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Background: The hallmark of Nephrotic syndrome is massive proteinuria, with associated enlarged kidneys. However the association between remission status and size of the kidneys in patients with nephrotic syndrome is not known. This study is aimed at determining the dimensions of the kidneys of children with nephrotic syndrome and to compare kidney dimensions in patients with nephrotic syndrome who were in remission compared with the dimensions in patients who were not in remission. Subjects and Methods: This is a case-control study, where fiftythree children with diagnosis of Nephrotic syndrome and fifty- seven age matched healthy controls were analysed. The kidneys of both cases and controls were scanned using the B- mode ultrasound and the kidneys Bipolar, anteroposterior and transverse dimensions and volume were determined. Results: The mean renal longitudinal, anteroposterior, transverse dimensions and volume were higher in cases compared to the controls, bilaterally (p

One of the urgent problems of medicine is to clarify the pathogenetic mechanisms of glomerulonephritis (GN) with refractory nephrotic syndrome (NS). In 30% of cases, refractory NS has a genetic nature. The role of human histocompatibility system (HLA) genes in the development of refractory NS has not been sufficiently studied. The purpose of this study was to study the association of two-locus haplotypes of HLA class II gene alleles with GN manifested by refractory NS. The typing of HLA class II genes in 136 patients with NS was performed by polymerase chain reaction (PCR), which included the identification of 13 alleles of the DRB1, 8 – DQA1, and 12 – DQB1 loci. The cohort of the examined patients was divided into two groups: a group of patients with refractory NS and a group of patients with rare relapses, with a lack of refractoriness to the therapy. Persons of Chuvash nationality were selected for the study. In the studied groups of patients, the values of the nonequilibrium coupling of alleles (D) were determined to identify characteristic two-locus haplotypes and their frequency according to the formulas of Piazza A. and coauthors. To assess the association of refractory NS with HLA haplotypes, relative risk values (RR) were calculated using the formula Woolf B. and Haldane J. The statistical significance of the association was assessed using the twosided Fisher exact method for four-field tables (PF). The highest value of RR was found in the haplotype HLA-DRB1*11(05)-DQA1*0301. Its value was 42.1 (PF = 0.005). Another statistically significant value was the RR value of the haplotype HLA-DRB1*15(02)-DQB1*0602-8, equal to 0.2 (PF = 0.004). As a result of the study, the haplotype DRB1*11(05)-DQA1*0301, associated with an increased risk of refractory NS, and the protective haplotype DRB1*15(02)-DQB1*0602-8, reducing the risk of refractory NS were found in the HLA genotype of individuals in the Chuvash population.

Abstract Background Hemorrhagic varicella (HV) is a particular form of chicken pox.,with high mortality in adults. This form of the disease is rare, to date, approximately 4 cases have been reported. Occasional cases of HV have been documented in adults with hematologic disorders or other diseases. While there is one reported case of simultaneous reactivation of cytomegalovirus in an adult with chickenpox, there is a lack of information regarding changes in liver function indicators for such patients. This is unfortunate, as CMV reactivation can further exacerbate liver failure and increase mortality. In this report, we present a case of hemorrhagic varicella reactivation with cytomegalovirus and provide some relevant discussions. Case presentation We present the case of a 25-year-old male with HV, who had a history of nephrotic syndrome generally controlled with orally administered prednisone at a dosage of 50 mg per day for two months. The patient arrived at the emergency room with complaints of abdominal pain and the presence of hemorrhagic vesicles on his body for the past 3 days. Despite medical evaluation, a clear diagnosis was not immediately determined. Upon admission, the leukocyte count was recorded as 20.96 × 109/L on the first day, leading to the initiation of broad-spectrum antibiotic treatment. Despite the general interpretation that a positive IgG and a negative IgM indicate a previous infection, the patient’s extraordinarily elevated IgG levels, coupled with a markedly increased CMV DNA quantification, prompted us to suspect a reactivation of the CMV virus. In light of these findings, we opted for the intravenous administration of ganciclovir as part of the treatment strategy. Unfortunately,,the patient succumbed to rapidly worsening symptoms and passed away. Within one week of the patient’s demise, chickenpox gradually developed in the medical staff who had been in contact with him. In such instances, we speculate that the patient’s diagnosis should be classified as a rare case of hemorrhagic varicella. Conclusion Swift identification and timely administration of suitable treatment for adult HV are imperative to enhance prognosis.

Abstract Background Infection is the most common complication of pediatric patients with nephrotic syndrome. The factors associated with infection in nephrotic syndrome are lacking. The objective of the study was to identify the prevalence and associated factors among children with nephrotic syndrome aged 2 to 18 years. Methods We conducted a hospital-based retrospective cross-sectional study. The data collector installed an Epi5 collector electronic data-collecting tool from Google Play. Then, we exported the data to Stata version 15.1 for analysis. The mean, standard deviation, frequency, and percentage were used for descriptive statistics. The logistic regression model was used to identify the factors associated with infection. Results In this study, the prevalence of infection among nephrotic syndrome children is 39.8% (95%CI: 30.7, 49.7). The types of infection identified were pneumonia, urinary tract infection, diarrheal disease, cutaneous fungal infection, intestinal parasitic infection, and sepsis. The presence of hematuria increased the odds of infection by 5-times. On the other hand, low level of serum albumin increased the odds of infection by 7%. Being a rural resident increased the odds of infection by 3.3-times as compared to urban. Conclusions Serum albumin level, presence of hematuria, and rural residence were significantly associated with infection. We recommended a longitudinal incidence study on large sample size at multicenter to strengthen this finding.

Abstract Background Nephrotic syndrome (NS) can occur as a paraneoplastic disorder in association with various types of carcinoma. However, paraneoplastic nephrotic syndrome (PNS) is often misdiagnosed as idiopathic nephrotic syndrome or as an adverse effect of oncology treatment, leading to delayed diagnosis and suboptimal treatment. The characteristics of NS associated with solid malignancies are not yet elucidated. We systematically summarized the clinical data for 128 cases of NS combined with solid malignancies with the aim of informing the clinical management of PNS. Methods We searched the PubMed database for articles published from the date of inception through to October 2023 using the following keywords: “cancer” or “malignant neoplasms” or “neoplasia” or “tumors” and “nephrotic syndrome”, “nephrotic” or “syndrome, nephrotic”. All data were extracted from case reports and case series, and the extraction included a method for identifying individual-level patient data. Results A literature search yielded 105 cases of PNS and 23 of NS induced by cancer therapy. The median age at diagnosis was 60 years, with a male to female ratio of 1.8:1. In patients with PNS, manifestations of NS occurred before, concomitantly with, or after diagnosis of the tumor (in 36%, 30%, and 34% of cases, respectively). Membranous nephropathy (49%) was the most prevalent renal pathology and found particularly in patients with lung, colorectal, or breast carcinoma. Regardless of whether treatment was for cancer alone or in combination with NS, the likelihood of remission was high. Conclusion The pathological type of NS may be associated with specific malignancies in patients with PNS. Prompt identification of PNS coupled with suitable therapeutic intervention has a significant impact on the outcome for patients.

Introduction: Anticoagulant use in specific clinical scenarios such as venous thromboembolic disease or atrial fibrillation is common, with clearly established management guidelines. However, there is currently no unified clinical guidance for managing thrombosis in unusual scenarios, presenting a significant therapeutic challenge. Objective: To review the indications for anticoagulation and their regimens in unusual clinical situations based on available evidence. Methods: A literature review was conducted from 1997 to 2022, using relevant MeSH terms for anticoagulation, oral anticoagulants, embolism, and thrombosis. Information was extracted and synthesized in a structured manner. Results: In nephrotic syndrome, warfarin is recommended, although alter-natives such as low molecular weight heparins, aspirin, or direct oral anti-coagulants exist. For intracavitary thrombus, warfarin is the drug of choice, with demonstrated equivalent efficacy to direct oral anticoagulants. For peripheral arterial disease, rivaroxaban at vascular protection dose + aspirin has shown benefit in major adverse cardiovascular outcomes depending on individual profile. Conclusions: Management of these scenarios requires proper clinical assessment and selection of the type of anticoagulant, ensuring maximum reduction of both thrombosis and bleeding risks.

Introduction: Rituximab (RTX) has been reported as an effective treatment alternative in primary forms of minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS) associated with steroid dependence and frequent relapses. However, the optimal RTX regimen and the outcomes of further doses of RTX remain unclear. This study aimed to evaluate the use of induction and maintenance RTX therapy for adults with primary podocytopathies. Methods: We performed a retrospective case series on adult patients with steroid-dependent podocytopathies who received an induction RTX therapy. Maintenance therapy was performed at physician’s discretion. Remission and relapse rates, concomitant corticosteroids and immunosuppressants use, B-cell depletion and adverse events were analyzed. Results: Fourteen patients (mean age at start of RTX 29.1 ± 21.9 years) with MCD (n = 7) or FSGS (n = 7) were treated with 2 doses of 1,000 mg 2 weeks apart (n = 13) or four doses of 375 mg/m2 (n = 1) of RTX. At last follow-up (mean 47.3 ± 101.7 months), 10 patients were in complete remission and two remained in partial remission. A reduction in the number of relapses, number of patients under corticosteroids and immunosuppressants, and dose of prednisolone was observed when compared to baseline (14 [100%] vs. 5 [35.7%]; 8/14 [57.1%] vs. 4/12 [33.3%]; 13/14 [92.9%] vs. 7/12 [58.3%]; 20 mg/day vs. 5.25 mg/day, respectively). Maintenance RTX therapy was used in 6 patients, with sustained complete remission. Infusion reactions were observed in 4 patients (one required treatment withdrawal). Conclusions: Our findings support the use of RTX for a steroid-free remission in podocytopathies and suggest that maintenance RTX is well-tolerated and associated with prolonged remission. Further studies are needed to confirm its efficacy and safety and establish the optimal induction and maintenance RTX regimen in steroid-dependent podocytopathies.

Background. Minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS) are two common forms of childhood nephrotic syndrome (NS). Separating MCD from FSGS is essential to determine the treatment therapy and the prognosis. Cluster of differentiation 80 (CD80) in urine is now considered a valuable marker for detecting MCD. Therefore, we conducted this research to evaluate the role of urinary CD80 concentrations in differentiating MCD from FSGS. Methods. A cross-sectional descriptive study was performed on 67 children with NS who underwent kidney biopsy and 67 children in the control group. Results. MCD accounted for the majority (61.2%), while FSGS accounted for 38.8% with an MCD/FSGS ratio of 1.6:1. There were no significant differences in age, gender, hematuria, hypertension, urinary protein-creatinine ratio, and albumin concentrations between the two study groups (p>0.05). There were statistically significant differences in glomerular filtration rate (GFR) between the two study groups with p

Amyloidosis is characterized by damage to several organ systems, which leads to diagnostic delays and progression of the pathological process. The described clinical case demonstrates a long diagnostic search in a patient with AL-amyloidosis. According to the literature, the most often described manifestation of the disease is kidney damage that manifests as nephrotic syndrome. This case is interesting because the reason for hospitalization was liver damage. Laboratory tests revealed cholestatic and cytolytic syndromes and dyslipidemia. Differential diagnostic included diseases with liver damage. In the hospital nephrotic syndrome was identifi ed, renal biopsy was performed that proved the diagnosis of AL-amyloidosis with combined damage to the gastrointestinal tract, liver and kidneys.

Abstract Background Hypercoagulability emerges as a central pathological feature and clinical complication in nephrotic syndrome. Increased platelet activation and aggregability are closely related to hypercoagulability in nephrotic syndrome. Monocyte-platelet aggregates (MPAs) have been proposed to represent a robust biomarker of platelet activation. The aim of this study was to investigate levels of the circulating MPAs and MPAs with the different monocyte subsets to evaluate the association of MPAs with hypercoagulability in nephrotic syndrome. Methods Thirty-two patients with nephrotic syndrome were enrolled. In addition, thirty-two healthy age and sex matched adult volunteers served as healthy controls. MPAs were identified by CD14 monocytes positive for CD41a platelets. The classical (CD14 + + CD16-, CM), the intermediate (CD14 + + CD16+, IM) and the non-classical (CD14 + CD16++, NCM) monocytes, as well as subset specific MPAs, were measured by flow cytometry. Results Patients with nephrotic syndrome showed a higher percentage of circulating MPAs as compared with healthy controls (p

Abstract Background Nephrotic syndrome (NS) is a disease with favorable outcomes in most cases. Failure to respond to steroids is one of the most important predictors of clinical outcome. We performed a retrospective study on 51 primary idiopathic SRNS cases presenting between January 2011 and June 2021 at Alexandria University Children’s Hospital (AUCH) by retrieving data from their clinic files. Cases with secondary causes of NS or suspected genetic causes were excluded from our study. Patients’ demographic data, clinical and laboratory findings at the time of presentation, complications occurring during the course of treatment, immunosuppressants used along with steroids, and outcomes at the last follow-up were recorded. The mean duration of follow-up was 5.67 ± 3.07 years. Results Our results showed that there was male predominance (2.9:1), the mean age at first presentation was 4.53 ± 3.03 years, and persistent hematuria and systemic hypertension were found in 41.6% and 57.1%, respectively. The most common biopsy finding was MesPGN (37.3%). The most utilized immunosuppressant was cyclosporine (80.4%). Complete remission was fortunately the most prevalent outcome among our cases (52.2%). Conclusions Our study concluded that biopsy findings and thepresence of hematuria in SRNS cases are the most crucial factors in determining the final outcome. MCNS finding in biopsy is significantly correlated with complete remission (p value = 0.043). Persistent hematuria whether gross or microscopic was significantly correlated with unremitting disease (p value = 0.017).

Introduction. Stroke in young individuals is becoming increasingly prevalent worldwide. Its causes can vary widely, so a thorough investigation by a multidisciplinary team is needed. Pinpointing the precise underlying pathology responsible for the stroke yields benefits for patients, particularly in recurrent events. Case presentation. A 38-year-old man presented to the emergency department with symptoms suggestive of stroke, including right hemiparesis, dysarthria, ataxic gait, and right central facial palsy. The brain magnetic resonance image revealed an ischemic lesion located in the left basal ganglia and near the corona radiata. Following an extensive workup, a diagnosis of nephrotic was reached. Histopathology and the exclusion of secondary causes confirmed primary membranous nephropathy as the underlying condition. The patient underwent treatment tailored to address the specific glomerulopathy, along with anticoagulation therapy and immunosuppression as per current guidelines. Subsequent assessments showed stabilization of renal function, resolution of the edema, and the absence of new thromboembolic events during follow-up. Conclusion. The nephrotic syndrome should be recognized as a potential underlying cause of stroke in young patients and, therefore, it should be included in the differential diagnosis during the evaluation of patients with coagulopathies. Nephrotic syndrome screening may be done by conducting a simple urinalysis readily available in most healthcare facilities. This underlines the importance of considering renal pathology in the assessment of stroke etiologies, especially when coagulation abnormalities are present.

Background: Focal segmental glomerulosclerosis (FSGS) is a prevalent glomerular disease that often leads to nephrotic syndrome. It is characterized by consolidating a portion of the glomerular capillary tuft connected to Bowman's capsule. This retrospective cohort study aimed to determine the demographic characteristics, risk factors, and prognostic indicators associated with FSGS in Shiraz, Iran. Methods: The study included 53 primary FSGS patients aged over 18 years who were referred to clinics affiliated with Shiraz University of Medical Sciences. Data were collected through a comprehensive data-gathering sheet encompassing demographic information, medical history, laboratory test results, and histopathological findings. Statistical analysis was performed using SPSS 18, considering a significance level of p

The aim of this study was to observe the remission of primary membranous nephropathy (PMN) and evaluate the efficacy of tacrolimus (TAC) monotherapy for PMN in comparison with TAC combined with a low-dose glucocorticoid (GC) protocol (TAC + GC).

Introduction: Nephrotic syndrome may persist despite end-stage kidney disease and result in dyslipidaemia, thrombosis and a significantly increased cardiovascular risk. Treatment of refractory nephrotic syndrome includes surgical bilateral nephrectomy, renal artery embolization and pharmacologic nephrectomy. Case Presentation: We present a case of a haemodialysis patient with refractory nephrotic syndrome who underwent pharmacologic nephrectomy. The procedure decreased the patient‘s cardiovascular risk and enabled the patient to become a candidate for kidney transplantation. Conclusion: In certain situations residual renal function may be harmful. In such instances, nephrectomy should be considered. Pharmacologic nephrectomy using nephrotoxic drugs is a non-invasive approach with least potential complications.

Abstract Background Adult nephrotic syndrome is a well-known kidney disease that causes heavy proteinuria, hypoalbuminemia, hypercholesterolemia, edema, and hypertension. The treatment varies according to its underlying cause but often faces medication resistance or adverse drug effects. Case presentation A Japanese woman in her 80s presented with nephrotic syndrome after a 3 year latent period of urinary protein and occult blood. She did not have any secondary causes of nephrotic syndrome. Renal biopsy revealed thin glomerular basement membrane, partial foot process fusion on electron microscopy with minor glomerular change on light microscopy, and slight coarse immunoglobulin M deposition in the mesangium on immunofluorescence microscopy, which was inconsistent with any other glomerular diseases. Without steroid treatment, she dramatically remitted from proteinuria after the administration of the renal protective agents enalapril, ezetimibe, rosuvastatin, and dapagliflozin. Recurrence after 8 months of follow-up subsided with the administration of additional doses of the agents. Conclusions This case illustrated the novel outcomes of combining medical treatment without steroid use for nephrotic syndrome with thin glomerular basement membrane disease. At the time of writing this report, the patient’s renal function was stable and she was free of edema, although moderate proteinuria and occult hematuria persisted. The final diagnosis was uncertain because of the lack of genetic investigation; however, the response to the aforementioned medical treatment suggests the effectiveness of the supportive therapy.

Background: Acute kidney injury (AKI) is a common comorbidity in critically ill children and is associated with an increased risk of morbidity and mortality in children with nephrotic syndrome. The etiology of AKI in nephrotic syndrome is complex and multifactorial. Aims and Objectives: To determine the proportion, risk factors, and outcomes of patients with AKI in children hospitalized with nephrotic syndrome. Materials and Methods: This cross-sectional study was done on children between 1 month and 14 years of age attending the pediatric department for 1 year from October 2020 to June 15, 2022. Detailed history, physical examination, and investigations were done in children with nephrotic syndrome as per pro forma. Information was collected on demographic variables, vital signs, anthropometry, diagnosis, comorbidities, therapies, presence of hypovolemia, sepsis or shock, use of diuretics or nephrotoxic medications, need for oxygen, mechanical ventilation, vasopressors or renal replacement therapy, length of hospital stay, and outcome at discharge. For those children having AKI, clinical outcomes were measured in terms of duration of hospital stay, recovery of S.creatinine, and urine output. Results: A total of 64 patients with nephrotic syndrome were enrolled in this study. Fifteen patients had AKI (23.4%) among children with nephrotic syndrome. The most common etiology being sepsis accounting for 4 out of 15 (26.7%) of total cases followed by acute respiratory distress syndrome (13.3%), nephrotoxic medications (13.3%), renal parenchymal disease (13.3%), Post-streptococcal glomerulonephritis (13.3% each) and Sickle cell disease with nephritis and acute fulminant hepatitis (6.7%). There was significantly more mortality among subjects with stage 2 (14.3%) and 3 (14.3%) kidney disease. Conclusion: AKI is common in our children with nephrotic syndrome and the main risk factors of AKI include sepsis, gross hematuria, urinary tract infections, peritonitis, and exposure to potentially nephrotoxic medications. There is a need to identify the patients with nephrotic syndrome at risk of AKI and apply strategies to prevent AKI in focused at-risk groups.

Objective: There is limited evidence on which immunosuppressive agents produce the best outcomes for adult patients with steroid-dependent or frequently relapsing nephrotic syndrome (SDNS/FRNS). This review compares the remission rate and adverse effects of various immunosuppressants used. Methods: Studies of adult patients with biopsy-proven SDNS/FRNS, administered any immunosuppressive agents and reported complete remission results as one of the clinical outcomes were included. Articles were independently screened by two researchers. ROBINS-I was used for risk of bias assessment. Random-effects model was used for statistical analysis and corresponding 95% confidence intervals (CIs) were calculated. Results: 574 patients across 28 studies were included in the analysis. Patients receiving rituximab have a complete remission rate of 89% (95% CI = 83% to 94%; τ2 = 0.0070; I2 = 62%; overall p < 0.01, low certainty) and adverse event rate of 0.26, cyclosporine (CR 40%; 95% CI = 21% to 59%; τ2 = 0.0205; I2 = 55%; overall p = 0.08, low certainty), tacrolimus (CR 84%; 95% CI = 70% to 98%; τ2 = 0.0060; I2 = 33%; overall p = 0.21, moderate certainty), mycophenolate mofetil (CR 82%; 95% CI = 74% to 90%; τ2 < 0.0001; I2 = 15%; overall p = 0.32, moderate certainty) and cyclophosphamide (CR 79%; 95% CI = 69% to 89%; τ2 = 0; I2 = 0%; overall p = 0.52, moderate certainty). Conclusion: Among the commonly used immunosuppressive agents, only rituximab has a statistically significant effect in achieving complete remission among patients with SDNS/FRNS and has a relatively good safety profile, but this is limited by low quality of evidence with high degree of heterogeneity causing a lack of statistical power. [ABSTRACT FROM AUTHOR]

The differences between the serum albumin determined by bromocresol green (BCG) and immunonephelometry (IN) were inconsistent in past studies, and the samples were all adults. We sought to determine the differences in children and reveal the impacts of these differences on the clinical diagnosis and treatments of primary nephrotic syndrome (PNS). Repeated measurements from 576 PNS children showed that albumin measured by BCG and IN (ALB-B and ALB-I) were 19.95 (11.15) g/L and 15.30 (11.05) g/L, respectively, and the mean difference was 4.68 g/L (P < 0.001). The cut-offs we calculated for hypoalbuminemia and severe hypoalbuminemia based on the IN were 25 and 15 g/L, which were 5 g/L lower than the cut-offs recommended by KIDGO, respectively. A pair of historical control samples (206 vs. 216) with ALB-B or ALB-I showed that the proportion of severe hypoalbuminemia was 14.60% greater in IN group (75.20% vs. 60.60%, P < 0.001). The misdiagnosis rate of severe hypoalbuminemia by IN was 33.77% when 20 g/L rather than 15 g/L was used as the cut-off. Furthermore, the proportion of patients receiving albumin injections increased by 10.20%, and the average consumption increased by 97.06% (P = 0.01) along with the use of IN. So, our results suggested that the difference between ALB-B and ALB-I led to misdiagnosis and prescription abuse in PNS children. [ABSTRACT FROM AUTHOR]

Introduction: Kimura’s disease (KD) is a rare chronic inflammatory disorder characterized by subcutaneous lymphoid hyperplasia with peripheral eosinophilia. Kidney involvement is reported in 15%–18% of adult patients with KD, in many cases as nephrotic syndrome. We present a case of overlapping membranous nephropathy and IgA nephropathy associated with KD. Case report: A 27-year-old man was admitted with a history of bilateral leg edema for the last 2 months and concomitant progressive increase of cervical mass and fever. Laboratory findings were as follows: peripheral leukocyte count, 10,080/mm³; eosinophils, 3,200/mm³ (31.7%); serum creatinine, 0.83 mg/dL; and eGFR: 140 mL/min per 1.73 m². Urinalysis revealed the presence of hematuria and proteinuria and the following results: 24-h proteinuria, 12.9 g; serum albumin, 1.3 g/dL; and elevated IgE level, 750 kU/L. Serologies for hepatitis B, hepatitis C, HIV, and VDRL were all negative. Complement C3 and C4 levels were normal. No monoclonal protein was detected in blood and urine. Parasite infestation was discarded. A biopsy of the cervical lymph node revealed eosinophilic lymphoid hyperplasia, suggesting KD. A kidney biopsy revealed findings consistent with the overlapping of membranous nephropathy with IgA nephropathy. The patient was treated for KD with prednisone 1 mg/kg/d with progressive dose tapering and posterior association of methotrexate 15 mg/week. A renin–angiotensin system inhibitor was prescribed for nephrotic syndrome. The cervical mass regressed, and proteinuria achieved partial remission, with an increase in serum albumin level and normalization of eosinophils and IgE levels. Conclusion: Although uncommon, kidney involvement must be considered in patients with KD. Glomerular diseases are the most frequent form of kidney injury. [ABSTRACT FROM AUTHOR]

Background: Adolescents with Nephrotic Syndrome (NS) confront unique challenges that influence their Meaning of Life (MOL), a concept crucial for psychological resilience. The chronic nature of NS and its associated burdens necessitate a deeper exploration of MOL and its determinants within this demographic, previously underexamined in research. This study aims to investigate MOL among Chinese adolescents with NS, identifying key factors influencing their sense of meaning and examining the interrelations with Subjective Well-Being (SWB) and Psychological Security (PS). Methods: Employing a cross-sectional survey design, we analyzed 150 adolescents with NS from Baise City, Guangxi, using the Purpose in Life (PIL) scale alongside PS and SWB scales. Sociodemographic and disease-related variables were assessed for their impact on MOL. Data analysis included descriptive statistics, multiple linear regression, and correlation analyses to explore predictors of MOL and its association with SWB and PS. Results: A significant proportion (62.0%) of participants exhibited MOL scores below the threshold, indicating diminished life meaning. Critical factors impacting MOL included ‘left-behind’ status, family structure, educational disruptions, and NS duration. Strong correlations emerged between MOL, SWB (r  =  0.70, p  <  0.01), and PS, highlighting the interdependence of these psychological dimensions. The study further revealed ‘Proactivity’ as a vital component of MOL, suggesting that despite their challenges, adolescents with NS maintain a keen engagement with life. Key aspects such as ‘Certainty in Control’, ‘Mood of Melancholy or Pleasure’, and ‘Vitality’ emerged as crucial for intervention. Conclusion: The findings underline the profound impact of NS on adolescents’ MOL, influenced by both sociodemographic and disease-specific factors. By identifying key areas for psychological intervention, this study contributes to the holistic care and treatment of adolescents with NS, advocating for integrated approaches that address their unique challenges and support systems. [ABSTRACT FROM AUTHOR]

Background: Nephrotic syndrome (NS) is a common kidney disease in children. While Steroid-Sensitive Nephrotic Syndrome (SSNS) is frequently observed, Steroid-Resistant Nephrotic Syndrome (SRNS) has a poor prognosis and often leads to chronic kidney disease. The pathogenesis of SRNS is complex, with immunological modulation of T helper subtypes 1 and 2 cytokines increasing susceptibility to the disease. Currently, no established biomarkers can accurately predict SRNS. However, a group of cytokines might serve as potential indicators of responsiveness, aiding in the identification of patients with SRNS. The discovery of these cytokines as novel biomarkers for early diagnosis could greatly benefit patients. This includes preventing the adverse effects of glucocorticoid treatment and enabling a timely transition to more effective therapeutic alternatives. Methods: This study aims to investigate the association between the gene expression patterns of cytokines, including IL-2, IL-4, IL-5, IL-6, IL-9, IL-10, IL-13, IL-17A, NF-κB, and TNFα, in healthy participants (n = 100), SSNS patients (n = 100), and SRNS patients (n = 100). Using qRT-PCR, followed by Receiver-operating characteristic analysis, the study assesses their potential as biomarkers. Additionally, clinicodemographic data were analyzed, and bioinformatic analyses such as coexpression analysis, gene enrichment, pathway analysis, and Cytoscape were performed to enhance our understanding of the inflammatory cascade initiating podocyte injury in NS. Results: The results of our study suggest that specific candidate genes, including IL-2, IL-5, IL-6, IL-9, IL-17A, IL-10, IL-13, and TNFα, exhibit upregulation and hold significant importance, with an Area Under the Curve value of 0.9. Conclusion: These genes have the potential to serve as valuable prognostic and management tools for NS, forming a promising panel of inflammatory gene biomarkers. Furthermore, conducting an extensive analysis that integrates cytokine genes with their respective targeted microRNAs could offer deeper insights into the pathogenesis of the disease. [ABSTRACT FROM AUTHOR]

Background: Membranous nephropathy (MN) is a common type of nephrotic syndrome (NS) in adults, accounting for about 20–30% of cases. Although secondary to specific factors, the coexistence of MN and mantle cell lymphoma (MCL) has been scarcely reported in clinical literature. Case presentation: A 59-year-old Chinese male was admitted to the hospital with a generalized pruritic rash with bilateral lower extremity edema, which did not improve significantly after symptomatic treatment. He had undergone renal biopsy, and the diagnosis was thought to be secondary MN (SMN), therefore, we did a lymph node biopsy on the patient and found that MN was complicated with MCL. Soon after, the patient was admitted to the hematology department for a BR chemotherapy regimen (composed of bendamustine 90 mg/m2 BSA (body surface area), rituximab 375 mg/m2 BSA and dexamethasone 5 mg), and during the post-treatment follow-up, both his symptoms and renal function improved. Conclusions: The mechanism underlying the combination of SMN and MCL remains elusive and exceedingly rare, consequently often overlooked in clinical practice. This case serves to offer valuable clinical insights for diagnosis and treatment, while emphasizing the pivotal role of renal pathology in clinical assessment. [ABSTRACT FROM AUTHOR]

Context: Nephrotic syndrome(NS) has emerged as a worldwide public health problem. Renal fibrosis is the most common pathological change from NS to end-stage renal failure, seriously affecting the prognosis of renal disease. Although tremendous efforts have been made to treat NS, specific drug therapies to delay the progression of NS toward end-stage renal failure are limited. Epimedium is generally used to treat kidney disease in traditional Chinese medicine. Icariin is a principal active component of Epimedium. Methods: We used Sprague Dawley rats to establish NS models by injecting doxorubicin through the tail vein. Then icariin and prednisone were intragastric administration. Renal function was examined by an automatic biochemical analyzer. Pathology of the kidney was detected by Hematoxylin-Eosin and Masson staining respectively. Furthermore, RT-PCR, Enzyme-Linked Immunosorbent Assay, Immunohistochemistry, Western Blot and Terminal-deoxynucleotidyl Transferase Mediated Nick End Labeling staining were employed to detect the proteins related to pyroptosis and EMT. HK-2 cells exposed to doxorubicin were treated with icariin, and cell viability was assessed using the MTT. EMT was assessed using Enzyme-Linked Immunosorbent Assay and Western Blot. Results: The study showed that icariin significantly improved renal function and renal fibrosis in rats. In addition, icariin effectively decreased NOD-like receptor thermal protein domain associated protein 3,Caspase-1, Gasdermin D, Ly6C, and interleukin (IL)-1β. Notably, treatment with icariin also inhibited the levels of TGF-β, α-SMA and E-cadherin. Discussion and conclusions: It is confirmed that icariin can improve renal function and alleviate renal fibrosis by inhibiting pyroptosis and the mechanism may be related to epithelial-to-mesenchymal transition. Icariin treatment might be recommended as a new approach for NS. [ABSTRACT FROM AUTHOR]

Background: Infection is the most common complication of pediatric patients with nephrotic syndrome. The factors associated with infection in nephrotic syndrome are lacking. The objective of the study was to identify the prevalence and associated factors among children with nephrotic syndrome aged 2 to 18 years. Methods: We conducted a hospital-based retrospective cross-sectional study. The data collector installed an Epi5 collector electronic data-collecting tool from Google Play. Then, we exported the data to Stata version 15.1 for analysis. The mean, standard deviation, frequency, and percentage were used for descriptive statistics. The logistic regression model was used to identify the factors associated with infection. Results: In this study, the prevalence of infection among nephrotic syndrome children is 39.8% (95%CI: 30.7, 49.7). The types of infection identified were pneumonia, urinary tract infection, diarrheal disease, cutaneous fungal infection, intestinal parasitic infection, and sepsis. The presence of hematuria increased the odds of infection by 5-times. On the other hand, low level of serum albumin increased the odds of infection by 7%. Being a rural resident increased the odds of infection by 3.3-times as compared to urban. Conclusions: Serum albumin level, presence of hematuria, and rural residence were significantly associated with infection. We recommended a longitudinal incidence study on large sample size at multicenter to strengthen this finding. [ABSTRACT FROM AUTHOR]

Background: Hemorrhagic varicella (HV) is a particular form of chicken pox.,with high mortality in adults. This form of the disease is rare, to date, approximately 4 cases have been reported. Occasional cases of HV have been documented in adults with hematologic disorders or other diseases. While there is one reported case of simultaneous reactivation of cytomegalovirus in an adult with chickenpox, there is a lack of information regarding changes in liver function indicators for such patients. This is unfortunate, as CMV reactivation can further exacerbate liver failure and increase mortality. In this report, we present a case of hemorrhagic varicella reactivation with cytomegalovirus and provide some relevant discussions. Case presentation: We present the case of a 25-year-old male with HV, who had a history of nephrotic syndrome generally controlled with orally administered prednisone at a dosage of 50 mg per day for two months. The patient arrived at the emergency room with complaints of abdominal pain and the presence of hemorrhagic vesicles on his body for the past 3 days. Despite medical evaluation, a clear diagnosis was not immediately determined. Upon admission, the leukocyte count was recorded as 20.96 × 109/L on the first day, leading to the initiation of broad-spectrum antibiotic treatment. Despite the general interpretation that a positive IgG and a negative IgM indicate a previous infection, the patient's extraordinarily elevated IgG levels, coupled with a markedly increased CMV DNA quantification, prompted us to suspect a reactivation of the CMV virus. In light of these findings, we opted for the intravenous administration of ganciclovir as part of the treatment strategy. Unfortunately,,the patient succumbed to rapidly worsening symptoms and passed away. Within one week of the patient's demise, chickenpox gradually developed in the medical staff who had been in contact with him. In such instances, we speculate that the patient's diagnosis should be classified as a rare case of hemorrhagic varicella. Conclusion: Swift identification and timely administration of suitable treatment for adult HV are imperative to enhance prognosis. [ABSTRACT FROM AUTHOR]

Background: Nephrotic syndrome is characterized by the presence of proteinuria, more than 3.5 g per 24 h, hypoalbuminemia, edema and hyperlipidaemia. The spectrum of diseases causing nephrotic syndrome has changed globally over the last few decades. The current study aimed to investigate the clinicopathological characteristics of adults presenting with nephrotic syndrome. Methods: This study included patients aged 18 to >60 years who were diagnosed with nephrotic syndrome. All patients underwent renal biopsies and were examined using electron microscopy (EM), immunofluorescence (IF), and light microscopy.Results: Our study included 39 patients diagnosed with nephrotic syndrome who received treatment at hospitals affiliated with Bangalore Medical College and Research Institute. Among these patients, 61.5% were men, and 38.5% were women. The majority of cases (53.8%) occurred during the third decade of life. The most common initial symptom observed in these patients was pedal oedema. Systemic hypertension, hypercholesterolaemia, and hypothyroidism were found in 76.9%, 87.2%, and 69.2% of patients, respectively. Elevated serum creatinine levels were detected in 25.6% of patients, predominantly in those with focal segmental glomerulosclerosis (FSGS) and IgA nephropathy. The 24-hour urine protein levels ranged from to 3.6-12.3g/day, with an average of 6.52g/day. The most prevalent histological variant is membranous glomerulonephritis (MGN), followed by IgA nephropathy and mesangiocapillary glomerulonephritis (MCGN). Conclusion: Membranous glomerulonephritis is the predominant aetiology of nephrotic syndrome in adults. Nephrotic syndrome typically manifests during the third decade of life and has a higher incidence in males. Most patients exhibit comorbidities such as hypertension, hypothyroidism, and hyperlipidaemia. [ABSTRACT FROM AUTHOR]

Background: Nephrotic syndrome is usually accompanied by retention of water and sodium. Nephrotic syndrome is a collection of clinical findings due to kidney damage. This includes protein in the urine, low blood albumin levels, high blood lipids, and significant edema. Objectives: This study aimed to observe the pattern of lipid profile in the children with idiopathic nephrotic syndrome and the effect of treatment on the same. Methods: The present observational, cross sectional of 40 cases of nephrotic syndrome children aged between 2-8 years was carried out from June 2022 to July 2023 in Department of Pediatrics of Medical College & Hospital, Kolkata, West Bengal, India. Statistical data were analysed by using Microsoft Excel and SPSS V.20 software. Results: Number of nephrotic children presented during first episode (FE) was 10 (25%). Majority (65%) were under the category of infrequent relapser (I:R) and only 4 children (10%) were frequent relapse. It was observed that during relapse, 4 cases (10%) having their serum albumin level < 1.5 gm/dl showed a very high mean serum cholesterol value of 596 mg/dl. Among 40 nephrotic children during their relapse before treatment, majority of cases (60%) showed their serum LDL cholesterol value in between 200 - 300 mg /dl. Only few cases (8%) showed very high LDL cholesterol level above 401 mg /dl. The mean + SD value (mg / dl of LDL-cholesterol among control children was 94.8 +30.39 Conclusions: This study suggests that serum lipid profile evaluation be done at regular intervals in children with nephrotic syndrome with follow up even during remission. [ABSTRACT FROM AUTHOR]

While there remains no universally accepted definition for resistant edema, it is generally acknowledged as edema that fails to respond to maximally administered doses of diuretics. Nephrotic edema is characterized by high levels of proteinuria, notably urinary concentrations of serine proteases, which possess the ability to activate the epithelial sodium channel (ENaC), resulting in persistent fluid retention. Loop diuretics are typically preferred as first-line therapy for hypervolemia. However, the sustained activation of ENaC channels may lead to resistance to treatment. Consequently, the blockade of ENaC represents a potential therapeutic approach. We report the case of a 36-year-old man presenting with nephrotic edema managed with a combination of furosemide and human albumin. Despite an initially favorable response, the patient subsequently developed oliguria and progressive weight gain. On the third day, a regimen comprising amiloride (5 mg/day) and hydrochlorothiazide (50 mg/day) was initiated, resulting in complete resolution of edema after 11 days. No hyperkalemia was observed, with only a slight elevation in serum creatinine levels. Although clinical trials are limited, mounting evidence from human and animal studies supports the concept of nephrotic syndrome as an overfill phenomenon and underscores the potential benefits of ENaC blockers in the management of nephrotic edema. [ABSTRACT FROM AUTHOR]

Background: Children with nephrotic syndrome experience many side effects and frequent relapses when treated with steroids and other drugs. Mycophenolic acid (MPA) is one of the effective and least toxic drug for the treatment of nephrotic syndrome. This drug needs to be monitored for maximal efficacy and minimal toxicity. The therapeutic reference range for this drug is not established for the aforementioned patient population of Indian origin. Materials and Methods: In this observational study, children with nephrotic syndrome on mycophenolate mofetil were followed up for a minimum duration of three months. Following this, their clinical status (relapse/remission) was determined and the mycophenolate exposure was measured for over 12 hours. Results: A total of 34 participants were included, with 17 (50%) in relapse. Median MPA Area under the curve over 12 hours (AUC0--12h) (36.5 µg·h/ml) in the remission group differed significantly compared to that in the relapse group (17.2 µg·h/ml). Conclusion: Higher exposure to MPA AUC0--12h is associated with clinical remission of pediatric nephrotic syndrome. [ABSTRACT FROM AUTHOR]

Introduction. In recent years, the incidence of pediatric nephrotic syndrome (NS) has been increasing, and timely and effective treatment is critical to protect the health of children with NS. This study is an attempt to compare the therapeutic effects of prednisone (Pred) plus dipyridamole (DIP) versus Pred plus valsartan (VAL) on pediatric NS. Methods. Two hundred pediatric cases of NS were selected as the research participants, including 109 cases (group A) receiving Pred + DIP and 91 cases (group B) receiving Pred + VAL. The clinical efficacy, adverse reactions, and renal, coagulation functions and blood lipid levels, as well as the pre- and post-treatment levels of inflammatory factors (IFs) and immunoglobulins (Igs) were comparatively analyzed. Results. No statistically significant differences were found between groups in terms of clinical efficacy, incidence of adverse reactions and renal function (P > .05). After receiving the corresponding treatment, group A showed better coagulation and immune functions than group B, but higher levels of IFs and poorer blood lipid function (P < .05). Conclusion. Both Pred + DIP and Pred + VAL combination therapies can be used for the treatment of pediatric NS, with the former contributing to more obviously enhanced coagulation and immune functions, and the latter leading to more significantly inhibited inflammation and better regulated blood lipid function. [ABSTRACT FROM AUTHOR]

Aims: Prednisolone is the cornerstone of treatment for idiopathic nephrotic syndrome in children, but is associated with marked side‐effects. Therapeutic drug monitoring using saliva would be a patient‐friendly option to monitor prednisolone treatment. To assess the feasibility of saliva monitoring, we described the pharmacokinetics (PK) of unbound prednisolone in plasma and saliva of children with first onset steroid‐sensitive nephrotic syndrome (SSNS). Methods: Children (age 2–16 years) with SSNS participating in a randomized, placebo‐controlled trial with levamisole were treated with an 18‐week tapering schedule of prednisolone. Five serial samples were collected at 4 (saliva) and 8 weeks (saliva and plasma) after first onset. A nonlinear mixed‐effects model was used to estimate the PK parameters of unbound prednisolone and the saliva‐to‐plasma ratio. Monte Carlo simulations were performed to assess the predictive performance of saliva monitoring. Results: From 39 children, 109 plasma and 275 saliva samples were available. Estimates (relative squared error) of unbound plasma clearance and volume of distribution were 93 (5%) L h−1 70 kg−1 and 158 (7%) L 70 kg−1, respectively. Typical saliva‐to‐plasma ratio was 1.30 (8%). Monte Carlo simulations demonstrated that on basis of 4 saliva samples and a single plasma sample unbound plasma area‐under‐the‐concentration–time curve can be predicted within 20% imprecision in 79% of the patients compared to 87% based on 4 plasma samples. Conclusion: Saliva proved to be a reliable and patient‐friendly option to determine prednisolone plasma exposure in children with SSNS. This opens opportunities for further PK and pharmacodynamics studies of prednisolone in a variety of paediatric conditions. [ABSTRACT FROM AUTHOR]

Since the significance of viral infections in children and adolescents with nephrotic syndrome (NS) is yet to be defined, this study intended to estimate the occurrence, pattern, and outcomes of some DNA viral infections in children with NS. Methods: A prospective study was conducted to determine the genome identification of the viruses Epstein-Barr (EBV), human cytomegalovirus (HCMV), human herpesvirus 6 (HHV-6 type A and type B) and 7 (HHV-7), polyomavirus (BKV), and human adenovirus (HAdV) in plasma and urine samples of pediatric patients with NS. Results: A total of 35 patients aged 1 to 18 years with NS and under immunosuppressant drugs participated in the study. Plasma and urine samples were collected at regular intervals during a median follow-up of 266 days (range 133–595), and DNA was analyzed to detect the selected DNA viruses. Eleven patients (31.4%) had active virus infections, and patterns were classified as coinfection, recurrent, and consecutive. Of these, six patients (54.5%) presented viral coinfection, six (54.5%) viral recurrence, and seven patients (63.3%) had viral consecutive infection. Ten of the eleven patients with active infection had a proteinuria relapse (91%) and eight (72.7%) were hospitalized (p = 0.0022). Active HCMV infection was the most frequent infection and was observed in six patients (54.5%), three of the eleven patients (27.2%) had suspected HCMV disease in the gastrointestinal tract, and one had HHV-7 coinfection. The frequency of other infections was: 9% for HHV-6, 45.5% for BKV, 27.3% for HHV-7, 18.2% for EBV, and 18.2% for HAdV. Conclusion: viral infections, especially HCMV, can be an important cause of morbidity and nephrotic syndrome relapse in children. [ABSTRACT FROM AUTHOR]

Objective To establish and validate a model for predicting preeclampsia with nephrotic syndrome, providing reference for obstetricians to identify and diagnose preeclampsia with nephrotic syndrome early. Methods A retrospective analysis was conducted on 60 cases of pre-eclampsia with nephrotic syndrome hospitalized for delivery from January 2012 to January 2021 at the First Affiliated Hospital of Guangxi Medical University and Wuming Hospital affiliated to Guangxi Medical University. Additionally, 202 cases of non preeclampsia with nephrotic syndrome patients during the same period were selected as the control group. The cases were randomly divided into a training set and a validation set at a ratio of 7:3. Logistic univariate and multivariate analyses were performed on the training set according to whether preeclampsia with nephrotic syndrome was clinically diagnosed to establish a prediction model. The discriminative ability and calibration of the model were tested, and internal validation was conducted using bootstrap and validation set data. The model was visualized using a column line chart. Results Logistic univariate and multivariate analysis ultimately retained 4 variables: early onset type, primiparity, total protein, and fibrinogen. A diagnostic prediction model was established and presented using a column line chart. The internal validation area under the ROC curve was 0. 951 7, indicating good generalization of the model. The HL test statistic for internal validation was 5 376. 1, with P <0.001. Conclusion The constructed prediction model can serve as a reference for clinical physicians to diagnose preeclampsia with nephrotic syndrome. [ABSTRACT FROM AUTHOR]

Extracellular vesicles (EVs) are membranous cargo particles that mediate intercellular communication. They are heterogeneous in size and mechanism of release, and found in all biological fluids. Since EV content is in relation to the originating cell type and to its physiopathological conditions, EVs are under study to understand organ physiology and pathology. In addition, EV surface cargo, or corona, can be influenced by the microenvironment, leading to the concept that EV-associated molecules can represent useful biomarkers for diseases. Recent studies also focus on the use of natural, engineered, or synthetic EVs for therapeutic purposes. This review highlights the role of EVs in kidney development, pediatric kidney diseases, including inherited disorders, and kidney transplantation. Although few studies exist, they have promising results and may guide researchers in this field. Main limitations, including the influence of age on EV analyses, are also discussed. [ABSTRACT FROM AUTHOR]

Background: In the current study, longitudinal BP and lipid measurements were examined in a NEPTUNE cohort of children with newly diagnosed nephrotic syndrome (cNEPTUNE). We hypothesized that hypertensive BP and dyslipidemia would persist in children with nephrotic syndrome, regardless of steroid treatment response. Methods: A multi-center longitudinal observational analysis of data obtained from children < 19 years of age with new onset nephrotic syndrome enrolled in the Nephrotic Syndrome Study Network (cNEPTUNE) was conducted. BP and lipid data were examined over time stratified by disease activity and steroid exposure. Generalized estimating equation regressions were used to find determinants of hypertensive BP and dyslipidemia. Results: Among 122 children, the prevalence of hypertensive BP at any visit ranged from 17.4% to 57.4%, while dyslipidemia prevalence ranged from 40.0% to 96.2% over a median of 30 months of follow-up. Hypertensive BP was found in 46.2% (116/251) of study visits during active disease compared with 31.0% (84/271) of visits while in remission. Dyslipidemia was present in 88.2% (120/136) of study visits during active disease and in 66.0% (101/153) while in remission. Neither dyslipidemia nor hypertensive BP were significantly different with/without medication exposure (steroids and/or CNI). In regression analysis, male sex and urine protein:creatinine ratio (UPC) were significant determinants of hypertensive BP over time, while eGFR was found to be a determinant of dyslipidemia over time. Conclusions: Results demonstrate persistent hypertensive BPs and unfavorable lipid profiles in the cNEPTUNE cohort regardless of remission status or concurrent steroid or calcineurin inhibitor treatment. [ABSTRACT FROM AUTHOR]

Background: Eighty-five percent of infants with congenital nephrotic syndrome (CNS) and 66% with infantile NS (INS) are likely to have a monogenic etiology. There exists a significant genetic variability between different regions and ethnic groups. This study aimed to determine the genetic defects in children with CNS and INS by establishing a registry in western India. Methods: In this cross-sectional study, pediatric nephrologists from 13 private and government institutions shared relevant clinical data and details of the genetic evaluation of children presenting with NS within the first year of life. Results: The median age at presentation was 9 months (range 1–23, IQR 3–13 months), history of consanguinity between parents existed in 14 patients (34%), family history of similar illness in 6 (15%), and extra-renal manifestations in 17 (41%). Twenty-five (61%) were confirmed to have a monogenic etiology. NPHS1 gene was the most implicated (9/25) followed by PLCE1 (5/25). There were 12 variants of uncertain significance (VUS) involving 10 genes (10/25, 40%), and no definite genetic abnormality was found in 4 (25%). A re-analysis of these VUS attempted 2–3 years later facilitated reclassification of 7/12 (58%); increasing the diagnostic yield from 61 to 68.2%. Conclusions: Consistent with worldwide data, variants in NPHS1 gene were the most common cause of NS in infancy; however, PLCE1 was implicated more frequently in our cohort. NUP93 and COL4A3 were reported in early onset NS for the first time. Reclassification of VUS should be attempted, if feasible, since it may lead to a useful revision of diagnosis. [ABSTRACT FROM AUTHOR]

Background: Membranous nephropathy (MN) is an autoimmune disease in which circulating autoantibodies bind to a podocyte antigen, causing nephrotic syndrome. Despite several treatment options, their long-term benefits are still not established. The aim of this study is to investigate the long-term efficacy of cyclosporine and methylprednisolone in MN treatment. Methods: In this retrospective study, we included adults with biopsy-proven idiopathic MN. At diagnosis, all patients received conservative treatment, and those who did not show remission after 6 months received immunosuppression with cyclosporine and methylprednisolone. Those that despite no remission chose not to receive immunosuppression served as controls. Results: Sixty patients were included in the study with a follow-up of 94.8 ± 55.5 months. Forty-nine patients had nephrotic syndrome, and 47 received immunosuppression, while 13 received no treatment. Out of those who received immunosuppression, 63.8% showed complete, 14.9% partial, and 19.1% no remission of nephrotic syndrome. End-stage kidney disease (ESKD) or doubling of serum creatinine was reached by 12. Out of those who received no immunosuppression, 23.1% showed complete, 30.8% partial, and 23.1% had no remission. Doubling of serum creatinine or ESKD was reached by 1. Kidney survival was not altered by immunosuppression and was not different among those who received immunosuppression and had no or up to 3 relapses. Either complete or partial remission was accompanied by better kidney survival. Conclusion: Treatment of idiopathic MN with cyclosporine and methylprednisolone offers a reliable option for proteinuria remission. Nevertheless, this option does not alter long-term disease progression. [ABSTRACT FROM AUTHOR]

Key Clinical Message: The commonest renal involvement after bee stings is acute kidney injury due to rhabdomyolysis. Nephrotic syndrome combined with AKI is unusual complication of Hymenoptera stings. We diagnosed a minimal change disease and six‐year follow up relapses. [ABSTRACT FROM AUTHOR]