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Biomedicines
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Nephrotic Syndrome: Pathomechanism, Diagnostics and Novel Treatment Options
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Nephrotic Syndrome: Pathomechanism, Diagnostics and Novel Treatment Options

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

Deadline for manuscript submissions: closed (31 December 2024) | Viewed by 32958

Special Issue 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 that focus on NS. Articles focused 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 100 words) can be sent to the Editorial Office for announcement on this website.

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-blind 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 (9 papers)

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Editorial

Jump to: Research, Review

3 pages, 158 KiB  
Editorial
Special Issue “Nephrotic Syndrome: Pathomechanism, Diagnostics, and Novel Treatment Options”
by Kan Katayama, Ryosuke Saiki and Kaoru Dohi
Biomedicines 2024, 12(12), 2862; https://doi.org/10.3390/biomedicines12122862 - 17 Dec 2024
Viewed by 788
Abstract
Nephrotic syndrome (NS) is characterized by massive proteinuria, hypoproteinemia, and edema [...] Full article
(This article belongs to the Special Issue Nephrotic Syndrome: Pathomechanism, Diagnostics and Novel Treatment Options)

Research

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10 pages, 592 KiB  
Article
The Urine Light Chain/eGFR Quotient as a Tool to Rule out Cast Nephropathy in Myeloma-Associated Kidney Failure
by David Klank, Christian Löffler, Julian Friedrich, Martin Hoffmann, Peter Paschka and Raoul Bergner
Biomedicines 2024, 12(5), 1032; https://doi.org/10.3390/biomedicines12051032 - 8 May 2024
Viewed by 1201
Abstract
Kidney involvement with resulting kidney failure leads to increased mortality in patients with multiple myeloma (MM). Cast nephropathy (CN), in particular, if left untreated, quickly leads to kidney failure requiring dialysis and has a very poor prognosis for the affected patient. The gold [...] Read more.
Kidney involvement with resulting kidney failure leads to increased mortality in patients with multiple myeloma (MM). Cast nephropathy (CN), in particular, if left untreated, quickly leads to kidney failure requiring dialysis and has a very poor prognosis for the affected patient. The gold standard for diagnosing kidney involvement is a kidney biopsy. However, due to bleeding risk, this cannot be done in every patient. We recently reported that a quotient of urine light chain (LCurine) to glomerular filtration rate (eGFR) is a non-invasive diagnostic tool for patients with kidney involvement in MM. But this quotient has not yet been tested in everyday clinical practice. In this study, our LCurine/eGFR ratio was tested on 67 patients in two centers. Enrollment took place between January 2019 and September 2023. A total of 18 of the 67 patients had CN. With the threshold defined in our initial paper, we were able to show a sensitivity of 100% with a specificity of 85.7% for CN in patients with MM. As a result, the LCurine/eGFR quotient recognizes 100% of all CN and can therefore detect this group, which has a very poor prognosis, without the need for a kidney biopsy. Full article
(This article belongs to the Special Issue Nephrotic Syndrome: Pathomechanism, Diagnostics and Novel Treatment Options)
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11 pages, 864 KiB  
Article
Consequences of Nephrotic Proteinuria and Nephrotic Syndrome after Kidney Transplant
by María José Ortega, Miguel Martínez-Belotto, Cristina García-Majado, Lara Belmar, Covadonga López del Moral, Jose María Gómez-Ortega, Rosalía Valero, Juan Carlos Ruiz and Emilio Rodrigo
Biomedicines 2024, 12(4), 767; https://doi.org/10.3390/biomedicines12040767 - 30 Mar 2024
Viewed by 1612
Abstract
Proteinuria is the main predictor of kidney graft loss. However, there is little information regarding the consequences of nephrotic proteinuria (NP) and nephrotic syndrome (NS) after a kidney transplant. We aimed to describe the clinical and histopathological characteristics of kidney recipients with nephrotic-range [...] Read more.
Proteinuria is the main predictor of kidney graft loss. However, there is little information regarding the consequences of nephrotic proteinuria (NP) and nephrotic syndrome (NS) after a kidney transplant. We aimed to describe the clinical and histopathological characteristics of kidney recipients with nephrotic-range proteinuria and compare the graft surveillance between those who developed NS and those who did not. A total of 204 patients (18.6% of kidney transplants in the study period) developed NP, and 68.1% of them had NS. Of the 110 patients who underwent a graft biopsy, 47.3% exhibited ABMR, 21.8% the recurrence of glomerulonephritis, 9.1% IFTA, and 7.3% de novo glomerulonephritis. After a median follow-up of 97.5 months, 64.1% experienced graft loss. The graft survival after the onset of NP declined from 75.8% at 12 months to 38% at 5 years, without significant differences between those with and those without NS. Patients who developed NS fewer than 3 months after the onset of NP exhibited a significantly higher risk of death-censored graft loss (HR: 1.711, 95% CI: 1.147–2.553) than those without NS or those with late NS. In conclusion, NP and NS are frequent conditions after a kidney transplant, and they imply extremely poor graft outcomes. The time from the onset of NP to the development of NS is related to graft survival. Full article
(This article belongs to the Special Issue Nephrotic Syndrome: Pathomechanism, Diagnostics and Novel Treatment Options)
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Review

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14 pages, 1020 KiB  
Review
Comparisons of Intradialytic Exercise Versus Home-Based Exercise in Hemodialysis Patients: A Narrative Review
by Chao-Lin Lee, Ping-Chen Wang, Yi-Ling Chen, Zen-Yong Chen, Ching-Cherng Uen, Hsien-Yung Lai and Chih-Chung Shiao
Biomedicines 2024, 12(10), 2364; https://doi.org/10.3390/biomedicines12102364 - 16 Oct 2024
Cited by 1 | Viewed by 2718
Abstract
With the increasing prevalence of end-stage kidney disease, the number of patients requiring hemodialysis (HD) continues to rise. While life-sustaining, HD is often associated with adverse effects such as muscle loss, physical deconditioning, fatigue, and compromised health-related quality of life (HRQoL). Recent research [...] Read more.
With the increasing prevalence of end-stage kidney disease, the number of patients requiring hemodialysis (HD) continues to rise. While life-sustaining, HD is often associated with adverse effects such as muscle loss, physical deconditioning, fatigue, and compromised health-related quality of life (HRQoL). Recent research suggests that intradialytic exercise (IDE) and home-based exercise (HBE) may mitigate these adverse effects and improve patient outcomes. However, the existing literature mainly focuses on the outcomes of both exercises, whereas the comparison of types is often omitted. Hence, this review consolidates findings from studies investigating the effectiveness, implementation, safety, feasibility, and adherence of different types of IDE and HBE in HD patients. Overall, the current literature bolsters the significance of IDE and HBE for improving health in HD patients. IDE and HBE enhance physical function, cardiopulmonary capacity, HRQoL, and cognitive well-being. Some research proposed an indirect link between IDE and survival rates. Despite these benefits, challenges remain in implementing these exercise modalities, including patient adherence and the feasibility of routine exercise during HD sessions. Integrating these exercises into routine care allows healthcare providers to enhance outcomes for HD patients. Further research is suggested to optimize exercise protocols and explore long-term effects and cost-effectiveness. Full article
(This article belongs to the Special Issue Nephrotic Syndrome: Pathomechanism, Diagnostics and Novel Treatment Options)
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40 pages, 1887 KiB  
Review
An Updated Comprehensive Review on Diseases Associated with Nephrotic Syndromes
by Ralph Wendt, Alina Sobhani, Paul Diefenhardt, Moritz Trappe and Linus Alexander Völker
Biomedicines 2024, 12(10), 2259; https://doi.org/10.3390/biomedicines12102259 - 4 Oct 2024
Cited by 1 | Viewed by 11568
Abstract
There have been exciting advances in our knowledge of primary glomerular diseases and nephrotic syndromes in recent years. Beyond the histological pattern from renal biopsy, more precise phenotyping of the diseases and the use of modern nephrogenetics helps to improve treatment decisions and [...] Read more.
There have been exciting advances in our knowledge of primary glomerular diseases and nephrotic syndromes in recent years. Beyond the histological pattern from renal biopsy, more precise phenotyping of the diseases and the use of modern nephrogenetics helps to improve treatment decisions and sometimes also avoid unnecessary exposure to potentially toxic immunosuppression. New biomarkers have led to easier and more accurate diagnoses and more targeted therapeutic decisions. The treatment landscape is becoming wider with a pipeline of promising new therapeutic agents with more sophisticated approaches. This review focuses on all aspects of entities that are associated with nephrotic syndromes with updated information on recent advances in each field. This includes podocytopathies (focal segmental glomerulosclerosis and minimal-change disease), membranous nephropathy, membranoproliferative glomerulonephritis, IgA nephropathy, fibrillary glomerulonephritis, amyloidosis, and monoclonal gammopathy of renal significance in the context of the nephrotic syndrome, but also renal involvement in systemic diseases, diabetic nephropathy, and drugs that are associated with nephrotic syndromes. Full article
(This article belongs to the Special Issue Nephrotic Syndrome: Pathomechanism, Diagnostics and Novel Treatment Options)
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17 pages, 967 KiB  
Review
Endocrine Disorders in Nephrotic Syndrome—A Comprehensive Review
by Maja Mizdrak, Bozo Smajic, Ivan Mizdrak, Tina Ticinovic Kurir, Marko Kumric, Ivan Paladin, Darko Batistic and Josko Bozic
Biomedicines 2024, 12(8), 1860; https://doi.org/10.3390/biomedicines12081860 - 15 Aug 2024
Cited by 1 | Viewed by 4786
Abstract
Nephrotic syndrome is a clinical syndrome characterized by massive proteinuria, called nephrotic range proteinuria (over 3.5 g per day in adults or 40 mg/m2 per hour in children), hypoalbuminemia, oncotic edema, and hyperlipidemia, with an increasing incidence over several years. Nephrotic syndrome [...] Read more.
Nephrotic syndrome is a clinical syndrome characterized by massive proteinuria, called nephrotic range proteinuria (over 3.5 g per day in adults or 40 mg/m2 per hour in children), hypoalbuminemia, oncotic edema, and hyperlipidemia, with an increasing incidence over several years. Nephrotic syndrome carries severe morbidity and mortality risk. The main pathophysiological event in nephrotic syndrome is increased glomerular permeability due to immunological, paraneoplastic, genetic, or infective triggers. Because of the marked increase in the glomerular permeability to macromolecules and the associated urinary loss of albumins and hormone-binding proteins, many metabolic and endocrine abnormalities are present. Some of them are well known, such as overt or subclinical hypothyroidism, growth hormone depletion, lack of testosterone, vitamin D, and calcium deficiency. The exact prevalence of these disorders is unknown because of the complexity of the human endocrine system and the differences in their prevalence. This review aims to comprehensively analyze all potential endocrine and hormonal complications of nephrotic syndrome and, vice versa, possible kidney complications of endocrine diseases that might remain unrecognized in everyday clinical practice. Full article
(This article belongs to the Special Issue Nephrotic Syndrome: Pathomechanism, Diagnostics and Novel Treatment Options)
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15 pages, 1515 KiB  
Review
Swollen Feet: Considering the Paradoxical Roles of Interleukins in Nephrotic Syndrome
by Maria E. Kovalik, Monique A. Dacanay, Steven D. Crowley and Gentzon Hall
Biomedicines 2024, 12(4), 738; https://doi.org/10.3390/biomedicines12040738 - 26 Mar 2024
Viewed by 1862
Abstract
Interleukins are a family of 40 bioactive peptides that act through cell surface receptors to induce a variety of intracellular responses. While interleukins are most commonly associated with destructive, pro-inflammatory signaling in cells, some also play a role in promoting cellular resilience and [...] Read more.
Interleukins are a family of 40 bioactive peptides that act through cell surface receptors to induce a variety of intracellular responses. While interleukins are most commonly associated with destructive, pro-inflammatory signaling in cells, some also play a role in promoting cellular resilience and survival. This review will highlight recent evidence of the cytoprotective actions of the interleukin 1 receptor (IL-1R)- and common gamma chain receptor (IL-Rγc)-signaling cytokines in nephrotic syndrome (NS). NS results from the injury or loss of glomerular visceral epithelial cells (i.e., podocytes). Although the causes of podocyte dysfunction vary, it is clear that pro-inflammatory cytokines play a significant role in regulating the propagation, duration and severity of disease. Pro-inflammatory cytokines signaling through IL-1R and IL-Rγc have been shown to exert anti-apoptotic effects in podocytes through the phosphoinositol-3-kinase (PI-3K)/AKT pathway, highlighting the potential utility of IL-1R- and IL-Rγc-signaling interleukins for the treatment of podocytopathy in NS. The paradoxical role of interleukins as drivers and mitigators of podocyte injury is complex and ill-defined. Emerging evidence of the cytoprotective role of some interleukins in NS highlights the urgent need for a nuanced understanding of their pro-survival benefits and reveals their potential as podocyte-sparing therapeutics for NS. Full article
(This article belongs to the Special Issue Nephrotic Syndrome: Pathomechanism, Diagnostics and Novel Treatment Options)
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16 pages, 618 KiB  
Review
Complement Activation in Nephrotic Glomerular Diseases
by Dominik Nell, Robert Wolf, Przemyslaw Marek Podgorny, Tobias Kuschnereit, Rieke Kuschnereit, Thomas Dabers, Sylvia Stracke and Tilman Schmidt
Biomedicines 2024, 12(2), 455; https://doi.org/10.3390/biomedicines12020455 - 18 Feb 2024
Cited by 2 | Viewed by 3521
Abstract
The nephrotic syndrome holds significant clinical importance and is characterized by a substantial protein loss in the urine. Damage to the glomerular basement membrane or podocytes frequently underlies renal protein loss. There is an increasing belief in the involvement of the complement system, [...] Read more.
The nephrotic syndrome holds significant clinical importance and is characterized by a substantial protein loss in the urine. Damage to the glomerular basement membrane or podocytes frequently underlies renal protein loss. There is an increasing belief in the involvement of the complement system, a part of the innate immune system, in these conditions. Understanding the interactions between the complement system and glomerular structures continually evolves, challenging the traditional view of the blood–urine barrier as a passive filter. Clinical studies suggest that a precise inhibition of the complement system at various points may soon become feasible. However, a thorough understanding of current knowledge is imperative for planning future therapies in nephrotic glomerular diseases such as membranous glomerulopathy, membranoproliferative glomerulonephritis, lupus nephritis, focal segmental glomerulosclerosis, and minimal change disease. This review provides an overview of the complement system, its interactions with glomerular structures, and insights into specific glomerular diseases exhibiting a nephrotic course. Additionally, we explore new diagnostic tools and future therapeutic approaches. Full article
(This article belongs to the Special Issue Nephrotic Syndrome: Pathomechanism, Diagnostics and Novel Treatment Options)
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16 pages, 798 KiB  
Review
Recent Advances in Proteinuric Kidney Disease/Nephrotic Syndrome: Lessons from Knockout/Transgenic Mouse Models
by Ryosuke Saiki, Kan Katayama and Kaoru Dohi
Biomedicines 2023, 11(7), 1803; https://doi.org/10.3390/biomedicines11071803 - 23 Jun 2023
Cited by 2 | Viewed by 3268
Abstract
Proteinuria is known to be associated with all-cause and cardiovascular mortality, and nephrotic syndrome is defined by the level of proteinuria and hypoalbuminemia. With advances in medicine, new causative genes for genetic kidney diseases are being discovered increasingly frequently. We reviewed articles on [...] Read more.
Proteinuria is known to be associated with all-cause and cardiovascular mortality, and nephrotic syndrome is defined by the level of proteinuria and hypoalbuminemia. With advances in medicine, new causative genes for genetic kidney diseases are being discovered increasingly frequently. We reviewed articles on proteinuria/nephrotic syndrome, focal segmental glomerulosclerosis, membranous nephropathy, diabetic kidney disease/nephropathy, hypertension/nephrosclerosis, Alport syndrome, and rare diseases, which have been studied in mouse models. Significant progress has been made in understanding the genetics and pathophysiology of kidney diseases thanks to advances in science, but research in this area is ongoing. In the future, genetic analyses of patients with proteinuric kidney disease/nephrotic syndrome may ultimately lead to personalized treatment options. Full article
(This article belongs to the Special Issue Nephrotic Syndrome: Pathomechanism, Diagnostics and Novel Treatment Options)
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