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Background and Aim: Primary glomerular disease accounts for one-sixth of all chronic kidney diseases (CKDs) in India. We remain limited in our ability to effectively treat these conditions because of lack of understanding of the disease mechanisms and lack of predictors to identify the clinical course and therapeutic responsiveness. We propose to develop a network of investigators in glomerular diseases, collect information in a systematic fashion to understand the clinical outcomes, answer translational research questions better, and identify and recruit patients for clinical trials. Materials and Methods: This is a prospective, observational study. The Indian TrANslational GlomerulonephrItis BioLogy nEtwork (I-TANGIBLE) cohort will enroll patients (>18 years) with biopsy-proven minimal change disease (MCD), focal segmental glomerulonephritis (FSGS), membranous nephropathy (MN), IgA nephropathy (IgAN), or membranoproliferative glomerulonephritis (MPGN) (immune complex- and complement-mediated), with first biopsy taken within 2 years of enrollment. Patients with estimated glomerular filtration (eGFR) rate <15 ml/min/1.73 m² for >3 months at the time of screening, kidney transplant or bone marrow transplant recipients, patients with active malignancy, and patients with active hepatitis B/C replication or human immunodeficiency virus (HIV)-I/II will be excluded. Clinical details including history, medication history and details, and family history will be obtained. Consenting patient's blood and urine samples will be collected and stored, aligned to their clinical follow-up. Expected Outcomes: The network will allow accurate ascertainment of disease burden of glomerular diseases across study sites, establishment of the treatment pattern of common glomerular diseases, investigation of medium- and long-term outcomes (remission, relapse, rate of eGFR decline), and building a suitable infrastructure to carry out clinical trials in primary glomerular disease. [ABSTRACT FROM AUTHOR]

The primary objective of the current study was to create a mathematical model utilizing fractional-order calculus for the purpose of analyzing the symmetrical characteristics of Wolbachia dissemination among Aedesaegypti mosquitoes. We investigated various strains of Wolbachia to determine the most sustainable one through predicting their dynamics. Wolbachia is an effective tool for controlling mosquito-borne diseases, and several strains have been tested in laboratories and released into outbreak locations. This study aimed to determine the symmetrical features of the most efficient strain from a mathematical perspective. This was accomplished by integrating a density-dependent death rate and the rate of cytoplasmic incompatibility (CI) into the model to examine the spread of Wolbachia and non- Wolbachia mosquitoes. The fractional-order mathematical model developed here is physically meaningful and was assessed for equilibrium points in the presence and absence of disease. Eight equilibrium points were determined, and their local and global stability were determined using the Routh–Hurwitz criterion and linear matrix inequality theory. The basic reproduction number was calculated using the next-generation matrix method. The research also involved conducting numerical simulations to evaluate the behavior of the basic reproduction number for different equilibrium points and identify the optimal CI value for reducing disease spread. [ABSTRACT FROM AUTHOR]

In this paper, the ABC fractional derivative is used to provide a mathematical model for the dynamic systems of substance addiction. The basic reproduction number is investigated, as well as the equilibrium points' stability. Using fixed point theory and nonlinear analytic techniques, we verify the theoretical results of solution existence and uniqueness for the proposed model. A numerical technique for getting the approximate solution of the suggested model is established by using the Adams type predictor‐corrector rule for the ABC‐fractional integral operator. There are several numerical graphs that correspond to different fractional orders. Furthermore, we present a numerical simulation for the transmission of substance addiction in two scenarios with fundamental reproduction numbers greater than and fewer than one. [ABSTRACT FROM AUTHOR]

It is estimated that 50% of patients with coronavirus disease 2019 (COVID-19) have varying degrees of renal involvement. In this clinical biomarker development research, we examined in a retrospective study design the temporal changes in biochemical laboratory parameters in relation to the development of acute kidney injury (AKI). In a sample of 399 patients admitted from May 2020 to May 2021 to a tertiary health care intensive care unit (ICU), the incidence of AKI was 27.3%, and the median time to AKI was on 7th day of ICU admission. Most common etiology of AKI was kidney hypoperfusion. Within 72 h of developmental of low blood pressure, 63.76% developed AKI. The likelihood of AKI was higher in those with elevated serum ferritin, aspartate transaminase, and thrombocytopenia (low platelet count). A cutoff value of 750.3 ng/mL [area under the ROC curve (AUC) = 0.777] for serum ferritin, and 40.05 U/L for alanine aminotransferase (AUC = 0.677) 1 day before development of AKI displayed, respectively, a sensitivity of 76.2% and 64.3%, whereas the specificity was 69.5% and 64.1%, respectively, for these two biochemical predictors. A cutoff value of platelets (152.50 × 109/L [AUC = 0.75]) measured 4 days before development of AKI, displayed 83.3% sensitivity and 16.4% specificity. Taken together, our study thoroughly examined the temporal association of various clinical and laboratory parameters with AKI and prediction models were developed as per results of the time series data. These observations in a tertiary health care setting contribute to ongoing efforts for biomarker discovery and development using routine biochemical tests so as to forecast AKI in patients with COVID-19. [ABSTRACT FROM AUTHOR]

Background. Allograft rejection postkidney transplantation (KTx) is a major clinical challenge despite increased access to a healthcare system and improvement in immunosuppressive (IS) drugs. In recent years, mesenchymal stromal cells (MSCs) have aroused considerable interest in field of transplantation due to their immunomodulatory and regenerative properties. This study was aimed at investigating safety, feasibility, and immunological effects of autologous MSCs (auto-MSCs) and allogeneic MSCs (allo-MSCs) as a complement to IS drug therapy in KTx patients. Methods. 10 patients undergoing KTx with a living-related donor were analysed along with 5 patients in the control group. Patients were given auto-MSCs or allo-MSCs at two time points, i.e., one day before transplant (D-0) and 30 days after transplant (D-30) at the rate of 1.0- 1.5 × 10 6 MSCs per kg body weight in addition to immunosuppressants. Patients were followed up for 2 years, and 29 immunologically relevant lymphocyte subsets and 8 cytokines and important biomarkers were analysed at all time points. Results. Patients displayed no signs of discomfort or dose-related toxicities in response to MSC infusion. Flow cytometric analysis revealed an increase in B regulatory lymphocyte populations and nonconventional T regulatory cells and a decrease in T effector lymphocyte proportions in auto-MSC-infused patients. No such favourable immune responses were observed in all MSC-infused patients. Conclusion. This study provides evidence that auto-MSCs are safe and well tolerated. This is the first ever report to compare autologous and allogeneic MSC infusion in KTx patients. Importantly, our data demonstrated that MSC-induced immune responses in patients did not completely correlate with clinical outcomes. Our findings add to the current perspective of using MSCs in KTx and explore possibilities through which donor/recipient chimerism can be achieved to induce immune tolerance in KTx patients. [ABSTRACT FROM AUTHOR]

This study focuses on the synchronization analysis of Hindmarsh–Rose neurons coupled through a common memristor (coupled mHRN). Initially, we thoroughly examine the synchronization of two mHRNs coupled via a common memristor before exploring synchronization in a network of mHRNs. The stability of the proposed model is analyzed in three cases, demonstrating the existence of a single equilibrium point whose stability is influenced by external stimuli. The stable and unstable regions are investigated using eigenvalues. Through bifurcation analysis and the determination of maximum Lyapunov exponents, we identify chaotic and hyperchaotic trajectories. Additionally, using the next-generation matrix method, we calculate the chaotic number C 0 , demonstrating the influence of coupling strength on the chaotic and hyperchaotic behavior of the system. The exponential stability of the synchronous mHRN is derived analytically using Lyapunov theory, and our results are verified through numerical simulations. Furthermore, we explore the impact of initial conditions and memristor synapses, as well as the coupling coefficient, on the synchronization of coupled mHRN. Finally, we investigate a network consisting of n number of mHRNs and observe various collective behaviors, including incoherent, coherent, traveling patterns, traveling wave chimeras, and imperfect chimeras, which are determined by the memristor coupling coefficient. [ABSTRACT FROM AUTHOR]

To meet the increased customer demands, microgrid evolved. The structure of microgrid changes dynamically due to the intermittent nature of renewable-based generation, status of the distributed generator and opening of breakers for fault/maintenance. Hence, the magnitude of fault current is dynamic in nature. In order to deal with these dynamic changes, this paper addresses an adaptive central microgrid controller-based protection and relay coordination scheme, which revises the relay settings dynamically (both radial and looped configuration) for every change in topology. In the proposed algorithm, the primary relay responds to a fault immediately since the individual relays are given with fault level setting. For any abnormality in the network, the fault location is determined both via local relay and microgrid central controller (MCC). Hence, even though the local relay fails to identify the fault due to high fault impedance, the MCC locates the fault accurately and isolates the minimum faulty part. The coordination between relays is carried out by MCC in a time-graded manner based on microgrid central protection and relay coordination algorithm. The proposed algorithm is tested using Matlab in a microgrid built based on the IEEE 33 bus distribution network. [ABSTRACT FROM AUTHOR]

This research paper deals with the passivity and synchronization problem of fractional-order memristor-based competitive neural networks (FOMBCNNs) for the first time. Since the FOMBCNNs' parameters are state-dependent, FOMBCNNs may exhibit unexpected parameter mismatch when different initial conditions are chosen. Therefore, the conventional robust control scheme cannot guarantee the synchronization of FOMBCNNs. Under the framework of the Filippov solution, the drive and response FOMBCNNs are first transformed into systems with interval parameters. Then, the new sufficient criteria are obtained by linear matrix inequalities (LMIs) to ensure the passivity in finite-time criteria for FOMBCNNs with mismatched switching jumps. Further, a feedback control law is designed to ensure the finite-time synchronization of FOMBCNNs. Finally, three numerical cases are given to illustrate the usefulness of our passivity and synchronization results. [ABSTRACT FROM AUTHOR]

Keywords: Endosonography; Tuberculosis; Fine needle biopsy; Histoplasmosis EN Endosonography Tuberculosis Fine needle biopsy Histoplasmosis 2204 2206 3 05/18/23 20230601 NES 230601 Histoplasmosis, caused by I Histoplasma capsulatum i , is an uncommon life-threatening infection in immunocompromised patients [[1]]. In tuberculosis (TB) endemic areas like India, TB is the commonest cause of mediastinal lymphadenopathy, even in immunocompromised patients and histoplasmosis is rarely reported [[2]]. [Extracted from the article]

This article interrogates the issue of global asymptotic stability and synchronization analysis of uncertain multi‐agent systems (MASs) with multiple time‐varying delays (discrete and distributed) and impulsive perturbations. In this problem, we prove the existence and uniqueness of the equilibrium point using Brouwer degree properties for the addressed MASs. By applying the approach of Lyapunov–Krasovskii function into the considered system, we achieve the global asymptotic stability and the synchronization criteria by designing the pining control strategy into the MAS. Finally, we provide two numerical calculations along with the computational simulations to check the validity of the theoretical findings reported in this article. [ABSTRACT FROM AUTHOR]

Background The increasing burden of kidney failure (KF) in India necessitates provision of cost-effective kidney replacement therapy (KRT). We assessed the comparative cost-effectiveness of initiating KRT with peritoneal dialysis (PD) or haemodialysis (HD) in the Indian context. Methods The cost and clinical effectiveness of starting KRT with either PD or HD were measured in terms of life years (LYs) and quality-adjusted life years (QALYs) using a mathematical Markov model. Complications such as peritonitis, vascular access–related complications and blood-borne infections were considered. Health system costs, out-of-pocket expenditures borne by patients and indirect costs were included. Two scenarios were considered: Scenario 1 (real-world scenario)—as per the current cost and utilization patterns; Scenario 2 (public programme scenario)—use in the public sector as per Pradhan Mantri National Dialysis Programme (PMNDP) guidelines. The lifetime costs and health outcomes among KF patients were assessed. Results The mean QALYs lived per KF person with PD and HD were estimated to be 3.3 and 1.6, respectively. From a societal perspective, a PD-first policy is cost-saving as compared with an HD-first policy in both Scenarios 1 and 2. If only the costs directly attributable to patient care (direct costs) are considered, the PD-first treatment policy is estimated to be cost-effective only if the price of PD consumables can be brought down to INR70/U. Conclusions PD as initial treatment is a cost-saving option for management of KF in India as compared with HD first. The government should negotiate the price of PD consumables under the PMNDP. [ABSTRACT FROM AUTHOR]

There is evidence to suggest that M‐type phospholipase A2 (PLA2R) antibodies activate the mannose‐binding lectin (MBL) cascade, resulting in glomerular damage and proteinuria in patients with primary membranous nephropathy (PMN). Furthermore, there are few reports indicating that aberrant MBL activation is associated with endothelial dysfunction and accelerated atherosclerosis. While PMN is a common cause of adult nephrotic syndrome, and patients are at increased risk of cardiovascular disease (CVD), there is a lack of research that explores the factors that contribute to this condition. This study aims to determine the MBL levels in PMN and their relation to the clinical activity and endothelial dysfunction in PMN. The MBL levels of 22 biopsy‐confirmed PMN patients were assessed at baseline and after 6 months of immunosuppressive therapy. In order to evaluate endothelial dysfunction in PMN patients, flow‐mediated vasodilation (FMD) was measured at baseline and after treatment. A total of 22 healthy controls were included in this study to measure MBL levels and FMD. A significant difference was observed between MBL levels in PMN patients and healthy controls (p < .01). MBL levels decreased significantly after immunosuppressive therapy (p = .04). The baseline MBL levels and FMD levels exhibited a strong correlation (Spearman correlation coefficient [ρ] = 0.51: p = .01). In conclusion, the study signals the activation of the MBL cascade and its association with endothelial dysfunction in PMN patients. [ABSTRACT FROM AUTHOR]

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]

India is seeing a rapid rise in Coronavirus disease-2019 (COVID-19). Immunosuppression is a possible risk factor for severe COVID-19, although their exact interaction is unclear. A total of 13 cases with active lupus nephritis (LN, with or without extra-renal manifestations) were managed with intense immunosuppression between January 2020 and June 2020 during the COVID-19 pandemic at our center. There were no other comorbidities in any patient. All patients received hydroxychloroquine as a part of standard of care. Vigorous precautionary measures were taken for preventing infection in all. One patient developed acute respiratory distress syndrome but was tested negative for COVID-19. None of the other 12 patients developed symptoms suggestive of COVID-19. We report safe management of patients with active LN with intense immunosuppression along with vigorous precautions amidst the COVID-19 pandemic. The role of hydroxychloroquine along with timely precautions needs to be further explored as protective measures against COVID-19 among systemic lupus erythematosus patients. [ABSTRACT FROM AUTHOR]

Aim: This cross‐sectional survey aimed to determine the prevalence of Interventional Nephrology (IN) practice amongst nephrologists in the Asia‐Pacific Region (APR), specifically related to dialysis access (DA). Methods: The Association of VA and intervenTionAl Renal physicians (AVATAR) Foundation from India conducted a multinational online survey amongst nephrologists from the Asia‐Pacific to determine the practice of IN in the planning, creation, and management of dialysis access. The treatment modalities, manpower and equipment availability, monthly cost of treatment, specifics of dialysis access interventions, and challenges in the training and practice of IN by nephrologists were included in the survey. Results: Twenty‐one countries from the APR participated in the survey. Nephrologists from 18 (85.7%) countries reported performing at least one of the basic dialysis access‐related IN procedures, primarily the placement of non‐tunnelled central catheters (n‐TCC; 71.5%). Only 10 countries (47.6%) reported having an average of <4% of nephrologists performing any of the advanced IN access procedures, the most common being the placement of a peritoneal dialysis (PD) catheter (20%). Lack of formal training (57.14%), time (42.8%), incentive (38%), institutional support (38%), medico‐legal protection (28.6%), and prohibitive cost (23.8%) were the main challenges to practice IN. The primary obstacles to implementing the IN training were a lack of funding and skilled personnel. Conclusion: The practice of dialysis access‐related IN in APR is inadequate, mostly due to a lack of training, backup support, and economic constraints, whereas training in access‐related IN is constrained by a lack of a skilled workforce and finances. [ABSTRACT FROM AUTHOR]

This article examines the role of B cells in the development and treatment of primary podocytopathies, specifically minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS). The study focuses on adults with new-onset nephrotic syndrome (NS) caused by primary podocytopathy and compares them to healthy controls. The findings indicate that B cell proportions differ between children and adults, and specific B cell subsets are associated with disease activity and predict response to steroid treatment. These findings have implications for targeted therapies for primary podocytopathies. However, more research is needed to confirm these results. [Extracted from the article]

Background: Factors associated with pulmonary mucormycosis (PM) among subjects with diabetes mellitus (DM) remain unclear. Following the coronavirus disease (COVID‐19)‐associated mucormycosis outbreak in India, specific environmental exposures (especially cattle dung exposure) were proposed as possible aetiology. We hypothesized that environmental factors are associated with PM. We compared subjects with DM with (cases) and without PM (controls). Methods: In this case‐control study, for each PM case, we included five unmatched diabetic controls (hospital [n = 2], community [n = 3]) without PM. We collected information on demography, COVID‐19 infection, glycated haemoglobin% (HbA1c), the type of house (pucca vs. kutcha) where the participants reside, and other environmental factors. The primary exposure tested was cattle dung exposure (CDE; using cattle dung cakes as fuel or cattle handling). We performed a multivariate logistic regression to explore factors associated with PM and report the association as an adjusted odds ratio (OR) with 95% confidence intervals (CI). Results: We enrolled 39 PM cases and 199 controls (hospital [n = 80], community [n = 119]). CDE (OR 0.68, 95% CI [0.14‐3.31]; p = 0.63) was not associated with increased PM in DM. We found male sex (OR 4.07, 95% CI [1.16‐14.31]), higher HbA1c (OR 1.51, 95% CI [1.18‐16.32]), COVID‐19 (OR 28.25, 95% CI [7.02‐113.6]) and residence at kutcha house (OR 4.84, 95% CI [1.33‐17.52]) associated with PM. Conclusion: Cattle dung exposure was not associated with PM in subjects with DM. Instead, male sex, poor glycaemic control, COVID‐19 and the type of housing were associated with pulmonary mucormycosis. [ABSTRACT FROM AUTHOR]

This article discusses the outcomes of primary membranous nephropathy (PMN) patients who are refractory to immunosuppressants. PMN is a common cause of adult-onset nephrotic syndrome, and current guidelines recommend immunosuppressive therapy. However, a significant proportion of patients do not respond to initial treatment and require rescue therapy. The article presents a systematic review of 18 observational studies that analyzed different rescue therapies for refractory membranous nephropathy. The findings suggest that rituximab, adrenocorticotrophic hormone, and obinutuzumab show promising results, but there is no clear winner. The choice of treatment should be based on individual patient characteristics and side effect profiles. Further controlled studies are needed to investigate these agents. [Extracted from the article]

Background: Reports on long-term complications of childhood-onset nephrotic syndrome (NS), such as obesity, osteoporosis, growth failure, and hypertension, are mostly from developed countries not representing South Asian ethnicities. Furthermore, data on cardiovascular health among patients with childhood-onset NS are limited. Methods: This was an observational study involving patients attending a tertiary care center. Patients aged 15 years and older were examined for long-term complications and remission of NS at their visit in December 2021. Childhood-onset NS meant onset of NS before 10 years of age. Long-term complications included obesity, growth failure, low bone mineral density (BMD) Z score, hypertension, and increased carotid intima-media thickness (cIMT). Long-term remission was defined as no relapse for the last ≥ 3 consecutive years without immunosuppressive medication to maintain remission. Results: Of 101 patients studied (~ 80% with frequent relapsing (FR)/steroid-dependent (SD) NS), the mean age was 17.6 (± 2.4) years at the time of study. Long-term complications were noted in 89.1% of patients which included one or more of the following: obesity (22.7%), growth failure (31.7%), low BMD Z score (53.5%), hypertension (31.7%), and high cIMT (50.5%). Thirty-nine patients (38.6%) were in long-term remission at the time of the study. Growth failure and low BMD Z scores were less frequent in patients with long-term remission compared to those without long-term remission. Conclusions: In patients with childhood-onset NS (predominantly FR/SDNS) who were studied at ≥ 15 years of age, ~ 90% had long-term complications which included high cIMT in 50%. Only ~ 40% were in long-term remission. [ABSTRACT FROM AUTHOR]

Immunoglobulin A nephropathy (IgAN) is a rare but important systemic disease with or without ocular manifestations. We describe 4 cases of IgAN presenting with scleritis and review the various ocular manifestations in patients with IgAN. We found 55 cases with ocular manifestations in patients with prior or newly-diagnosed IgAN described in 38 publications. The most common ocular manifestations of IgAN were episcleritis (23.6%), scleritis (16.4%), hypertensive retinopathy or retinal vasculopathy (20.0%), and uveitis (14.5%). The median age at presentation was 36.5 years, with 54.5% female patients. 61.8% had history of IgAN prior to ocular involvement, while 29.1% had ocular presentations as the first manifestation of IgAN. The majority received systemic corticosteroids and/or immunosuppressants. Additionally, we report 4 women with anterior scleritis and previous diagnosis of IgAN. All 4 were treated with topical and systemic corticosteroids. Three out of 4 patients had no recurrence for at least 1 year since the first presentation. IgAN is a rare but important systemic association to be considered in ocular inflammatory conditions. Timely recognition and comanagement of the disease with nephrologist could reduce disease morbidity. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

The objective of the study is to report the outcomes of COVID-19 in ANCA-associated vasculitis (AAV) patients. This was a registry-based observational study conducted at a tertiary care center in north India. AAV patients with at least one follow-up visit between March 2020 and September 2021 were included. Demographic features, clinical manifestations, disease activity, and treatment details of underlying AAV were noted in all patients. Details of COVID-19 infection including severity, treatment, and outcomes were noted. Predictors of COVID-19 severity were determined using univariate analysis. A total of 33 (18.3%) out of 180 AAV patients contracted COVID-19 infection. Moderate COVID-19 infection was seen in 33.3% and severe or critical infection was seen in 36.3% of patients. Seventeen patients (51.5%) required supplemental oxygen therapy. Nine patients had active disease at the time of COVID-19 infection and three of them died due to COVID-19 infection. The risk of COVID-19 infection and its severity did not differ between patients receiving different immunosuppressants including rituximab induction. Hypothyroidism (p = 0.046) and ocular (p = 0.038) involvement due to AAV predicted the development of moderate to severe/critical COVID-19. Three (9.1%) patients died from COVID-19 and the rate of AAV flare after COVID-19 was similar to that in non-COVID-19 patients (15.3/100 person-year vs. 15.6/100 person-year, p = 0.95). Majority of the patients with AAV had moderate to severe or critical COVID-19 infection. The rate of death due to COVID-19 in AAV is higher than in general population. Use of standard remission induction regimens did not lead to increased risk of COVID-19 infection in our AAV cohort. [ABSTRACT FROM AUTHOR]

Organ transplantation is the primary therapy for organ failure caused by telomere biology disorder (TBD). We describe the first documented case of simultaneous liver and kidney transplantation (SLKTx) for TBD, although the diagnosis of TBD was reached only three months following SLKTx. The patient was born prematurely, displayed growth retardation, and developed chronic kidney and liver diseases. His pre-SLKTx autoimmune, metabolic, and viral assessments were negative, and persistent pancytopenia (bone marrow cellularity 70–80%) was attributed to renal disease-associated bone marrow changes. Following SLKTx, he was discharged with stable graft function on tacrolimus and prednisolone. Although mycophenolate mofetil was discontinued on the second postoperative day, his pancytopenia persisted. Despite extensive evaluations, including drug, immune, nutritional, and viral assessments, all results were negative. A bone marrow biopsy conducted three months post-transplant revealed significant hypocellularity (40-50%). Whole genome sequencing revealed a likely pathogenic variant of the TINF2 gene. The patient was subsequently treated with danazol. At the nine-month follow-up post-SLKTx, he exhibited stable graft function and improved cell counts while maintaining triple-drug immunosuppression. Given the lack of uniform diagnostic criteria for TBD, healthcare providers must be vigilant with patients presenting with multi-organ failure and persistent cytopenias. Effective pre-transplant screening for TBD can lead to timely diagnoses, better management, and improved post-transplant outcomes. [ABSTRACT FROM AUTHOR]

Posterior reversible encephalopathy syndrome (PRES) is a clinico-radiological phenomenon commonly associated with kidney diseases, especially chronic kidney disease. A consequence of endothelial dysfunction, PRES is usually associated with uncontrolled blood pressures and can rarely have atypical radiological findings involving the brain stem and spinal cord, called posterior reversible encephalopathy with spinal cord involvement (PRES-SCI). These atypical features may be confused with other etiologies causing a delay in diagnosis and management. We describe a young male patient who presented with neurological symptoms suggestive of PRES; however, the atypical radiological findings along with concomitant rapidly progressive glomerulonephritis led to a diagnostic dilemma. Repeat neuro-imaging after appropriate blood pressure control showed disappearance of the lesions confirming the diagnosis of PRES-SCI, and kidney biopsy showed advanced IgA nephropathy. Knowledge of atypical features of PRES is crucial amongst nephrologists as it is a common association with kidney disease and prompt identification and management avoid irreversible sequelae and unnecessary investigations. [ABSTRACT FROM AUTHOR]

For the foreseeable future, vaccines are the cornerstone in the global campaign against the Coronavirus Disease-19 (COVID-19) pandemic. As the number and fatalities due to COVID-19 decline and the lockdown anywise rescinded, we recognize an increase in the incidence of autoimmune disease post-COVID-19 vaccination. However, the causality of the most vaccine-induced side effects is debatable and, at best, limited to a temporal correlation. We herein report a case of a 51-year-old gentleman who developed Anti-Neutrophil Cytoplasmic Antibody (ANCA)-associated vasculitis (AAV) 2 week post-COVID-19 vaccination. The patient responded favorably to oral steroids and rituximab. Additionally, we conducted a case-based review of vaccine-associated AAV describing their clinical manifestations and treatment response of this emerging entity. [ABSTRACT FROM AUTHOR]

Primary membranous nephropathy (PMN), an autoimmune disease, is the most common cause of nephrotic syndrome in middle-aged non-diabetic adults. PMN pathophysiology includes Th1/Th2 paradigm. The IL-23/IL-17 pathway is implicated in autoimmune kidney disorders, but no study has examined its relationship with PMN. In several unrelated studies, PMN patients reported to have paradoxical IL-17 levels. This manuscript describes the best possible association of IL-23/IL-17 axis with PMN. Biopsy-proven PMN patients and age, gender-matched healthy controls were enrolled. Serum-PLA2R (Euroimmune, Germany), IL-23 and IL-17 (R&D; USA), was measured using ELISA along with biochemical parameters. Appropriate statistical tools were used for analysis. One hundred eighty-nine PMN patients (mean age 41.70 ± 12.53 years) and 100 controls (mean age 43.92 ± 10.93 years) were identified. One hundred forty were PLA2R-related. PMN patients had median proteinuria, serum albumin, and creatinine of 6.12 (3.875, 9.23) g/day, 2.32 (1.96, 2.9) g/dl, and 0.89 (0.7, 1.1) mg/dl, respectively. IL-17, but not IL-23, was significantly increased in PMN patients compared to controls (IL-17, median: 12.07 pg/ml (9.75, 24.56) vs median: 9.75 pg/ml (8.23, 17.03) p = 0.0002); (IL23, median: 6.04 pg/ml (4.22, 10.82) vs median: 5.46 pg/ml (3.34, 9.96) p = 0.142). IL-17 and IL-23 correlated significantly (p 0.05) in PMN patients, and similar trend was seen when grouped into PLA2R-related and -unrelated groups. The levels of IL-23 (p = 0.057) and IL-17 (p = 0.004) were high in MN patients that did not respond to the treatment. The current finding may indicate or suggest the involvement of IL-23/IL-17 PMN pathogenesis. A comprehensive investigation is needed to evaluate IL-23/IL-17 axis with renal infiltrating immune cells, and external stimuli. [ABSTRACT FROM AUTHOR]

Introduction. Anti-Compliment Factor H antibody hemolytic uremic syndrome (AFH-HUS) is a common cause of paediatric atypical HUS in India. We wanted to study the outcome of patients receiving less than recommended plasma exchange (PLEX) but adequate immunosuppression, with respect to hypertension, preservation of renal function and proteinuria. Methods. A retrospective study was performed in 15 children admitted from 2016 to 2018 with AFH-HUS. Follow up details including physical examination, hematological parameters, renal function test and urine examination performed at 3, 6, and 12 months were noted. Risk stratification and staging for chronic kidney disease (CKD) were done according to the Kidney Disease Improving Global Outcomes (KDOQI) guidelines. Standard statistical tests which were appropriate were used. Results. Mean age of our study cohort was 7.8 ± 1.9 years. 14 children had hypertension. Mean nadir hemoglobin was 5.8 ± 1.0 g/dL and platelet = 58 ± 37.7 × 109 cells/L. Median anti factor H (AFH) level was 316 AU/mL (150 to 452). Hemodialysis was required in 7 children. Fourteen children received PLEX with a mean of 11 ± 6 cycles. Thirteen children received 6 cycles of intravenous cyclophosphamide. After six months, therapy was switched to mycophenolate mofetil in 4 children, steroids alone in 2 children and 9 children with azathioprine. On follow-up, risk of CKD reduced from 80% at 3 months to 26% at 12 months (P = .01). Only 40% patients had CKD stage 2 at the end of 12 months (mean eGFR = 95.0 ± 19.4 mL/min/1.73m2). Conclusion. The adequate number of PLEX needed in AFH-HUS needs further studies. Till such reports come, PLEX in recommended strategies or lesser, if not available, with immunosuppression in AFH-HUS can decrease progression to CKD. [ABSTRACT FROM AUTHOR]

Background: Peritoneal dialysis (PD) as a modality of kidney replacement therapy (KRT) is largely underutilized globally. We analyzed PD utilization, impact of economic status, projected growth and impact of state policy(s) on PD growth in South Asia and Southeast Asia (SA&SEA) region. Methods: The National Nephrology Societies of the region responded to a questionnaire on KRT practices. The responses were based on the latest registry data, acceptable community‐based studies and societal perceptions. The representative countries were divided into high income and higher‐middle income (HI & HMI) and low income and lower‐middle income (LI & LMI) groups. Results: Data provided by 15 countries showed almost similar percentage of GDP as health expenditure (4%–7%). But there was a significant difference in per capita income (HI & HMI ‐US$ 28 129 vs. LI & LMI ‐ US$ 1710.2) between the groups. Even after having no significant difference in monthly cost of haemodialysis (HD) and PD in LI & LMI countries, they have poorer PD utilization as compared to HI & HMI countries (3.4% vs. 10.1%); the reason being lack of formal training/incentives and time constraints for the nephrologist while lack of reimbursement and poor general awareness of modalities has been a snag for the patients. The region expects ≥10% PD growth in the near future. Hong Kong and Thailand with 'PD first' policy have the highest PD utilization. Conclusion: Important deterrents to PD underutilization were lack of PD centric policies, lackadaisical patient/physician's attitude, lack of structured patient awareness programs, formal training programs and affordability. SUMMARY AT A GLANCE: This questionnaire study examined the dialysis practice and utilization of peritoneal dialysis (PD) in the south and southeast Asia region and found that low and low‐middle income countries had worse PD utilization rate. The major barriers were related to healthcare policy, lack of training programs and patients' and physicians' awareness. [ABSTRACT FROM AUTHOR]

Proliferative lupus nephritis (LN) is histologically characterized by endocapillary hypercelMarity and large immune deposits on light microscopy. Immunofluorescence shows almost all immunoglobulins and complement staining. The presence of antinuclear antibodies (ANA) is important for diagnosing systemic lupus erythematosus (SLE). Absence of ANA rules out the possibility of SLE according to the 2019 European League Against Rheumatism/American College of Rheumatology classification criteria for SLE. We report a young boy with fever, nephrotic-nephritic syndrome and pancytopenia consistent with hemophagocytic lymphohistiocytosis. Renal biopsy was consistent with LN; however, his initial ANA was negative. In view of pathological features of LN and persistent pancytopenia, high dose steroid therapy was started. Repeat ANA, done during the illness, turned positive. In this report, we describe the relevance of pathological patterns and the uncertainties of ANA positivity in making a diagnosis of SLE. [ABSTRACT FROM AUTHOR]