Minimal Change Nephrotic Syndrome Research Articles

Circulating RAC1 contributed to steroid-sensitive nephrotic syndrome: Mendelian randomization, single-cell RNA-sequencing, proteomic, and experimental evidence

Objectives The small GTPase Rac1 (RAC1) has been linked to podocyte disorders and steroid-sensitive nephrotic syndrome (SSNS). The aim of this study was to explore and validate the potential causal association between circulating RAC1 and SSNS. Methods The association between circulating RAC1 and SSNS at both gene expression and proteomic levels was investigated using Mendelian randomization analysis, and further validated by single-cell RNA-sequencing, proteomic analysis, and experimental studies. The genetic instruments comprised cis-expression quantitative trait loci (cis-eQTLs) associated with RAC1 gene expression and protein QTLs correlated with plasma RAC1 protein levels. Causal associations were estimated utilizing the inverse variance weighted and MR-PRESSO methods. Validation of RAC1 expression was conducted through single-cell RNA-sequencing of peripheral blood mononuclear cells from patients with SSNS and healthy controls. Proteomic analysis was performed among patients with minimal change nephrotic syndrome. Experimental validation was conducted using a puromycin aminonucleoside (PAN)-induced nephrosis model. Results Increased expression of RAC1 was associated with a higher risk of SSNS (gene expression level: odds ratio [OR], 1.53; 95% confidence interval [CI], 1.02–2.28; protein level: OR, 1.82; 95% CI, 1.05–3.17). The results of MR-PRESSO were consistent (gene expression level: OR, 1.49; 95% CI, 1.17–1.92; protein level: OR, 1.81; 95% CI, 1.16–2.85). Single-cell RNA sequencing and proteomic analysis confirmed elevated RAC1 expression in patients with SSNS compared to healthy controls. Experimental data further supported increased RAC1 expression in PAN-induced nephropathy. Conclusions Increased expression of RAC1 might be causally associated with SSNS, suggesting that targeting RAC1 might represent a potential therapeutic strategy for SSNS.

Clinical Characteristics of Young and Elderly Patients with Minimal Change Nephrotic Syndrome

Clinical Characteristics of Young and Elderly Patients with Minimal Change Nephrotic Syndrome

Synaptobrevin, a Major Protein of v-SNARE, Participates in Maintenance of Foot Processes of Podocytes: Downregulation of Synaptobrevin Is an Initiation Event Leading to Podocyte Injury in Minimal Change Nephrotic Syndrome (MCNS) Model

Synaptobrevin, a Major Protein of v-SNARE, Participates in Maintenance of Foot Processes of Podocytes: Downregulation of Synaptobrevin Is an Initiation Event Leading to Podocyte Injury in Minimal Change Nephrotic Syndrome (MCNS) Model

Intravenous methylprednisolone for nephrotic syndrome with minimal change lesions in adults: a randomized controlled trial.

Patients with minimal change nephrotic syndrome (MCNS) usually experienced severe edema which can affect the absorption of oral corticosteroid during the first 2 weeks. We conducted a randomized controlled trial to compare the efficacy of intravenous isovalent methylprednisolone induction followed by oral prednisone therapy with conventional oral prednisone therapy in highly edematous MCNS patients, aiming to provide a better therapy for MCNS patients. A single-center, open-label, parallel-arm randomized controlled trial was performed in the Nephrology Department of the Affiliated Hospital of Guangdong Medical University. Patients who met the inclusion were enrolled in our study from May 2015 to October 2020, and were randomized to receive conventional oral steroid or 2 weeks intravenous methylprednisolone followed by oral prednisone. 117 patients were enrolled and randomly assigned to either the sequential group (N=57) or the oral group (N=60). Total remission rate in the sequential group was higher than the oral group after treatment for 2 weeks and 4 weeks (P=0.032, P=0.027). Complete remission rate was higher in the sequential group than in the oral group (63.3% vs. 41.5%, P=0.031) after treatment for 2 weeks. The time to achieve CR is shorter in the sequential group than the oral group, with a statistically significant difference (14.0 days, 95% CI [13.5 to 14.5] vs. 16.0 days, 95% CI [12.7 to 19.3], P=0.024). There were no significant difference in relapse rate (24.5% vs 28.3%, P=0.823) and time to relapse (155±103 days vs 150.7±103.7 days, P=0.916) between two groups. This study suggested that highly edematous MCNS patients received intravenously isovalent methylprednisolone induction therapy follow by oral prednisone achieved earlier remission than the conventional oral prednisone regimen without differences in relapse rates or adverse effects. Short-term intravenous methylprednisolone followed by oral prednisone may be a better choice for MCNS patients with highly edema.

Aberrant localization of β1 integrin in podocyte cytoplasm of primary FSGS with cellular lesion.

Podocyte detachment is a major trigger in pathogenesis of focal segmental glomerulosclerosis (FSGS). Detachment via β1 integrin (ITGB1) endocytosis, associated with endothelial cell injury, has been reported in animal models but remains unknown in human kidneys. The objectives of our study were to examine the difference in ITGB1 dynamics between primary FSGS and minimal change nephrotic syndrome (MCNS), among variants of FSGS, as well as between the presence or absence of cellular lesions (CEL-L) in human kidneys, and to elucidate the pathogenesis of FSGS. Thirty-one patients with primary FSGS and 14 with MCNS were recruited. FSGS cases were categorized into two groups: those with CEL-L, defined by segmental endocapillary hypercellularity occluding lumina, and those without CEL-L. The podocyte cytoplasmic ITGB1 levels, ITGB1 expression, and degrees of podocyte detachment and subendothelial widening were compared between FSGS and MCNS, FSGS variants, and FSGS groups with and without CEL-L (CEL-L( +)/CEL-L( -)). ITGB1 distribution in podocyte cytoplasm was significantly greater in CEL-L( +) group than that in MCNS and CEL-L( -) groups. ITGB1 expression was similar in CEL-L( +) and MCNS, but lower in CEL-L( -) compared with others. Podocyte detachment levels were comparable in CEL-L( +) and CEL-L( -) groups, both exhibiting significantly higher detachment than the MCNS group. Subendothelial widening was significantly greater in CEL-L( +) compared with CEL-L( -) and MCNS groups. The findings of this study imply the existence of distinct pathological mechanisms associated with ITGB1 dynamics between CEL-L( +) and CEL-L( -) groups, and suggest a potential role of endothelial cell injury in the pathogenesis of cellular lesions in FSGS.

Herpes Zoster Infection in A Case of Nephrotic Syndrome (On Alternate Day Prednisolone Therapy) - A Case Report

Herpes Zoster infection is caused by Varicella Zoster virus. Its characteristic feature is the appearance of unilateral clustered vesicles on an erythematous base. The vesicles heals within two weeks from appearance of vesicles. The symptoms of Nephrotic Syndrome include severe proteinuria, edema, hypoalbuminemia and hyperlipidemia. Minimal Change Nephrotic Syndrome is the most prevalent type of nephrotic syndrome in children, for which prednisolone is the preferred medication. Administration of prolonged corticosteroid causes decreased cell mediated immunity which causes immunosuppression and therefore chance of infection increases. Herpes zoster infection is usually a self-limiting disease. It is usually diagnosed clinically on the basis of characteristic appearance of the rash. Treatment is mainly by administration of oral acyclovir for 7 days. Severe illness may require hospitalization for intravenous acyclovir. In individuals with nephrotic syndrome on corticosteroids or other immunosuppressive medications, complication arising from varicella infections can be life-threatening. Keywords: Herpes zoster, Nephrotic syndrome, Minimal Change Nephrotic Syndrome, Prednisolone, Acyclovir, Cell mediated immunity

#1883 SUrvey of renal Biopsy database and Anti-cancer dRUg therapy in Japan (SUBARU-J study)

Abstract Background and Aims The frequency of consultations for renal complications associated with anti-cancer drug therapy has greatly increased in recent years, but the actual situations remain yet to be fully understood. We aimed to investigate the clinical outcomes of anti-cancer drug therapy-associated renal complications, using the Japan Renal Biopsy Registry (J-RBR), a nationwide registry system in Japan. Method This nationwide survey was performed in 449 cases with “drug-induced” checked in J-RBR database from 2018 to 2021. In total 449 subjects, top-ranked histopathological diagnoses were as follows, TIN (49.8%), TMA (14.5), MN (10.0), MCNS (6.5), FSGS (4.4), Vasculitis (2.2) and others (12.2). Regardless of the causative agent, TIN was the most common diagnosis. Though, it was followed by TMA or MN in anti-cancer drug (n = 139)- or non-anticancer drug (n = 190)-related cases, respectively. Because there was also TMA included as 2nd highest in causative-agent unidentified cases (n = 120), we employed total 259 subjects of anti-cancer drug-related and causative-agent unidentified for this survey. Our surveillance files investigating on renal pathology, medications and cancer prognosis were distributed. Of the 224 cases with a file uploaded, a total of 135 anti-cancer drug-related cases confirmed by the information were analyzed. Patients’ clinical data collected at pre- and post-biopsy as well as histopathological diagnoses were used for epidemiological and clinicopathological analyses. Mann-Whitney U test or chi-square test was used for continuous or categorical variables. Overall survival (OS) was estimated using Kaplan–Meier method and compared by log-rank test. Results In total 135 subjects, top-ranked histopathological diagnoses were as follows: tubulointerstitial nephritis (TIN; 64 cases), thrombotic microangiopathy (TMA; 48), focal segmental glomerulosclerosis (FSGS; 5), IgA nephropathy (IgAN; 4), acute tubular necrosis (ATN; 3), minimal change nephrotic syndrome (MCNS; 3), crescentic glomerulonephritis (CrGN; 2), membranoproliferative glomerulonephritis (MPGN; 2) and others (4). 80% of TIN and 75% of TMA, the most frequent diagnoses in our survey, were associated with immune-checkpoint inhibitors (ICI) and anti-angiogenic therapy (AAG), respectively. All IgAN, MCNS and CrGN cases were ICI-related. Proton pump inhibitors and antibiotics were used concomitantly in TIN (p = 0.054) and TMA (p < 0.001), respectively. Among cases with TIN, their OS in the subgroups of targeted therapy (Tx), and the combination with Tx and conventional cytotoxic agents (Combi) seemed to be worse than that of cytotoxic agents alone (CTx); it may be due to more cases in the Tx and Combi subgroups with treatment response category of progressive disease (by RECIST criteria). In contrast, among TMA cases, OS in each of the three subgroups of Tx, CTx, and Combi seemed to be overlapped, suggesting the prognostic impact of TMA on OS. The serum LDH level was higher in TMA associated with CTx (CTx-TMA) than that with others (Fig. 1: 393.2 vs. 225.1 U/L, p = 0.06). Of note, Kaplan-Meier curves for OS in TMA cases with increased LDH (>220 U/L) showed poor prognosis in CTx-TMA than in others-TMA (Fig. 2: p = 0.018). Conclusion Our nationwide survey using J-RBR registry database with renal biopsy data provides a snapshot of kidney complications associated with anti-cancer drug therapy. Higher LDH in CTx-TMA cases may be associated with systemic TMA, probably leading to the poor prognosis. To explore the detail of anti-cancer drug-associated TMA and other complications, further research employing more sample size is warranted in the future.

A case diagnosed with IgA nephropathy during a complete remission of minimal change nephrotic syndrome treated with rituximab.

We herein report a case of IgA nephropathy in a 20-year-old male who maintained a complete remission of minimal change nephrotic syndrome (MCNS) through the administration of rituximab (RTX). He was diagnosed with nephrotic syndrome at 4years of age. After he relapsed frequently, he was diagnosed with MCNS at 8years of age based on the findings of a kidney biopsy. At 13years of age, RTX therapy was initiated to maintain a complete remission after steroid treatment. MCNS recurred twice, including the time in which the interval between the RTX administrations was long. Whenever he relapsed, remission induction was achieved using steroids, and remission maintenance was achieved using RTX. Five months after the 7th RTX administration, the serum IgA level started to increase. After the 9th RTX administration, he demonstrated microhematuria despite the urinary protein level indicating complete remission. At the 10th administration, the urinary protein and the red-blood cell casts were also observed. A renal biopsy was performed 84months after the initial administration of RTX, and the patient was diagnosed with complications of IgA nephropathy. RTX is not considered to be a useful treatment for IgA nephropathy. The reasons for this are due to the fact that IgA1 does not decrease even following the administration of RTX, because B cells residing in the mucosa may not be deleted by RTX, and IgA production may also continue due to the presence of CD20- long-lived plasma cells. Even when administering RTX, if there are findings of glomerulonephritis on urine testing, the possibility of IgA nephropathy must be considered.

Insights into Nephrotic Syndrome in Pediarics: Case Series Analysis

Nephrotic syndrome (NS) is one of the most common childhood kidney diseases. NS can affect children of any age from infancy to adolescence and predominantly occurs in the age group of 1–6 years. It is an illness caused by idiopathic diseases like minimal change nephrotic syndrome (MCNS) and focal segmental glomerulosclerosis (FSGS), membrane proliferative Glomerulonephritis, membranous Glomerulonephritis and is characterized by increased permeability across the glomerular filtration barrier. NS is classified as primary, secondary, and congenital. It consists of four clinical features like severe proteinuria, edema, hyperlipidaemia, hypoalbuminemia. The main cause of nephrotic syndrome are diseases associated with drugs and rarely genetic disorders. The pathogenesis of the disease affects various biological functions linked to loss of proteins negatively, which results in systemic complications which may be disease and drug-associated complications. Using drugs to treat NS leads to several complications which include improper growth, metabolism, behavior change inpatient. Itis a chronic relapsing disease for most of the steroid response drugs for treatment of nephrotic syndrome. Glucocorticoids, immunosuppressants and biological agents are used for the treatment of the diseases. In this case series, we presented a case series of nephrotic syndrome in pediatrics. Total 4 patients were taken into considerations. The patients came with a chief complaints of swelling of face, foamy urine, abdominal pain, dark coloured urine with decreased urine output, joint pains. The laboratory investigations for all the 4 patients showed that there were decrease in albumin levels (hypoalbuminemia), decrease in serum creatinine levels and protein in urine, although kidney biopsy couldn’t be perform but the diagnosis was made based on clinical and laboratory findings. The patients were mainly treated with prednisolone, pantoprazole, enalapril, nifedipine, amoxiclav, and even albumin transfusion were done. NS was successfully managed and fortunately the patients recovered.

Safety and efficacy of peptide receptor radionuclide therapy in neuroendocrine tumors: A single center experience.

Peptide receptor radionucleotide therapy (PRRT) with 177Lu-dotatate is widely used for the treatment of patients with neuroendocrine tumors (NETs). We analyzed data from 104 patients with NETs treated with 177Lu -dotatate at a US academic center between December 2017 and October 2020 to better understand patterns of long-term efficacy, safety, and toxicity in the real-world setting. 177Lu-dotatate (200 mCi) was administered every eight weeks for four doses. The most common sites of primary disease were small intestine NETs (n = 49, 47%), pancreatic NETs (n = 32, 31%), and lung NETs (n = 7, 7%). Twenty-seven percent had Ki-67 <3%, 49% had Ki-67 between 3-20%, and 13.5% had Ki-67 >20%. The cohort had been pretreated with a median of two prior lines of treatment. Forty percent had received prior liver-directed treatment. Seventy-four percent of patients completed all four doses of treatment. The objective response rate was 18%. The median time-to-treatment failure/death was significantly longer for small-bowel NETs when compared to pancreatic NETs (37.3 months vs. 13.2 months, p = 0.001). In a multivariate model, Ki-67, primary site, and liver tumor burden ≥50% were found to independently predict time-to-treatment failure/death. Around 40% of patients experienced adverse events of ≥grade 3 severity. Treatment-related adverse events leading to discontinuation of therapy happened in 10% of patients. Preexisting mesenteric/peritoneal disease was present in 33 patients; seven of these patients developed bowel-related toxicities including two grade 5 events. We also report two cases of delayed-onset minimal change nephrotic syndrome, which occurred 14 and 27 months after the last dose of PRRT. Lastly, we describe six patients who developed rapid tumor progression in the liver leading to terminal liver failure within 7.3 months from the start of PRRT, and identify potential risk factors associated with this occurrence, which will need further study.

Implication of acute tubular injury in minimal change nephrotic syndrome.

While acute tubular injury (ATI) is known to occur in a significant number of minimal change disease (MCD) nephrotic syndrome cases with acute kidney injury (AKI), the clinical significance is not certain, and AKI may also occur without ATI. This study aimed to evaluate whether the severity of AKI defined by Kidney Disease Improving Global Outcomes (KDIGO) criteria correlated with the presence or severity of ATI in a series of adult patients with MCD. We also looked at whether time to remission of nephrotic syndrome (NS) with treatment correlated with the presence of ATI in those with and without AKI. We excluded patients with secondary MCD. Of 61 patients, 20 had AKI (33%). ATI was significantly more likely to occur in those with AKI than in those without AKI (60 vs. 24%). Overall, the severity of AKI did not clearly correspond with the severity of ATI. Remission rates at 4 weeks were lowest (25%) in those with both AKI and ATI, while they were highest (100%) in those with neither AKI nor ATI. Patients with AKI but no ATI and those with no AKI but having ATI were intermediate in remission rates and similar to each other (60 and 62%, respectively). The time to remission in the group of those without AKI was significantly longer in those with ATI than in those without (p=0.0027), but the numerical difference in remission did not reach statistical significance in the smaller group of AKI patients. Patients with ATI were older and more often male than those without ATI. It appears that having ATI may predict a slower remission rate in MCD though the reason for this is unclear. The different demographics of those with ATI may also play a role.

Patients with infusion-related reactions on fixed-dose rituximab treatment have higher body surface area than those without infusion-related reactions in adults with frequently relapsing minimal change nephrotic syndrome: a retrospective study

BackgroundInfusion-related reactions (IRRs) are major side effects of rituximab administration. Male sex, high body weight, body surface area (BSA), and body mass index are predictive markers of rituximab-induced IRRs. However, as rituximab was not administered at a fixed dosage in a previous study, whether a higher dosage or factors associated with a larger physique are more strongly associated with rituximab-induced IRRs is unknown.Main bodyThirteen adults with frequently relapsing minimal change nephrotic syndrome (MCNS) who received an initial rituximab dose of 500 mg between September 2015 and November 2022 were retrospectively evaluated. Data on IRRs were collected from medical records. The incidence of rituximab-induced IRRs was 38.5% (5/13). The IRR group had a significantly higher BSA than the non-IRR group (median, 1.86 vs. 1.48 m2; p = 0.045). Additionally, rituximab dosage normalized by BSA in the IRR group was significantly lower than that in the non-IRR group (median, 268.8 vs. 337.9 mg/m2; p = 0.045).ConclusionsOur study revealed that adults with frequently relapsing MCNS who experienced IRRs tend to have a higher BSA, even with fixed-dose rituximab treatment. Therefore, when patients with higher BSA receive rituximab treatment, clinicians should be careful about monitoring patient condition whether the dosage is fixed or not.

Steroid-resistant minimal change nephrotic syndrome associated with thymoma treated effectively with rituximab following thymectomy and cyclosporine: a case report

BackgroundMinimal change nephrotic syndrome (MCNS) can be complicated by thymoma; however, no standard therapy for thymoma-associated MCNS has yet been established. We herein describe a case of steroid-resistant MCNS associated with thymoma, treated effectively with rituximab.Case presentationA 71-year-old Japanese man was referred to our department with severe proteinuria (20 g/gCr). Renal biopsy showed minimal change disease and computed tomography revealed an anterior mediastinal mass. Based on these findings, he was diagnosed with thymoma-associated MCNS. He was treated with oral prednisolone (50 mg/day) and cyclosporine, and underwent thymectomy and plasma exchange. However, no improvement in proteinuria was observed. He therefore received intravenous rituximab 500 mg, resulting in a marked decrease in proteinuria from 5328 to 336 mg/day after 1 week.ConclusionsThis case suggests that rituximab might be an effective therapy in patients with steroid-resistant MCNS associated with thymoma.

Bicellular Localization of Tricellular Junctional Protein Angulin-3/ILDR2 Allows Detection of Podocyte Injury

Podocytes serve as part of the renal filtration unit with slit diaphragms. Although the structure of slit diaphragms between two cells is well characterized, how the tricellular contact of podocytes is organized and how it changes in injured podocytes remains unknown. This study focused on a tricellular junction protein, angulin-3, and its localization in healthy podocytes, in developmental stages, and in pathologic conditions, using a newly established monoclonal antibody. Angulin-3 was confined at tricellular junctions of primordial podocytes, then transiently localized at bicellular junctions as foot process interdigitation developed and the intercellular junctions rearranged into slit diaphragm, and eventually distributed in a sparse punctate pattern on the foot processes of adult podocytes. In the rodent podocyte injury models, angulin-3 showed bicellular localization between the foot processes, and the localization turned from punctate to dashed linear pattern along the effaced foot processes with the progression of podocyte injury. Angulin-3 also accumulated between foot processes in a linear pattern in kidney biopsy samples of human nephrotic syndrome. Additionally, the line length of angulin-3 staining signal correlated with risk of relapse under glucocorticoid therapy in patients with minimal change nephrotic syndrome. This study proposes an image program to score the linearity of the accumulation pattern of angulin-3 to evaluate the relapse risk of patients with minimal change nephrotic syndrome.

Interleukin (IL)-27-Induced Podocyte Injury in Minimal Change Nephrotic Syndrome (MCNS)

Interleukin (IL)-27-Induced Podocyte Injury in Minimal Change Nephrotic Syndrome (MCNS)

Association of Minimal Change Nephrotic Syndrome with Mitochondrial Dysfunction in a Rat Model

Association of Minimal Change Nephrotic Syndrome with Mitochondrial Dysfunction in a Rat Model

"Idiopathic" minimal change nephrotic syndrome: a podocyte mystery nears the end.

The discovery of zinc fingers and homeoboxes (ZHX) transcriptional factors and the upregulation of hyposialylated angiopoietin-like 4 (ANGPTL4) in podocytes have been crucial in explaining the cardinal manifestations of human minimal change nephrotic syndrome (MCNS). Recently, uncovered genomic defects upstream of ZHX2 induce a ZHX2 hypomorph state that makes podocytes inherently susceptible to mild cytokine storms resulting from a common cold. In ZHX2 hypomorph podocytes, ZHX proteins are redistributed away from normal transmembrane partners like aminopeptidase A (APA) toward alternative binding partners like IL-4Rα. During disease relapse, high plasma soluble IL-4Rα (sIL-4Rα) associated with chronic atopy complements the cytokine milieu of a common cold to displace ZHX1 from podocyte transmembrane IL-4Rα toward the podocyte nucleus. Nuclear ZHX1 induces severe upregulation of ANGPTL4, resulting in incomplete sialylation of part of the ANGPTL4 protein, secretion of hyposialylated ANGPTL4, and hyposialylation-related injury in the glomerulus. This pattern of injury induces many of the classic manifestations of human minimal change disease (MCD), including massive and selective proteinuria, podocyte foot process effacement, and loss of glomerular basement membrane charge. Administration of glucocorticoids reduces ANGPTL4 upregulation, which reduces hyposialylation injury to improve the clinical phenotype. Improving sialylation of podocyte-secreted ANGPTL4 also reduces proteinuria and improves experimental MCD. Neutralizing circulating TNF-α, IL-6, or sIL-4Rα after the induction of the cytokine storm in Zhx2 hypomorph mice reduces albuminuria, suggesting potential new therapeutic targets for clinical trials to prevent MCD relapse. These studies collectively lay to rest prior suggestions of a role of single cytokines or soluble proteins in triggering MCD relapse.

Is Helminthiasis a Precipitating Factor for Frequently Relapsing Nephrotic Syndrome?

Nephrotic Syndrome (NS) is a common childhood illness. It is a disease of relapse and remission with major concern to manage the cases with relapse. Helminthiasis also a common problem in our subcontinent. So, it is very important to find out any relation with helminthiasis and relapse in children. In our study, 88 patients, clinically diagnosed as minimal change nephrotic syndrome, were studied to find out any relationship with helminthiasis and frequent relapse. 36 patients (40.9%) had no helminthic infection, 34 patients (38.6%) had helminthiasis and 18 patients (20.5%) had atopic disorders. 62 patients were successfully followed up for 6 months. Relapses occurred 47.8% (11/23) patients in non-helminthiasis group. 88.5% (23/26) in helminthiasis group and 84.6% (11/13) in atopic group, patients who completed follow up. Thirteen percent (3) patients in non-helminthiasis group, 50% (13) in helminthiasis group and 38.5% (5) in atopic group were found to be frequent relapser. The differences were statistically significant.&#x0D; Pulse Volume 12-14 2020-2022 p.10-12&#x0D;

Japanese clinical practice patterns of primary nephrotic syndrome 2021: a web-based questionnaire survey of certified nephrologists.

With the publication of the "Evidence-Based Clinical Practice Guideline for Nephrotic Syndrome 2020," we examined nephrologists' adherence to the recommendations of four of its clinical questions (CQs). This was a cross-sectional web-based survey conducted between November and December 2021. The target population comprised nephrologists certified by the Japanese Society of Nephrology who were recruited using convenience sampling. The participants answered six items regarding the four CQs about adult patients with nephrotic syndrome and their characteristics. In total, 434 respondents worked in at least 306 facilities, of whom 386 (88.9%) provided outpatient care for primary nephrotic syndrome. Of these patients, 179 (41.2%) answered that they would not measure anti- phospholipase A2 receptor antibody levels in cases of suspected primary membranous nephropathy (MN) in which kidney biopsy was not possible (CQ1). Regarding immunosuppressants as maintenance therapy after relapse of minimal change nephrotic syndrome (CQ2), cyclosporine was the most common choice (290 [72.5%] and 300 [75.0%] of 400 respondents after the first and second relapses, respectively). The most common treatment for steroid-resistant cases of primary focal segmental glomerulosclerosis (CQ3) was cyclosporine (323 of 387, 83.5%). For the initial treatment of primary MN with nephrotic-range proteinuria (CQ4), corticosteroid monotherapy was the most common choice (240 of 403, 59.6%), followed by corticosteroid and cyclosporine (114, 28.3%). Gaps in recommendations and practices regarding serodiagnosis and treatment of MN (i.e., CQ1 and 4) are observed, suggesting the need to address the barriers to their insurance reimbursement and the lack of evidence behind them.

Quality of Life in Children with Minimal Change Nephrotic Syndrome

Introduction: Nephrotic syndrome (NS) in children is a common, yet challenging, relapsing, and remitting renal disorder and exhibits a heterogeneous clinical phenotype ranging from a single episode, infrequently relapsing, frequently relapsing to steroid-resistant disease. Although 80% of these children are corticosteroid responsive, nearly half of them demonstrate a frequently relapsing or steroid-dependent course, often resulting in multiple complications, hospitalizations, or even chronic renal failure. Objective: To assess the quality of life in patients with minimal change nephrotic syndrome. Methods: This cross-sectional, comparative, questionnaire-based study was conducted in Dhaka Shishu Hospital, Dhaka, Bangladesh July to December 2022. The cases included children with MCD, attending the pediatric nephrology unit and outpatient clinic of nephrology unit. 100 children were selected then they were randomly subdivided into 2 groups, group 1 included 50 known cases of MCD aged 2–18 years and group 2 included 50 age matched children attending the general pediatric outpatient clinic and other pediatric subspecialty clinics. We distributed two questionnaires to 32 outpatients with MCNS. We also used the Self-Care Behavior Scale for patients with chronic kidney disease (CKD), which consists of 31 questions with 4 subscales. Results: Total of one hundred studied nephrotic patients with MCD and an equal number of controls with other chronic diseases aged 2.2–15 year and 3.5–13 years, respectively, were included in the study. Table 1 illustrated the demographic details of the studied cases and controls. Among children with MCD, 32% had first attack or infrequently relapsing variant while 68% had difficult to treat clinical phenotypes (frequently relapsing, steroid– dependent and steroid-resistant varieties). The SF-36v2 social functioning subscale was most impaired and bodily pain was least affected in patients with MCNS. The self-care subscales of information/communication and positive behavior had positive correlations with the QOL subscales of mental health (𝑃 &lt; 0.05) and vitality (p&lt;0.05). The correlation between social functioning and information/communication was close to significant (𝑃 = 0.051). Conclusion: In conclusion, our findings suggest that patients with MCNS have lower QOL based on low social functioning and that QOL is related to the positive behavior and thoughts of the patients. These results also show that healthcare professionals should be conscious of the QOL of children with MCNS.