Moreover, 83% in the control arm and 71.1% in the intervention arm became independent from dialysis at discharge or at 30 days (P = 0.189). No survival advantage of postdilution OL-HDF was observed (P >0.05). Similar plasma cytokine clearance levels were noted in both arms. The current study confirms the feasibility; however, it does not support the preferential use of postdilution OL-HDF over HF-HD in non-critical patients., (Copyright © 2022 Copyright: © 2022 Saudi Journal of Kidney Diseases and Transplantation.)

Acute kidney injury (AKI) increases the risk of morbidity, mortality, and progression to chronic kidney disease (CKD). There are few data on the risk of CKD following community-acquired AKI (CA-AKI) and its predictors from developing countries. We evaluated the association of a panel of serum and urine biomarkers at the time of hospital discharge with 4-month renal outcome in CA-AKI. Patients of either sex, aged between 18 and 70 years, with no underlying CKD, and with CA-AKI were recruited at the time of discharge from hospital in this prospective observational study. Levels of serum and urine biomarkers were analyzed and association between these markers and development of CKD, defined as eGFR < 60 ml/min/1.73 m 2 or dialysis dependence at 4 month after discharge, were analyzed using multivariate logistic regression analysis and penalized least absolute shrinkage and selection operator logistic regression. Out of a total 126 patients followed up for 4 months, 25 developed CKD. Those who developed CKD were older (p = 0.008), had higher serum creatinine (p < 0.001) and lower serum albumin (p = 0.001) at discharge. Adjusted logistic regression showed that each 10% increase in standardized serum myo-inositol oxygenase (MIOX) level increased the odds of progression to CKD by 13.5%. With 10% increase in standardized urine Neutrophil gelatinase-associated lipocalin (NGAL), serum creatinine and urine protein creatinine ratio (uPCR), increase in the odds of progression to CKD was 10.5%, 9.6% and 8%, respectively. Multivariable logistic model including serum MIOX, discharge serum creatinine and discharge uPCR, was able to predict the progression of CKD [AUC ROC 0.88; (95% CI 0.81, 0.95)]. High level serum MIOX levels at the time of discharge from hospital are associated with progression to CKD in patients with CA-AKI., (© 2022. The Author(s).)

Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease, and lupus nephritis (LN) is associated with increased morbidity and mortality. Renal biopsy is essential and the gold standard to diagnose LN. Extra-glomerular involvement is seen in up to 60% of patients with LN and is associated with poor outcomes. The revised International Society of Nephrology/Renal Pathology Society classification for LN has changed the parameters for activity index scoring, redefined crescent and highlighted the significance of extra-glomerular involvement. Repeat renal biopsy is done for resistant disease or during a flare, usually when atypical features are present or when the baseline biopsy showed non-proliferative histology. Protocol repeat biopsy may prove to be valuable as a monitoring tool in patients with LN. Newer modalities of therapy like multitargeted therapy and biological agents may pave a way for better outcomes with minimal adverse effects to the patients., (© Journal of the Association of Physicians of India 2011.)

Background & Aims: Myocardial dysfunction is one of the common complications in children with chronic kidney disease which results in significant morbidity and mortality. We aimed to find the impact of anemia with cardiac changes in children with chronic kidney disease (CKD)., Methods: In this cross-sectional pilot study, 54 children (38 males) up to the age of 16 years with different stages of CKD, not on dialysis, were enrolled. Cardiovascular functions were evaluated using 2D-echocardiography using EPIC-7 (Philips) machine by an independent trained Pediatric Cardiologist. The M-mode measurements of the left ventricle were measured, indexed for body surface area and z-scores were calculated. They were divided into two groups i. e with and without anemia., Results: Out of the 54 children with CKD, children with and without anemia were 34 and 20 respectively. The end-diastolic volume was significantly higher in patients with anemia when compared with those without anemia (46.43 ± 16.49 ml vs 32.51 ± 4.98 ml). The mean left ventricular mass (59.54 ± 23.99 vs 37.24 ± 7.88 g) and end-diastolic thickness of the interventricular septum (0.73 ± 0.14 vs 0.54 ± 0.05 cm) was significantly elevated in CKD children with anemia., Conclusions: Left ventricular hypertrophy along with left ventricular dimension and left ventricular diastolic dysfunction was found to be significantly correlating with the degree of anemia. CKD children with anemia should be screened for underlying cardiac dysfunction and appropriate dietary modification and nutritional rehabilitation for iron deficiency should be addressed., Competing Interests: Declaration of competing interest The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article., (Copyright © 2021 European Society for Clinical Nutrition and Metabolism. Published by Elsevier Ltd. All rights reserved.)

Neutrophils, the early responders of the immune system, eliminate intruders, but their over-activation can also instigate tissue damage leading to various autoimmune and inflammatory disease conditions. As approaches causing neutropenia are associated with immunodeficiency, targeting aberrant neutrophil infiltration offers an attractive strategy in neutrophil-centered diseases including acute lung injury. Rho GTPase family proteins Rho, Rac and Cdc42 play important role as regulators of chemotaxis in diverse systems. Rho inhibitors protected against lung injuries, while genetic Rho-deficiency exhibited neutrophil hyperactivity and exacerbated lung injury. These differential outcomes might be due to distinct effects on different cell types or activation/ inhibition of specific signaling pathways responsible for neutrophil polarity, migration and functions. In this study, we explored neutrophil centric effects of Rho signaling mitigation. Consistent with previous reports, Rho signaling inhibitor Y-27632 provided protection against acute lung injury, but without regulating LPS mediated systemic increase of neutrophils in the circulation. Interestingly, the adoptive transfer approach identified a specific defect in neutrophil migration capacity after Rho signaling mitigation. These defects were associated with loss of polarity and altered actin dynamics identified using time-lapse in vitro studies. Further analysis revealed a rescue of stimulation-dependent L-selectin shedding on neutrophils with Rho signaling inhibitor. Surprisingly, functional blocking of L-selectin (CD62L) led to defective recruitment of neutrophils into inflamed lungs. Further, single-cell level analyses identified MAPK signaling as downstream mechanism of Rho signaling and L-selectin mediated effects. p-AKT levels were diminished in detergent resistance membrane-associated signalosome upon Rho signaling inhibition and blockade of selectin. Moreover, inhibition of AKT signaling as well as selectin blocking led to defects in neutrophil polarity. Together, this study identified Rho-dependent distinct L-selectin and AKT signaling mediated regulation of neutrophil recruitment to inflamed lung tissue., (Copyright © 2021 Elsevier B.V. All rights reserved.)

Background: Cardiovascular disease is the leading cause of morbidity and mortality in children with chronic kidney disease (CKD). We aim to estimate the prevalence of cardiac abnormalities in children up to age 16 years with CKD and their association with various risk factors., Methods: This cross-sectional observational study was conducted on 107 CKD children. We assessed the systolic and diastolic function using 2D echocardiographic evaluation and M-mode measurements of the left ventricle (LV) indexed for BSA and z-scores were calculated. Results were compared with age, sex, stage of CKD, anaemia, estimated glomerular filtration rate (eGFR) and various laboratory parameters., Results: LV diastolic dysfunction was seen in 88%, followed by increased LV dimensions in 33.6%, LV systolic dysfunction in 16%, right ventricle systolic dysfunction in 11.2% while increased pulmonary artery (PA) systolic pressure was seen in 9.3% of cases. LV dimensions correlated directly with parathormone levels and inversely with eGFR, serum calcium and haemoglobin levels. Left ventricular hypertrophy correlated directly with parathormone while inversely with eGFR, serum calcium and haemoglobin. Ejection fraction directly correlated to eGFR and serum calcium while inversely related to parathormone. Left PA pressure directly correlated with age and inversely with eGFR. Right ventricular systolic function assessed by tricuspid annular plane systolic excursion correlated inversely with haemoglobin., Conclusion: LV diastolic dysfunction and increased LV dimensions were the most common cardiac abnormality in children with CKD. LV dimensions correlated directly with parathormone levels and inversely with eGFR, serum calcium and haemoglobin. Diastolic dysfunction positively correlated with serum creatinine and parathormone levels., (© The Author(s) [2021]. Published by Oxford University Press. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Introduction: Kidney Disease: Improving Global Outcomes (KDIGO) 2012 recommends cyclical cyclophosphamide plus glucocorticoids (GC) (modified Ponticelli regimen) or calcineurin inhibitors (CNIs) such as tacrolimus (TAC) or cyclosporine as the first-line agents for the management of primary membranous nephropathy (PMN) that is resistant to antiproteinuric therapy with renin-angiotensin system blockers. However, the long-term outcome of patients treated with CNIs is not known., Methods: We report the outcomes of 70 patients randomized 1:1 to receive modified Ponticelli regimen or TAC/GC for renin-angiotensin system-resistant PMN who were prospectively followed for 6 years. Patients were followed monthly for 12 months, then quarterly for 12 months, and then every 6 months through the end of 6 years., Results: At the end of 6 years, 21 (61.76%) and 9 (28.12%) patients maintained relapse-free remission in modified Ponticelli regimen and TAC/GC groups, respectively (relative risk [RR]: 2.19, 95% confidence interval [CI]: 1.23 to 4.15), and 30 (88.23%) and 17 (53.12%) patients were in remission (including relapses) in modified Ponticelli regimen and TAC/GC groups (RR: 1.66; 95% CI: 1.21 to 2.45), respectively. There was no significant difference in the proportion of patients who had a 40% decline in the estimated glomerular filtration rate (eGFR), death, or end-stage kidney disease between the groups. None of the patients treated with modified Ponticelli regimen reported a solid organ or hematological malignancy., Conclusions: To conclude, in the long-term, modified Ponticelli regimen is superior to TAC/GC as first-line therapy for the management of antiproteinuric-resistant PMN., (© 2021 Published by Elsevier, Inc., on behalf of the International Society of Nephrology.)

The decision regarding dialysis initiation is complex. Awareness that renal replacement therapy should not be regarded as default therapy for every patient with advanced renal failure is necessary. Decision to initiate dialysis and modality should be individualized in a shared decision-making process involving the treating nephrologist and the patient. Patients should receive predialysis education early in the course of chronic kidney disease so as to help prepare them well in advance for this eventuality. Withholding dialysis may be a reasonable option in a certain subset of patients, especially elderly patient with multiple co-morbid illnesses. Comprehensive conservation care should be offered in all patients where the decision to not dialyze is taken., Competing Interests: There are no conflicts of interest., (Copyright: © 2021 Indian Journal of Palliative Care.)

Introduction: Rifampicin is one of the most effective components of anti-tuberculous therapy (ATT). Since rifampicin is a hepatic enzyme (CYP3A4) inducer, in a post-renal transplant recipient, the dose of calcineurin inhibitors needs to be up-regulated and frequently monitored. In resource-limited (low- and lower-middle-income countries) setting this is not always feasible. Therefore, we evaluated a non-rifampicin-based ATT using levofloxacin in kidney transplant recipients., Methods: We retrospectively studied the medical records of renal transplant recipients diagnosed with tuberculosis in our institute between 2014 and 2017. After a brief discussion with patients regarding the nature and course of ATT, those who opted for a non-rifampicin based therapy due to financial constraints were included in the study and followed for a minimum of 6 months period after the completion of ATT., Results: Out of the 550 renal transplant recipients, 67 (12.2%) developed tuberculosis after a median period of 24 (1-228) months following transplantation, of them, 64 patients opted for non-rifampicin-based ATT. The mean age was 37.6 years. Only 25% were given anti-thymocyte globulin based induction, while the majority (56; 87.5%) of them were on tacrolimus-based triple-drug maintenance therapy. Extrapulmonary tuberculosis was noted in 33% of cases, while 12 (18.7%) had disseminated disease. The median duration of treatment was 12 months and the cure rate of 93.7% (n = 60) was achieved at the end of therapy., Conclusion: Levofloxacin based ATT appears to be a safe and effective alternative of rifampicin in kidney transplant recipients who cannot afford heightened tacrolimus dosage., (© 2020 Asian Pacific Society of Nephrology.)

Context: Hypothyroidism is associated with reversible decline in kidney function as measured by estimated glomerular filtration rate (eGFR). eGFR and proteinuria are the most important markers for clinical assessment of kidney function. Though hypothyroidism is associated with proteinuria in cross-sectional data, the impact of treatment on proteinuria is unknown., Objective: This study explores the effect of thyroid hormone replacement therapy on eGFR and 24-hour urine protein excretion in patients with severe primary hypothyroidism., Design and Participants: This study was a prospective, observational cohort study in adults with severe primary hypothyroidism (serum thyrotropin [TSH] > 50 µIU/mL). Individuals with preexisting or past kidney disease, kidney or urinary tract abnormalities, calculi or surgery, diabetes mellitus, or hypertension were excluded. The participants received thyroid hormone replacement therapy. Thyroid functions, eGFR, 24-hour urine protein excretion, and biochemical parameters were measured at baseline and 3 months., Setting: This study took place at a single center, a tertiary care referral and teaching hospital., Results: Of 44 enrolled participants, 43 completed 3 months of follow-up. At 3 months, serum TSH levels decreased and thyroxine levels increased (P < .001 for both). Significant increases in eGFR (mean difference, 18.25 ± 19.49 mL/min/1.73 m2; 95% CI, 12.25 to 24.25, P < .001) and declines in 24-hour urine protein excretion (mean difference, -68.39 ± 125.89 mg/day; 95% CI, -107.14 to -29.65, P = .001) were observed. Serum cholesterol and low-density lipoprotein levels also significantly decreased (P < .001)., Conclusions: Thyroid hormone replacement therapy in patients with severe primary hypothyroidism improves eGFR and decreases 24-hour urine protein excretion, thereby suggesting reversible alterations., (© The Author(s) 2020. Published by Oxford University Press on behalf of the Endocrine Society. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

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., Competing Interests: There are no conflicts of interest., (Copyright: © 2020 Indian Journal of Nephrology.)

Introduction: Fungi are ubiquitous organisms and significantly alter the post-transplant course. They are a major cause of morbidity and mortality and more so in developing countries., Aims: To study the clinical profile, etiology, risk factors, treatment, and outcome of fungal infections in post-renal transplant recipients., Materials and Methods: This was a cross-sectional observational retrospective study from January 2014 to June 2017 wherein renal transplant recipients with invasive fungal infection were included and were followed., Results: Amongst 550 renal transplant recipients, 56 (10.2%) patients developed invasive fungal infection. Mean age of patients was 40.61 ± 10.06 (13-66) years and mean duration of acquiring infection post-transplant was 25.33 ± 23.65 (1-96) months. Male to female ratio was 3:1. Fever was the commonest presentation observed in 89.3% patients. Cough (76.8%), breathlessness (64.3%), sputum (55.3%), hypoxia (50%), and hemoptysis (10.7%) were other common clinical symptoms at presentation. Mean serum creatinine at presentation was 1.70 mg/dl. Most common invasive fungal infection isolated was Mucormycosis 15 (26.7%), foolwed by Aspergillosis 13 (23.2%), Pneumocystis jiroveci 12 (21.4%), Cryptococcus 6 (10.7%), Candida 4 (7.1%), Histoplasmosis 3 (5.3%), Phaeohypomycosis 2 (3.5%), and 5 (8.9%) patients had undetermined fungal etiology. Twenty (35.7%) patients had evidence of dual infection. Use of antithymocyte globulin 27 (48.2%), post-transplant diabetes mellitus 18 (32.1%), Cytomegalovirus (CMV) infection 16 (28.5%), anti-rejection therapy 9 (16%), and Hepatitis C infection 7 (12.5%) were some identified risk factors. Ten (17.8%) patients had graft loss and 12 (21.4%) patients died in the study period., Conclusions: Invasive fungal infection is a serious threat to renal transplant recipients. Patient and graft survival is significantly affected by fungal infection in developing world.

Competing Interests: There are no conflicts of interest.

Histoplasmosis is a rare disease in nonendemic areas. We report a case of a 23-year-old male patient who presented with fever of unknown origin, cytopenias, organomegaly, and allograft dysfunction 4 months after renal transplant with father as donor. Bone marrow examination showed intracellular budding yeast cells, which was confirmed as histoplasmosis by culture of bone marrow biopsy sample. The patient was treated with intravenous liposomal amphotericin and responded well.

Alport syndrome (AS) is an inherited disorder of basement membranes caused by mutations affecting specific proteins of the type IV collagen family, presenting with nephropathy and extrarenal manifestations such as sensorineural deafness and ocular anomalies. Ten percentage to 15% of the patients with AS have autosomal recessive (ARAS) due to mutation in either COL4A3 or COL4A4 gene. We report a novel mutation in the COL4A3 gene in an Indian family with ARAS. The above-mentioned genetic anomaly was a missense variation in exon 26 of the COL4A3 gene (chr2:228137797G>A; c.1891G>A) that resulted in the amino acid substitution of Arginine for Glycine at codon 631 (p.Gly631Arg) that was present in the heterozygous state in the asymptomatic parents and homozygous state in the male offspring who presented with early-onset end-stage renal disease, lenticonus and hearing loss. The patient (male offspring) underwent successful renal transplantation with his mother as a donor., (© 2020 Asian Pacific Society of Nephrology.)

Background: C3 glomerulopathy (C3G) is related to dysfunction of alternative complement pathway (ACP) because of its hyperactivation. Triggering factors and genetic profile are likely to be different in developing countries as compared to the Western world. Data regarding C3G from South Asian is scanty., Study Design: In the present study, 115 patients of C3G from 2012 to 2017 were analyzed. Clinical details were reviewed; serological levels of C3, C4, complement factor H or B and autoantibody testing was done by nephelometry/ELISA. Limited genetics workup for CFH and CFHR5 genes was done., Results: The prevalence of C3G was 1.52%. There was no difference in demographic and histopathologic profiles of C3G patients. Majority of patients had low functional assay and C3 levels. C3 nephritic factor was present in 47.5% of DDD and 38.6% of C3GN. Autoantibodies to CFH were present more often in the patients of C3GN (29.5%) than DDD (12.5%). Autoantibodies to CFB were equally common in both groups. Past history of infections was present in one-third patients and monoclonal paraproteins were present only in two patients. No pathogenic variants were noted in CFH/CFHR5 gene. On follow-up (3.2 + 1.6 years), complete and partial remission was achieved in one-fourth patients and 26% had resistance disease. About 40% progressed to ESRD and 18 underwent renal transplantation of which nine had a post-transplant recurrence., Conclusions: Indian cohort had some differences in the immunological and genetic profile when compared to the Western literature; most significant was the absence of monoclonal immunoglobulins as a trigger for C3G.

Competing Interests: There are no conflicts of interest.

Introduction: Primary membranous nephropathy (PMN) is an autoimmune disease. Both T-regulatory cells (TREGs) and B-regulatory cells (BREGs) are decreased in patients with autoimmune disease. We evaluated the TREG and BREG population in patients of PMN treated with cyclical cyclophosphamide and steroid therapy (cCYC/GC)., Methods: Twenty-four patients with PMN resistant to a restrictive strategy and treated with cCYC/GC therapy and 10 healthy controls were enrolled. The proteinuria, serum creatinine, and serum albumin were tested at monthly intervals and blood samples were collected before starting cCYC/GC and at 6 and 8 (2 months wash out) months of therapy. The peripheral blood mononuclear cells (PBMCs) after staining with fluorochrome-conjugated antibodies were then subjected to flow cytometric analysis for detection of TREGs (CD3+CD4+CD25hiCD127loFoxP3+) and BREGs (CD19+CD5+CD1dhiIL10+). TREGs and BREGs are presented as the percentage of T and B cells, respectively. Cases with remission at month 18 were classified as responders, and those without any remission as nonresponders., Results: Patients with PMN had a lower percentage of TREGs ( P  = 0.07) and BREGs compared with healthy controls ( P  = 0.0007). As compared with baseline, there was a significant increase in both BREGs ( P  = 0.001) and TREGs ( P  = 0.02) with the treatment (8 months). Patients who responded to therapy by 18 months had an increase in TREG ( P  = 0.05) and BREG ( P  = 0.0001) at month 8 compared with baseline., Conclusion: As compared with healthy controls, patients with PMN displayed a lower percentage of BREGs. Both TREGs and BREGs significantly improved with disease-specific therapy. BREGs had an association with clinical activity., (© 2020 International Society of Nephrology. Published by Elsevier Inc.)

Deranged coagulogram is a common problem, which a nephrologist faces before doing a renal biopsy. We describe a rare cause of coagulopathy in a patient with rapidly progressive renal failure due to acquired factor X deficiency caused by systemic light chain amyloidosis (AL). The patient had prolonged prothrombin and activated partial thromboplastin time, which got corrected on mixing with normal plasma, and factor X activity was markedly reduced at 5%. Rectal biopsy and immunofixation electrophoresis established the diagnosis of AL and the patient was started on bortezomib-based chemotherapy. Hence, appropriate coagulation work-up should be conducted in patients with renal dysfunction with prolonged coagulation times, as it can sometimes reveal the underlying diagnosis in situations where renal biopsy could not be done due to high risk of bleeding., Competing Interests: There are no conflicts of interest., (Copyright: © 2020 Indian Journal of Nephrology.)

Background and Aims: The effect of nephrotic syndrome (NS) and its treatment on endothelial dysfunction is not evident. This study assessed endothelial dysfunction in adult-onset NS and its impact of immunosuppressive therapy., Methods: Newly diagnosed patients with adult-onset NS (podocytopathy and primary membranous nephropathy (PMN)) and normal renal function were enrolled. Flow mediated vasodilatation (FMD) assessed endothelial function and CD4 + CD28null T cells, E-selectin and pulse wave velocities (PWV) were measured at baseline and after treatment to characterize this further. Monitoring included monthly proteinuria, serum albumin, creatinine and lipid profile at baseline and post-treatment. The healthy control (HC) included 25 voluntary kidney donors who were assessed for markers of endothelial dysfunction., Results: Fifty participants with new-onset NS were studied. Amongst the NS group, 26 (52%) patients had PMN, while the remaining 24 (48%) had podocytopathy. Twenty-one (88%) patients in the podocytopathy and 18 (69%) patients in the PMN cohort were in either complete or partial remission at the end of 8 months. FMD at baseline in NS patients was significantly lower as compared to HC (p = 0.002) while PWV (p = 0.007), E-selectin (p < 0.001) and CD4 + CD28null T cells (p = 0.003) were significantly higher as compared with HC. Following treatment with immunosuppressive medication, FMD increased from 3 to 8% (p < 0.001). PWV also improved from a baseline of 7.70 to 6.65 m/s (p = 0.001). At the end of 8 months, E-selectin decreased significantly from 127 to 82 ng/ml (p = 0.002) while the CD4 + CD28null T cell population reduced from 5.20 to 3.70% (p = 0.032) of total CD4 + cells. In the PMN cohort, despite significant reduction, E-selectin and CD4 + CD28null T cells at follow-up remained higher than in healthy controls., Conclusion: Immunosuppressive treatment contributes substantially to the improvement of endothelial dysfunction present at baseline in NS patients. Persistent subtle endothelial dysfunction remains in the sub-group of patients with PMN., (Copyright © 2019 Elsevier B.V. All rights reserved.)

Competing Interests: There are no conflicts of interest.

Aim: A significant proportion of patients with minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS) are either steroid dependent or steroid resistant, requiring long-term calcineurin inhibitors (CNI) use. Rituximab has more favourable safety profile. The present study was undertaken to evaluate the efficacy and safety of rituximab in CNI-dependent patients., Methods: This was a prospective observational study conducted from July 2014 to February 2018. Steroid-dependent nephrotic syndrome or steroid-resistant nephrotic syndrome (biopsy proven MCD/FSGS), who were CNI dependent were enrolled. Mean age at enrolment was 22.77 ± 7.45 years. All patients received rituximab at a dose of 375 mg/m 2 at entry in the study. CD-19 levels were monitored monthly and patients having CD-19 levels >5/μL and/or > 1% received additional low-dose (100 mg) of rituximab., Results: A total of 24 patients were followed up for 12 months. At the end of 6 and 12 months, 87.5% and 79.16% of the patients achieved remission, respectively. Eight (33.33%) patients developed relapse. The mean dose of rituximab in the first year was 791 mg. The average cost of rituximab in the first year was 487.17$. Rituximab was well-tolerated, with mild infusion reactions, respiratory tract infection and oral candidiasis in 5 (20.83%), 5 (20.83%) and 1 (4.17%) patient, respectively., Conclusions: CD-19 targeted rituximab is a safe and cost-effective agent in remission maintenance in adults with CNI dependent. Over three-fourths of the patients with CNI-dependent podocytopathy maintain clinical remission with CD-19 targeted rituximab therapy., (© 2018 Asian Pacific Society of Nephrology.)

Background: Recent studies have documented high variation in epidemiologic transition levels among Indian states with noncommunicable disease epidemic rising swiftly. However, the estimates suffer from non-availability of reliable data for NCDs from sub populations. In order to fill the knowledge gap, the distribution and determinants of NCD risk factors were studied along with awareness, treatment and control of NCDs among the adult population in Haryana, India., Methods: NCD risk factors survey was conducted among 5078 residents, aged 18-69 years during 2016-17. Behavioural risk factors were assessed using STEPS instrument, administered through an android software (mSTEPS). This was followed by physical measurements using standard protocols. Finally, biological risk factors were determined through the analysis of serum and urine samples., Results: Males were found to be consuming tobacco and alcohol at higher rates of 38.9% (95% CI: 35.3-42.4) and 18.8% (95% CI: 15.8-21.8). One- tenth (11%) (95% CI: 8.6-13.4) of the respondents did not meet the specified WHO recommendations for physical activity for health. Around 35.2% (95%CI: 32.6-37.7) were overweight or obese. Hypertension and diabetes were prevalent at 26.2% (95% CI: 24.6-27.8) and 15.5% (95% CI: 11.0-20.0). 91.3% (95% CI: 89.3-93.3) of the population had higher salt intake than recommended 5gms per day., Conclusion: The documentation of strikingly high and uniform distribution of different NCDs and their risk factors in state warrants urgent need for evidence based interventions and advocacy of policy measures., Competing Interests: The authors have declared that no competing interests exist.

Background: Complement 3 glomerulopathy (C3G) results from dysfunction of the alternative complement pathway (ACP). No data are available on post-transplant C3G in South Asia., Methods: In this study, renal allograft biopsies of C3G patients performed from 2012 to 2017 were analysed for ACP functional assay (APFA), serum complement levels, complement factor H (CFH), complement factor B (CFB) and autoantibodies to CFH and CFB. Limited genetic screening for CFH / CFHR5 genes was carried out. All study patients were also followed up., Results: A total of 21 cases of C3G were included, of which 11 had native C3G disease (that is, recurrent C3G). Of these 11 recurrent cases, 7 presented with allograft dysfunction and 4 with proteinuria and renal dysfunction. Early post-transplant recurrence (<1 month) was noted in six patients, whereas recurrence in five patients occurred within 8-17 months of transplant. Biopsies showed mild focal mesangial expansion with or without endocapillary proliferation and thrombotic microangiopathy. Rejection was also noted in six patients. APFA/C3 levels were low in all cases. Serum CFH levels were low [dense deposit disease (DDD), 44%; C3 glomerulonephritis (C3GN), 25%], whereas CFB levels were normal. Autoantibodies to CFH, CFB and C3 nephritic factor were present in 11, 0 and 44% of DDD cases, respectively, and in 17, 17 and 33% of C3GN cases, respectively. Genetic analysis revealed only non-pathogenic CFH gene variants (93%). No novel mutation was found. At follow-up (140 months), stable graft was noted in 28% of cases, progressive renal failure in 19%, graft loss in 34%, and 19% of patients died., Conclusion: Post-transplant C3G can present with graft dysfunction and/or proteinuria. Subtle histological findings demand careful interpretation of immunofluorescence results. Autoantibodies to complement pathway regulatory proteins are common, and no novel mutation has been found from limited genetic workup. Clinical outcome is poor., (© The Author(s) 2019. Published by Oxford University Press on behalf of ERA-EDTA.)

Granulomatosis with polyangiitis (GPA) commonly affects upper/lower respiratory tract and kidneys. It causes necrotizing vasculitis of small and medium-sized blood vessels. Gastrointestinal (GI) involvement is an uncommon manifestation of GPA, and presentation with predominant GI manifestation is noteworthy. We report a case of 50-year-old male with melena due to GI vasculitis along with other systemic involvement. The patient was treated with pulse methylprednisolone, cyclophosphamide, and plasmapheresis. To manage the refractory GI bleed, the patient underwent surgical resection, and the histology of the surgical specimen confirmed necrotizing vasculitis., Competing Interests: There are no conflicts of interest., (Copyright: © 2019 Indian Journal of Nephrology.)

Competing Interests: There are no conflicts of interest.

Competing Interests: There are no conflicts of interest.

Competing Interests: There are no conflicts of interest.

Infection of the internal jugular vein (IJV) catheter continues to be a common cause of death in patients with end-stage renal disease undergoing hemodialysis (HD). The present study aimed to evaluate the effectiveness of personal hygiene on the incidence of IJV catheter-related bloodstream infection (CRBSI). A randomized, controlled, parallel, and non-inferiority trial was conducted on patients initiated on maintenance HD via right IJV catheter. Patients were randomly allocated to control and intervention group via computer-generated random table. Intervention package for the intervention group included hand washing (2-4 hourly and whenever visibly dirty), feet washing (12 hourly), and axillary shave (at any point during the study, no hair growth in axilla). Patients were provided with a pamphlet and reinforced to continue package till IJV catheter was in situ . Patients were followed up twice a week for one month from the date of catheter insertion for the incidence of CRBSI. The primary outcome of the study was percentage of patients free from CRBSI. On intention-to-treat analysis, the percentage of patients without CRBSI was 53.7% and 29.3% in the intervention and control arm, respectively [P = 0.04; 25.12% (1.43-45.28%)]. Positive blood cultures were higher in control (73.3%) as compared to the intervention group (28.6%) ( P = 0.19). Personal hygiene interventions are an effective method to reduce the incidence of CRBSIs among population undergoing maintenance HD via non-cuffed IJV catheter., Competing Interests: There are no conflicts of interest.

Cryptococcal infection constitutes around 3% of opportunistic infections in solid organ transplant recipients. Most common organ affected in renal transplant recipients (RTRs) is central nervous system and usually presents with chronic meningoencephalitis (CME). Ischaemic stroke as a consequence of cryptococcal meningoencephalitisis rare and possibly due to the involvement of intracranial vessel by exudates causing vasculitis-related thrombosis. In this context, we describe an unusual case of asymptomatic cryptococcaemia in an RTR, progressing on to acute ischaemic stroke secondary to acute CME with near complete neurological recovery following timely diagnosis, early and appropriate antifungal treatment. The index case attempts to re-emphasise the significance of mandatory screening required to exclude the possibility of dissemination of cryptococcaemia in RTRs besides highlighting the requirement of prolonged induction phase with combination therapy, particularly in presence of stroke., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Splenic artery aneurysm (SAA) is rare, with risk of rupture especially if diameter is >2 cm. It is usually asymptomatic and detected incidentally on imaging either in young pregnant women or elderly cirrhotic patients. Extracranial vascular abnormalities known to be associated with autosomal dominant polycystic kidney disease (ADPKD) include ascending aortic aneurysms, dissections of coronary and vertebral arteries and rarely SAA. We describe a rare case of incidentally detected SAA in a patient with end stage renal disease due to ADPKD during evaluation of persistent gross haematuria, with no evidence of cranial or other extracranial aneurysms., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)