Article
Urology & Nephrology
Ilse M. Rood, Aernoud Bavinck, Beata S. Lipska-Zietkiewicz, Dorien Lugtenberg, Franz Schaefer, Jeroen K. J. Deegens, Jack F. M. Wetzels
Summary: While corticosteroids may have therapeutic effects in patients with presumed primary focal segmental glomerular sclerosis (pFSGS), some patients are resistant to steroids and require additional immunosuppressive therapy. A decrease in proteinuria of >20% after 8 weeks of therapy can predict treatment response, but improved biomarkers are still needed to predict outcomes in patients with pFSGS.
KIDNEY INTERNATIONAL REPORTS
(2022)
Article
Urology & Nephrology
Michelle A. Hladunewich, Dan Cattran, Sanjeev M. Sethi, Salim S. Hayek, Jing Li, Changli Wei, Sarah Mullin, Heather N. Reich, Jochen Reiser, Fernando C. Fervenza
Summary: In this study, the use of rituximab in adult patients with treatment-resistant primary FSGS, high suPAR levels, and evidence of podocyte activation was found to be ineffective in improving proteinuria levels and glomerular filtration rate.
KIDNEY INTERNATIONAL REPORTS
(2022)
Article
Urology & Nephrology
Mahmoud Kallash, Yujie Wang, Abigail Smith, Howard Trachtman, Rasheed Gbadegesin, Carla Nester, Pietro Canetta, Chen Wang, Tracy E. Hunley, C. John Sperati, David Selewski, Isabelle Ayoub, Tarak Srivastava, Amy K. Mottl, Jeffrey Kopp, Brenda Gillespie, Bruce Robinson, Dhruti Chen, Julia Steinke, Katherine Twombley, Kimberly Reidy, Krzysztof Mucha, Larry A. Greenbaum, Brooke Blazius, Margaret Helmuth, Peleg Yonatan, Rulan S. Parekh, Susan Hogan, Virginie Royal, Vivette D'Agati, Aftab Chishti, Ronald Falk, Ali Gharavi, Lawrence Holzman, Jon Klein, William Smoyer, Matthias Kretzler, Debbie Gipson, Jason M. Kidd
Summary: Background: FSGS is a heterogeneous diagnosis with a guarded prognosis. APOL1 gene polymorphisms are associated with developing FSGS and faster progression to kidney failure. Understanding the natural history of FSGS patients with APOL1 risk alleles is important for patient care and intervention studies.
CLINICAL JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY
(2023)
Article
Hematology
Diletta Domenica Torres, Giulia Fonto, Luca Guastamacchia, Luisa Santangelo, Vincenza Carbone, Giovanni Piscopo, Federica Spadaccino, Elena Ranieri, Giuseppe Stefano Netti, Mario Giordano
Summary: In pediatric patients, early and intensive immunoadsorption (IA) treatment has shown efficacy in treating recurrent FSGS, reducing nephrotic proteinuria and improving clinical symptoms, while maintaining stable renal function.
BLOOD PURIFICATION
(2022)
Article
Urology & Nephrology
Pilar Aunon, Natalia Polanco, Maria Jose Perez-Saez, Emilio Rodrigo, Asuncion Sancho, Julio Pascual, Amado Andres, Manuel Praga
Summary: The recurrence rate of FSGS after kidney transplantation is related to the presence of nephrotic syndrome at the time of diagnosis and the effectiveness of pre-emptive rituximab treatment.
CLINICAL KIDNEY JOURNAL
(2021)
Article
Multidisciplinary Sciences
Suramath Isaranuwatchai, Ankanee Chanakul, Chupong Ittiwut, Rungnapa Ittiwut, Chalurmpon Srichomthong, Vorasuk Shotelersuk, Kanya Suphapeetiporn, Kearkiat Praditpornsilpa
Summary: The spectra of underlying genetic variants for FSGS vary among different populations. A study on biopsy-proven FSGS patients in Thailand identified disease-associated pathogenic/likely pathogenic variants in 11.3% of the cases. Genetic testing before treatment was found to be important to avoid unnecessary treatment.
SCIENTIFIC REPORTS
(2023)
Review
Medicine, General & Internal
Hamza Naciri Bennani, Lionel Elimby, Florian Terrec, Paolo Malvezzi, Johan Noble, Thomas Jouve, Lionel Rostaing
Summary: This study assessed graft survival in FSGS kidney-transplant recipients and compared patients with and without relapse. The results showed a recurrence rate of 47% for FSGS, and pretransplant prophylaxis did not seem to reduce the risk of relapse.
JOURNAL OF CLINICAL MEDICINE
(2022)
Article
Medicine, General & Internal
Debbie S. Gipson, Jonathan P. Troost, Cathie Spino, Samara Attalla, Joshua Tarnoff, Susan Massengill, Richard Lafayette, Virginia Vega-Warner, Sharon Adler, Patrick Gipson, Matthew Elliott, Frederick Kaskel, Damian Fermin, Marva Moxey-Mims, Richard N. Fine, Elizabeth J. Brown, Kimberly Reidy, Katherine Tuttle, Keisha Gibson, Kevin Lemley, Larry A. Greenbaum, Meredith A. Atkinson, Sangeeta Hingorani, Tarak Srivastava, Christine B. Sethna, Kevin Meyers, Cheryl Tran, Katherine M. Dell, Chia-shi Wang, Jennifer Lai Yee, Matthew G. Sampson, Rasheed Gbadegesin, J. J. Lin, Tammy Brady, Michelle Rheault, Howard Trachtman
Summary: This study examined whether there are differences in kidney health outcomes among children, adolescents, and adults with FSGS, and found that the association of FSGS with kidney survival and functional outcomes was comparable at all ages.
Article
Medicine, Research & Experimental
Su-Wei Hu, Yuan-Hung Wang, Jhy-Shrian Huang, Yea-Mey Yang, Chia-Chang Wu, Chao-Wen Cheng
Summary: The study found that vardenafil treatment can alleviate proteinuria, renal dysfunction, and hypercholesterolemia induced by focal segmental glomerulosclerosis, and improve the histopathological damage of the kidneys.
Article
Cell Biology
Lixia Wang, Jie Wang, Zhimin Wang, Jianhua Zhou, Yu Zhang
Summary: The study found that urine exosomal miR-193a levels were significantly higher in patients with primary FSGS compared to those with MCN and IgAN, and were positively correlated with glomerulosclerosis index. Exosomes from cultured podocytes could transport miR-193a-5p to recipient cells potentially through a calcium-dependent release mechanism.
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
(2021)
Article
Medicine, General & Internal
Hisashi Sugimoto, Naoki Sawa, Hajime Yamagiwa, Masahiro Kawada, Daisuke Ikuma, Yuki Oba, Hiroki Mizuno, Akinari Sekine, Masayuki Yamanouchi, Eiko Hasegawa, Tatsuya Suwabe, Aya Nishida, Kei Kono, Keiichi Kinowaki, Kenichi Ohashi, Yutaka Yamaguchi, Yoshifumi Ubara
Summary: A 72-year-old man with proteinuria and leg edema was diagnosed with essential thrombocythemia (ET), identified by thrombocytosis, elevated megakaryocytes, and JAK2 V617 mutation. Kidney biopsy revealed focal segmental glomerulosclerosis (FSGS) cellular variant and other FSGS variants, with positive megakaryocyte infiltrations. ET may cause FSGS by increasing intraglomerular pressure due to megakaryocyte infiltration.
Review
Urology & Nephrology
Conxita Jacobs-Cacha, Ander Vergara, Clara Garcia-Carro, Irene Agraz, Nestor Toapanta-Gaibor, Gema Ariceta, Francesc Moreso, Daniel Seron, Joan Lopez-Hellin, Maria Jose Soler
Summary: Primary or idiopathic FSGS is a kidney disease involving podocytes that leads to heavy proteinuria and may progress to end-stage renal disease. It has a poor prognosis, with 15% of patients being resistant to treatment and 30-50% experiencing disease recurrence after kidney transplantation. While confirming the diagnosis of primary FSGS can be complex, efforts to improve diagnostic approaches are ongoing to enhance understanding of the disease.
CLINICAL KIDNEY JOURNAL
(2021)
Review
Immunology
Giuseppe Salfi, Federica Casiraghi, Giuseppe Remuzzi
Summary: This article reviews the complex pathogenetic mechanisms of primary FSGS and its post-transplant recurrence, emphasizing the pivotal role of the immune system. By critically assessing existing literature, the study identifies crucial pathways for comprehensive understanding of FSGS pathogenesis and highlights important knowledge gaps. In-depth characterization of these intricate immune mechanisms could potentially lead to the identification of noninvasive biomarkers for accurately identifying high-risk patients and the development of targeted and personalized therapeutic approaches in the management of FSGS.
FRONTIERS IN IMMUNOLOGY
(2023)
Article
Transplantation
Takehiko Kawaguchi, Toshiyuki Imasawa, Moritoshi Kadomura, Hiroshi Kitamura, Shoichi Maruyama, Takaya Ozeki, Ritsuko Katafuchi, Kazumasa Oka, Yoshitaka Isaka, Hitoshi Yokoyama, Hitoshi Sugiyama, Hiroshi Sato
Summary: In this study, the associations between different variants of FSGS and renal outcomes were comprehensively investigated. The results showed that the FSGS variants alone did not have significant impacts on renal outcome after 5 years. However, proteinuria remission (PR) could predict improved renal prognosis for any variant.
NEPHROLOGY DIALYSIS TRANSPLANTATION
(2022)
Article
Pediatrics
Kenichiro Miura, Naoto Kaneko, Taeko Hashimoto, Kiyonobu Ishizuka, Yoko Shirai, Masataka Hisano, Hiroko Chikamoto, Yuko Akioka, Shoichiro Kanda, Yutaka Harita, Toshiyuki Yamamoto, Motoshi Hattori
Summary: This study aimed to differentiate pediatric kidney transplant recipients with focal segmental glomerulosclerosis (FSGS)/steroid-resistant nephrotic syndrome (SRNS) who are likely to have a monogenic cause from those who do not, and to predict post-transplant recurrence. The results showed that a systematic approach based on clinicopathological findings can be useful in identifying patients with a monogenic cause and predicting post-transplant recurrence in FSGS/SRNS.
PEDIATRIC NEPHROLOGY
(2023)
Article
Cell Biology
Laura Miesen, Peter Bandi, Brigith Willemsen, Fieke Mooren, Thiago Strieder, Eva Boldrini, Vedran Drenic, Jennifer Eymael, Roy Wetzels, Johannes Lotz, Nick Weiss, Eric Steenbergen, Toin H. van Kuppevelt, Merijn van Erp, Jeroen van der Laak, Nicole Endlich, Marcus J. Moeller, Jack F. M. Wetzels, Jitske Jansen, Bart Smeets
Summary: In focal segmental glomerulosclerosis (FSGS), the continuity of glomerular epithelial cells is disrupted, but non-sclerotic segments remain protected. Studies have shown that in FSGS, parietal epithelial cells (PECs) can cover the sclerotic lesions and restore the continuity of glomerular epithelial cells.
DISEASE MODELS & MECHANISMS
(2022)
Article
Urology & Nephrology
Brendon L. Neuen, Hocine Tighiouart, Hiddo J. L. Heerspink, Edward F. Vonesh, Juhi Chaudhari, Shiyuan Miao, Tak Mao Chan, Fernando C. Fervenza, Juergen Floege, Marian Goicoechea, William G. Herrington, Enyu Imai, Tazeen H. Jafar, Julia B. Lewis, Philip Kam-Tao Li, Francesco Locatelli, Bart D. Maes, Ronald D. Perrone, Manuel Praga, Annalisa Perna, Francesco P. Schena, Christoph Wanner, Jack F. M. Wetzels, Mark Woodward, Di Xie, Tom Greene, Lesley A. Inker
Summary: This study conducted a meta-analysis of randomized clinical trials to assess the magnitude and consistency of acute effects on CKD progression. The results showed that the acute effects vary across different interventions and are larger at higher baseline GFR. Understanding these acute effects can help improve the design of clinical trials evaluating CKD progression.
JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY
(2022)
Article
Physiology
Peter M. T. Deen, Michelle Boone, Horst Schweer, Emma T. B. Olesen, Claudia Carmone, Jack F. M. Wetzels, Robert A. Fenton, Marleen L. A. Kortenoeven
Summary: This study found that the differential effects of PGE(2) and PGF(2 alpha) on AQP2 abundance with or without dDAVP can be explained by reduced EP4 receptor and increased EP1/FP receptor expression by dDAVP.
FRONTIERS IN PHYSIOLOGY
(2022)
Article
Developmental Biology
Jitske Jansen, Bartholomeus T. van den Berge, Martijn van den Broek, Rutger J. Maas, Deniz Daviran, Brigith Willemsen, Rona Roverts, Marit van der Kruit, Christoph Kuppe, Katharina C. Reimer, Gianluca Di Giovanni, Fieke Mooren, Quincy Nlandu, Helmer Mudde, Roy Wetzels, Dirk den Braanker, Naomi Parr, James S. Nagai, Vedran Drenic, Ivan G. Costa, Eric Steenbergen, Tom Nijenhuis, Henry Dijkman, Nicole Endlich, Nicole C. A. J. van de Kar, Rebekka K. Schneider, Jack F. M. Wetzels, Anat Akiva, Johan van der Vlag, Rafael Kramann, Michiel F. Schreuder, Bart Smeets
Summary: Using organoid podocytes as a model, this study investigated idiopathic nephrotic syndrome and found that repaired organoids exhibited improved functionality, providing a valuable tool for disease modeling and therapy development.
Letter
Urology & Nephrology
Bryan van den Broek, Michiel van der Flier, Nicole C. A. J. van de Kar, Jack F. M. Wetzels, Marien I. de Jonge, Ronald de Groot, Kioa L. Wijnsma, Jeroen D. Langereis
KIDNEY INTERNATIONAL
(2022)
Letter
Urology & Nephrology
Coralien H. Vink, Bram van Cranenbroek, Joost W. van der Heijden, Hans P. J. M. Koenen, Jack F. M. Wetzels
KIDNEY INTERNATIONAL
(2022)
Editorial Material
Urology & Nephrology
Rutger J. Maas, Tom Nijenhuis, Johan van der Vlag
KIDNEY INTERNATIONAL REPORTS
(2022)
Article
Pediatrics
Agnes Trautmann, Olivia Boyer, Elisabeth Hodson, Arvind Bagga, Debbie S. Gipson, Susan Samuel, Jack Wetzels, Khalid Alhasan, Sushmita Banerjee, Rajendra Bhimma, Melvin Bonilla-Felix, Francisco Cano, Martin Christian, Deirdre Hahn, Hee Gyung Kang, Koichi Nakanishi, Hesham Safouh, Howard Trachtman, Hong Xu, Wendy Cook, Marina Vivarelli, Dieter Haffner
Summary: Idiopathic nephrotic syndrome is a common pediatric glomerular disease, and patients who are sensitive to steroids have a risk of relapse and may require long-term medication to maintain remission. The optimal dose and duration of steroid treatment to prolong time between relapses are still debated, and there is variation in practice regarding drug selection and timing. Therefore, international evidence-based clinical practice recommendations are needed to guide treatment and reduce practice variation.
PEDIATRIC NEPHROLOGY
(2023)
Article
Biochemistry & Molecular Biology
Dirk J. W. den Braanker, Rutger J. H. Maas, Guido van Mierlo, Naomi M. J. Parr, Marinka Bakker-van Bebber, Jeroen K. J. Deegens, Pascal W. T. C. Jansen, Jolein Gloerich, Brigith Willemsen, Henry B. B. Dijkman, Alain J. J. van Gool, Jack F. M. Wetzels, Markus M. M. Rinschen, Michiel Vermeulen, Tom Nijenhuis, Johan van der Vlag
Summary: This study aimed to investigate the possible factors and mechanisms leading to recurrence of focal segmental glomerulosclerosis (FSGS) after kidney transplantation. It was found that a protein called perilipin-2 was significantly increased in infiltrating cells of patients' glomeruli and was associated with lipid droplet accumulation, suggesting it as a potential biomarker. Further research should focus on understanding how these factors alter lipid metabolism in podocytes and provide new treatment strategies.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Review
Immunology
Ben Sprangers, Ute Hegenbart, Jack F. M. Wetzels
Summary: MGRS refers to kidney diseases caused by monoclonal immunoglobulins produced by B-cell or plasma-cell clones, which do not meet current hematologic criteria for therapy. The diseases can lead to end stage kidney disease. The decision of kidney transplantation in MGRS patients should be personalized, considering factors such as the subtype of MGRS-associated kidney disease, patient age and comorbidity, extrarenal complications, waiting time, living kidney donor availability, and previous hematological treatment.
Article
Urology & Nephrology
Coralien H. Vink, Anne-Els van de Logt, Renate G. van der Molen, Julia M. Hofstra, Jack F. M. Wetzels
Summary: An antibody-guided treatment schedule is effective in the treatment of primary membranous nephropathy, providing individualized therapy with many patients responding well to 8 weeks of treatment.
KIDNEY INTERNATIONAL REPORTS
(2023)
Meeting Abstract
Urology & Nephrology
Ilana Heckler, Coralien Vink, Anne-Els van de Logt, Alexander Kuehnl, Cornelia Daehnrich, Wolfgang Schlumberger, Jack Wetzels
AMERICAN JOURNAL OF KIDNEY DISEASES
(2023)
Article
Urology & Nephrology
Steven D. Podos, Howard Trachtman, Gerald B. Appel, Andrew S. Bomback, Bradley P. Dixon, Jack F. M. Wetzels, H. Terence Cook, Samir V. Parikh, Matthew C. Pickering, James Tumlin, Craig B. Langman, Liz Lightstone, C. John Sperati, Erica Daina, Koenraad Peter Bouman, Kara Rice, Jane A. Thanassi, Mingjun Huang, Carla Nester, Giuseppe Remuzzi
Summary: This study investigated biomarkers in patients with C3G and found associations between complement biomarkers, kidney function, and kidney histology. These findings are important for understanding C3G and characterizing patients with this heterogeneous disease.
AMERICAN JOURNAL OF NEPHROLOGY
(2023)
Article
Cell Biology
Jennifer Eymael, Brigith Willemsen, Joyce Xu, Fieke Mooren, Eric Steenbergen, Jack F. Wetzels, Henry Dijkman, Jitske Jansen, Johan van der Vlag, Bart Smeets
Summary: Multiple motile cilia with a central microtubular pair were found in kidney biopsies of patients with kidney diseases, and these cells were associated with tubular injury and interstitial fibrosis. This suggests the ability of proximal tubular cells to transdifferentiate into multiciliated cells.
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
(2022)
Meeting Abstract
Urology & Nephrology
Brad H. Rovin, Sharon G. Adler, Elion Hoxha, Ben Sprangers, Rolf Stahl, Jack F. M. Wetzels, Julia Jauch-Lembach, Janine Griese, Rainer Boxhammer, Lei Xu, Stefan Hartle, Pierre M. Ronco
AMERICAN JOURNAL OF KIDNEY DISEASES
(2022)