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Background Unlike adults, primary membranous nephropathy (PMN) comprises only 1-2% of childhood nephrotic syndrome. The clinical behaviour of PMN in children is not explicit and we report upon clinical presentation and outcome. Methods This prospective study includes children and adolescents (< 20 years) with biopsy-proven PMN without secondary causes. Anti-PLA2R assessment: before and after completing therapy. Outcome: percentage of patients achieving remission. Results Study cohort included 48 (M:F ratio 1.1:1) patients and median age 17 (IQR 15-18) years, with 35 (72.9%) PLA2R related. Median interval from symptom onset to presentation was 5 months, where median proteinuria, serum albumin and creatinine were 4.9 g/day, 2.1 g/dL and 0.63 mg/dL, respectively. Forty-seven patients received immunosuppressive therapy, with various agents used as first-line therapy: cyclical CYC/GC (53.1%), CNI/GC (21.3%), rituximab (14.9%), prednisolone alone (4.3%), azathioprine (4.3%) and mycophenolate mofetil (2.1%). Median follow-up was 29 (14, 59) months. At 6 months, 11 (24.4%) and 17 (37.7%) had complete remission (CR) or partial remission (PR), while at last follow-up (median 29 months), 20 (45.4%) and 14 (31.8%) had CR and PR respectively. No significant differences in outcome were observed with different agents. A total of 60% patients treated with rituximab as first line/for relapsing disease, and all cases with resistant disease receiving rituximab had CR or PR at last follow-up. PLA2R antibody presence was associated with clinical outcome. Conclusions Three-quarters of PMN in children and adolescents is PLA2R related and two-thirds respond to immunosuppressive therapy. Rituximab is a promising agent to manage PMN in children. Anti-PLA2R is associated with clinical outcomes., Author(s): Raja Ramachandran [sup.1] , Saurabh Nayak [sup.1] , Vinod Kumar [sup.2] , Ashwani Kumar [sup.3] , Neha Agrawal [sup.1] , Ritika Bansal [sup.1] , Karalanglin Tiewsoh [sup.4] , Ritambhra [...]

Introduction: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) rarely coexist with systemic thrombotic microangiopathy (TMA).The TMA can be in the form of either hemolytic uremic syndrome (HUS) or thrombotic thrombocytopenic purpura (TTP). This review explores the clinical characteristics, histopathological findings, treatment options, and outcomes in patients presenting as AAV with coexisting HUS/TTP. Methods: We conducted a search on the PubMed database and additional searches from January 1998 to September 2022 using the following terms: "ANCA", "Antineutrophil cytoplasmic antibody", "thrombotic thrombocytopenic purpura", "TTP", "thrombotic microangiopathy", "haemolytic uremic syndrome", and "HUS". We excluded articles that described renal-limited TMA. Two authors independently reviewed the full texts and extracted all critical data from the included case reports. Finally, we included 15 cases for this review. Hematological remission and kidney recovery in the form of independence from dialysis was assessed. Results: The median age of the patients was 61 years and a majority of them were females (66.7%). Myeloperoxidase (MPO)-ANCA positivity (66.67%) was more common than proteinase 3 (PR3)-ANCA positivity (33.33%). All patients had laboratory parameters consistent with systemic TMA (HUS or TTP), and only six (out of 11) cases showed histological features of renal TMA. Ten had crescentic glomerulonephritis, and two had advanced degrees of chronicity in histology. Eighty-six percent of cases had hematological remission, and sixty percent of cases became dialysis-independent after treatment. Conclusion: In conclusion, kidney outcome was worse in patients who manifested both AAV and systemic TMA. A paucity of literature regarding this diagnostic quandary calls for avid reporting of such cases. [ABSTRACT FROM AUTHOR]

Objectives SLE is associated with significant mortality, and data from South Asia is limited. Thus, we analysed the causes and predictors of mortality and hierarchical cluster-based survival in the Indian SLE Inception cohort for Research (INSPIRE). Methods Data for patients with SLE was extracted from the INSPIRE database. Univariate analyses of associations between mortality and a number of disease variables were conducted. Agglomerative unsupervised hierarchical cluster analysis was undertaken using 25 variables defining the SLE phenotype. Survival rates across clusters were assessed using non-adjusted and adjusted Cox proportional-hazards models. Results Among 2072 patients (with a median follow-up of 18 months), there were 170 deaths (49.2 deaths per 1000 patient-years) of which cause could be determined in 155 patients. 47.1% occurred in the first 6 months. Most of the mortality (n  = 87) were due to SLE disease activity followed by coexisting disease activity and infection (n = 24), infections (n = 23), and 21 to other causes. Among the deaths in which infection played a role, 24 had pneumonia. Clustering identified four clusters, and the mean survival estimates were 39.26, 39.78, 37.69 and 35.86 months in clusters 1, 2, 3 and 4, respectively (P  < 0.001). The adjusted hazard ratios (HRs) (95% CI) were significant for cluster 4 [2.19 (1.44, 3.31)], low socio-economic-status [1.69 (1.22, 2.35)], number of BILAG-A [1.5 (1.29, 1.73)] and BILAG-B [1.15 (1.01, 1.3)], and need for haemodialysis [4.63 (1.87,11.48)]. Conclusion SLE in India has high early mortality, and the majority of deaths occur outside the health-care setting. Clustering using the clinically relevant variables at baseline may help identify individuals at high risk of mortality in SLE, even after adjusting for high disease activity. [ABSTRACT FROM AUTHOR]

Background: Chronic kidney disease (CKD) patients might be zinc deficient due to compromised nutritional intake and dietary protein restrictions; protein foods being rich in zinc. Zinc deficiency might cause increased oxidative stress and consequently reduced antioxidant parameters. Tailored dietary counseling along with zinc supplementation may play a major role in affecting the zinc and antioxidant status of patients. Aim: The aim of this study was to investigate the effect of zinc supplementation and dietary counseling on oxidative stress and antioxidant parameters in CKD patients. Materials and Methods: 185 subjects were enrolled for the study (145 were predialysis patients [Group-1 (Stage-3 CKD) and Group-2 [Stage-4 CKD]) on the basis of Kidney Disease Outcomes Quality Initiative CKD stages from 1 to 5; and 40 apparently healthy controls. Zinc supplementation alone (Intervention 1) was given to 50 CKD patients (30 Group-1 and 20 Group-2). Diet counseling (Intervention 2) consisted of 45 patients (25 Group-1; 20 Group-2). 50 patients (30 Group-1 and 20 Group-2) received both zinc supplementations along with diet counseling (Intervention 3). Dietary zinc status, oxidative stress and antioxidants were accessed for all subjects. Results: Intervention-1 and 3 groups exhibited highly significant improvement in antioxidant status and lowering of oxidative stress. Conclusion: Improving zinc status through dietary counseling and supplementation may help in combating enhanced oxidative stress by improving antioxidant parameters in renal failure patients. [ABSTRACT FROM AUTHOR]

Purpose: To evaluate the role of improvement in inflammatory oxidative stress by periodontal therapy (NSPT) in chronic kidney disease (CKD) subjects. Methods: 50 stable subjects of CKD (stage III-IV) and having chronic periodontitis were enrolled for the present study. Group A (control group) subjects who did not receive NSPT and Group B (test group) subjects who received NSPT. Oral hygiene instructions were given to both groups, malondialdehyde *IDA) in gingival crevicular fluid (GCF) and serum, albumin creatinine ratio (ACR), urine protein creatinine ratio (UPCR), pocket depth (PD), clinical attachment loss (CAL), plague index (PI), gingival index (Gl), Interleukin 1-beta (IL-1 13), high sensitivity C-reactive protein (hs-CRP) in serum were assessed at baseline and 6 months. Results: There was a significant difference observed in PD, CAL. PI, Gl and MDA-GCF, hs-CRP, IL-113 in serum following NSPT in the test group compared to the control group at 6 months follow up. Within the limitations of the study, the results revealed that NSPT can be used as an effective method to reduce inflammatory oxidative stress in CKD subjects and improve renal health. Further well-designed longitudinal trials with larger sample size and longer follow ups are needed. [ABSTRACT FROM AUTHOR]

Byline: Aman. Sharma, Roopa. Rajan, Manish. Modi, Benzeeta. Pinto, Aadhaar. Dhooria, Manish. Rathi, Tarun. Mittal, Susheel. Kumar, Kusum. Sharma, Varun. Dhir, Ritambhra. Nada, Ranjana. Minz, Surjit. Singh Background: Neurological manifestations [...]