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Biomedicines
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Nephrotic Syndrome: Pathomechanism, Diagnostics and Novel Treatment Options—2nd Edition
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Nephrotic Syndrome: Pathomechanism, Diagnostics and Novel Treatment Options—2nd Edition

A special issue of Biomedicines (ISSN 2227-9059). This special issue belongs to the section "Molecular and Translational Medicine".

Deadline for manuscript submissions: 30 September 2026 | Viewed by 18471

Editor

Dr. Kan Katayama

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Guest Editor
Department of Cardiology and Nephrology, Mie University Graduate School of Medicine, 2-174 Edobashi, Tsu 514-8507, Japan
Interests: nephrotic syndrome; interstitial nephritis; thin basement membrane nephropathy
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Nephrotic syndrome (NS) is characterized by massive proteinuria, hypoproteinemia, and edema, and it is divided into steroid-sensitive NS (SSNS) and steroid-resistant NS (SRNS). While SSNS typically presents with minimal change NS, SRNS typically presents with focal segmental glomerulosclerosis (FSGS), which often leads to end-stage kidney failure. About 30% of cases of childhood-onset FSGS have been found to be hereditary FSGS caused by dozens of podocyte-related genes, and about 5-10% of cases of adult-onset FSGS have also been found to be hereditary FSGS.

The aim of this Special Issue is to gather original research articles and review articles focusing on NS. Articles with an emphasis on genetic forms of kidney disease are especially encouraged.

Dr. Kan Katayama
Guest Editor

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 250 words) can be sent to the Editorial Office for assessment.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-anonymized peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Biomedicines is an international peer-reviewed open access monthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2600 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • focal segmental glomerulosclerosis
  • genetic kidney disease
  • minimal change disease
  • nephrotic syndrome
  • podocyte

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Published Papers (5 papers)

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Research

Jump to: Review

16 pages, 6497 KB  
Article
Impact of Vascular Access Type and Obesity on Long-Term Thrombosis and Access Failure in Hemodialysis: A Real-World Cohort Study from the TriNetX Global Collaborative Network
by Hung-Jin Huang, Pao-Ting Wu, Li-Chin Sung, Cai-Mei Zheng and Hui-Wen Chiu
Biomedicines 2026, 14(6), 1380; https://doi.org/10.3390/biomedicines14061380 - 18 Jun 2026
Viewed by 461
Abstract
Background/Objectives: Optimal vascular access remains a critical determinant of outcomes in patients undergoing maintenance hemodialysis. While an arteriovenous fistula (AVF) is generally preferred over an arteriovenous graft (AVG), the impact of obesity and antithrombotic therapy on access-related complications remains incompletely defined. This [...] Read more.
Background/Objectives: Optimal vascular access remains a critical determinant of outcomes in patients undergoing maintenance hemodialysis. While an arteriovenous fistula (AVF) is generally preferred over an arteriovenous graft (AVG), the impact of obesity and antithrombotic therapy on access-related complications remains incompletely defined. This study evaluated the association between vascular access type, obesity status, and adverse outcomes in a large real-world cohort. Methods: We conducted a retrospective cohort study using de-identified electronic health record data from the TriNetX Global Collaborative Network. Adult patients (≥18 years) receiving maintenance hemodialysis were stratified by vascular access type (AVF vs. AVG), body mass index (normal: 18.5–24.9 kg/m2, obese: ≥30 kg/m2), and antithrombotic medication exposure. Propensity score matching (1:1) was performed within BMI strata. Primary outcomes included vascular access thrombosis, AVG failure, and AVF failure. Time-to-event analyses used Kaplan–Meier and Cox proportional hazards models. Results: AVG was associated with significantly higher rates of thrombosis and access failure compared with AVF in both obese and normal-weight cohorts (all p < 0.0001). In patients with obesity, thrombosis rates increased from 10.47% (AVF) to 17.54% (AVG) at 3 months to 34.32% versus 42.24% at 5 years. Kaplan–Meier analysis demonstrated early and persistent separation of thrombosis-free survival curves, with AVG associated with increased risk (HR 1.23; 95% CI, 1.07–1.41; log-rank p = 0.0001). Antithrombotic therapy reduced absolute risks but did not eliminate the relative disadvantage of AVG. Conclusions: In this large real-world cohort, AVG was consistently associated with higher risks of thrombosis and access failure compared with AVF, regardless of obesity status or medication exposure. These findings support preferential use of AVF and highlight the need for individualized vascular access strategies in patients undergoing hemodialysis. Full article
(This article belongs to the Special Issue Nephrotic Syndrome: Pathomechanism, Diagnostics and Novel Treatment Options—2nd Edition)
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11 pages, 1117 KB  
Article
Serum Protein Electrophoresis and the Albumin-to-Globulin Ratio in the Differential Diagnosis of Minimal Change Disease and Focal Segmental Glomerulosclerosis
by László Bitó, Tamás Lantos, Krisztina Jost, Amir Reza Manafzadeh, Béla Iványi and Levente Kuthi
Biomedicines 2026, 14(3), 720; https://doi.org/10.3390/biomedicines14030720 - 20 Mar 2026
Viewed by 1347
Abstract
Background/Objectives: Differentiating minimal change disease (MCD) from focal segmental glomerulosclerosis (FSGS) remains a diagnostic challenge. We hypothesised that differences in glomerular protein selectivity could translate into distinct serum protein electrophoresis (SPEP) profiles, particularly in severe nephrotic syndrome. Methods: We retrospectively analysed SPEP profiles [...] Read more.
Background/Objectives: Differentiating minimal change disease (MCD) from focal segmental glomerulosclerosis (FSGS) remains a diagnostic challenge. We hypothesised that differences in glomerular protein selectivity could translate into distinct serum protein electrophoresis (SPEP) profiles, particularly in severe nephrotic syndrome. Methods: We retrospectively analysed SPEP profiles of adults with biopsy-proven MCD (n = 27), primary FSGS (n = 27), and secondary FSGS (n = 20). Diagnoses were established according to KDIGO guidelines and the Mayo Clinic classification. A severe subgroup was defined by a relative albumin fraction <40% to evaluate patterns in marked hypoalbuminaemia. Results: Secondary FSGS demonstrated significantly higher albumin-to-globulin (A/G) ratios compared with immune-mediated podocytopathies (MCD and primary FSGS), yielding excellent discrimination (AUC > 0.98). In contrast, discriminatory performance between MCD and primary FSGS in the overall cohort was limited (AUC = 0.657). However, within the severe subgroup, the A/G ratio provided clinically meaningful separation (AUC = 0.787). An A/G ratio > 0.49 identified primary FSGS with 86.7% sensitivity and 81.2% specificity. Correlation analysis revealed a strong inverse association between albumin and α2-globulin fractions in immune-mediated podocytopathies (ρ < −0.8), whereas this relationship was attenuated in secondary FSGS (ρ = −0.57). Conclusions: The A/G ratio may represent a practical adjunctive biomarker in the evaluation of podocytopathies. Values > 1.0 strongly favour secondary FSGS, while markedly reduced ratios in severe nephrosis are characteristic of MCD. These findings suggest that differences in glomerular selectivity and the hepatic compensatory response are reflected in routine electrophoretic profiles. Full article
(This article belongs to the Special Issue Nephrotic Syndrome: Pathomechanism, Diagnostics and Novel Treatment Options—2nd Edition)
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Review

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16 pages, 1264 KB  
Review
Drug–Drug Interactions Between Oral Anticoagulants and Calcineurin Inhibitors in Nephrotic Syndrome for Management of Thromboembolism
by Toshinori Hirai and Kan Katayama
Biomedicines 2026, 14(8), 1666; https://doi.org/10.3390/biomedicines14081666 - 24 Jul 2026
Viewed by 440
Abstract
Thromboembolism is a life-threatening complication of nephrotic syndrome, which is treated with oral anticoagulants (warfarin and direct oral anticoagulants [DOACs]) that possess a variety of pharmacokinetic characteristics. This narrative review focused on drug–drug interactions between oral anticoagulants and calcineurin inhibitors (CNIs) (e.g., tacrolimus, [...] Read more.
Thromboembolism is a life-threatening complication of nephrotic syndrome, which is treated with oral anticoagulants (warfarin and direct oral anticoagulants [DOACs]) that possess a variety of pharmacokinetic characteristics. This narrative review focused on drug–drug interactions between oral anticoagulants and calcineurin inhibitors (CNIs) (e.g., tacrolimus, cyclosporine, and voclosporin) for the management of thromboembolism in nephrotic syndrome. While warfarin has less potential for interacting with CNIs, DOACs carry a risk of drug–drug interactions with CNIs owing to the inhibition of drug-metabolizing enzymes and transporters, including cytochrome P450 3A4 (CYP3A4) and P-glycoprotein. Specifically, a significant increase in DOAC exposure was observed when DOACs were co-administered with cyclosporine, a more potent P-glycoprotein inhibitor than tacrolimus. Limited evidence suggests that more pronounced drug interactions occur in specific cases: (1) apixaban and CNIs in patients with renal impairment, (2) edoxaban combined with cyclosporine in patients with renal impairment, and (3) a combination of rivaroxaban with dual inhibitors of P-glycoprotein and CYP3A4 (e.g., cyclosporine and fluconazole). From a pharmacological viewpoint, CNI-induced nephrotoxicity and hypertension may increase the risk of critical bleeding in patients receiving DOACs. In addition, the variation factors of pharmacokinetic parameters, such as renal function, pharmacogenomic profiles, and pathophysiological changes directly caused by nephrotic syndrome, may vary between patients, which could potentially augment the magnitude of drug–drug interactions between oral anticoagulants and CNIs. Further studies are required to understand the clinical significance of drug interactions in patients with nephrotic syndrome. Full article
(This article belongs to the Special Issue Nephrotic Syndrome: Pathomechanism, Diagnostics and Novel Treatment Options—2nd Edition)
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17 pages, 3944 KB  
Review
Multiscale Imaging of Human Adipose Tissue: A Neglected Partner in Proteinuria Linked to Obesity
by Davide Viggiano, Erica Bortone, Salvatore Tolone, Francesco Saverio Lucido, Claudio Gambardella, Giusiana Nesta, Giuseppe Gigliotti, Michelangelo Nigro, Maddalena Paolicelli, Vittorio D'Orlando and Ludovico Docimo
Biomedicines 2025, 13(11), 2719; https://doi.org/10.3390/biomedicines13112719 - 6 Nov 2025
Viewed by 1137
Abstract
Nephrotic syndrome (NS) is a systemic disorder characterized not only by glomerular dysfunction but also by profound dysregulation of lipid metabolism and microvascular integrity. Adipose tissue, as a central lipid-handling and endocrine organ, undergoes structural and functional remodeling in chronic renal conditions yet [...] Read more.
Nephrotic syndrome (NS) is a systemic disorder characterized not only by glomerular dysfunction but also by profound dysregulation of lipid metabolism and microvascular integrity. Adipose tissue, as a central lipid-handling and endocrine organ, undergoes structural and functional remodeling in chronic renal conditions yet remains underexplored in this context. The aim of this manuscript is to integrate adipose tissue imaging into the diagnostic and mechanistic framework of NS. To establish this perspective, we first summarize current knowledge on adipose tissue architecture and imaging in both physiological states and renal disease. We then present a multimodal imaging approach—combining ultrasound (US), histology, and atomic force microscopy (AFM)—applied to human adipose tissue as a potential diagnostic and pathophysiological marker in NS. Original imaging from our laboratory experience is presented as a demonstrative material, complemented by literature synthesis. Given that different modalities of imaging-based characterization of adipose tissue are sparse across the literature, this pictorial review offers a guide to identifying structural biomarkers of adipose remodeling in NS. By bridging imaging modalities with metabolic and vascular perturbations observed in NS, this work aims to guide future research toward the clinical application of adipose tissue imaging in renal disease. This provides insights into cell size heterogeneity, vascular topology, and subcellular features such as membrane wrinkles and nanodomain organization. We propose that such morphometric parameters, accessible via minimally invasive biopsies, could serve as surrogate markers of adipose remodeling in nephrotic syndrome. This sets the stage for integrating adipose tissue imaging into the diagnostic and mechanistic evaluation of systemic features in NS. Full article
(This article belongs to the Special Issue Nephrotic Syndrome: Pathomechanism, Diagnostics and Novel Treatment Options—2nd Edition)
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33 pages, 452 KB  
Review
Uncommon Factors Leading to Nephrotic Syndrome
by Ljiljana Bogdanović, Ivana Babić, Mirjana Prvanović, Dragana Mijač, Ana Mladenović-Marković, Dušan Popović and Jelena Bogdanović
Biomedicines 2025, 13(8), 1907; https://doi.org/10.3390/biomedicines13081907 - 5 Aug 2025
Cited by 3 | Viewed by 13926
Abstract
Nephrotic syndrome (NS) is characterized by proteinuria, hypoalbuminemia, edema, and hyperlipidemia. Apart from the traditional causes of NS, such as minimal change disease, focal segmental glomerulosclerosis, diabetes, infections, malignancies, autoimmune conditions, and nephrotoxic agents, there are also rare causes of NS, whose knowledge [...] Read more.
Nephrotic syndrome (NS) is characterized by proteinuria, hypoalbuminemia, edema, and hyperlipidemia. Apart from the traditional causes of NS, such as minimal change disease, focal segmental glomerulosclerosis, diabetes, infections, malignancies, autoimmune conditions, and nephrotoxic agents, there are also rare causes of NS, whose knowledge is of the utmost importance. The aim of this article was to highlight the less well-known causes that have a significant impact on diagnosis and treatment. Genetic syndromes such as Schimke immuno-osseous dysplasia, familial lecithin-cholesterol acyltransferase deficiency with two clinical variants (fish-eye Disease and the p.Leu364Pro mutation), lead to NS through mechanisms involving podocyte and lipid metabolism dysfunction. Congenital disorders of glycosylation and Nail–Patella Syndrome emphasize the role of deranged protein processing and transcriptional regulation in glomerular injury. The link of NS with type 1 diabetes, though rare, suggests an etiology on the basis of common HLA loci and immune dysregulation. Histopathological analysis, particularly electron microscopy, shows mainly podocyte damage, mesangial sclerosis, and alteration of the basement membrane, which aids in differentiating rare forms. Prompt recognition of these novel etiologies by genetic analysis, renal biopsy, and an interdisciplinary panel is essential to avoid delays in diagnosis and tailored treatment. Full article
(This article belongs to the Special Issue Nephrotic Syndrome: Pathomechanism, Diagnostics and Novel Treatment Options—2nd Edition)
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