Article
Pediatrics
Mira Krendel, Sabine Leh, Michael E. Garone, Alcia Edwards-Richards, Jen-Jar Lin, Damien Brackman, Per Knappskog, Alexei Mikhailov
Summary: Pathogenic mutations in non-muscle single-headed myosin 1E (Myo1e) are a rare cause of pediatric focal segmental glomerulosclerosis (FSGS). These mutations primarily affect the functions of actin cytoskeleton and cell adhesion, resulting in dysfunction of podocyte contractile actin cables and abnormalities in podocytes and glomerular filtration barrier. They are most commonly observed in children aged 1-11 and can lead to kidney failure. Clinical and histological analysis can tentatively differentiate this condition from other genetic podocytopathies and Alport syndrome.
PEDIATRIC NEPHROLOGY
(2023)
Article
Urology & Nephrology
Susan T. Veissi, Bart Smeets, Joanna A. E. van Wijk, Rene Classens, Thea J. A. M. van der Velden, Annelies Jeronimus-Klaasen, Floor Veltkamp, E. M. Mak-Nienhuis, William Morello, Giovanni Montini, Antonia H. M. Bouts, Lambertus P. W. J. van den Heuvel, Michiel F. Schreuder
Summary: This study demonstrates the presence of circulating permeability factors (CPFs) in plasma of FSGS patients through a series of in vitro assays. The results suggest that CPFs can cause podocyte injury, leading to focal segmental glomerulosclerosis-related diseases.
KIDNEY INTERNATIONAL REPORTS
(2022)
Article
Urology & Nephrology
Martina Tedesco, Federica Mescia, Isabella Pisani, Marco Allinovi, Giovanni Casazza, Lucia Del Vecchio, Marisa Santostefano, Luigi Cirillo, Francesca Ferrario, Ciro Esposito, Pasquale Esposito, Domenico Santoro, Roberta Lazzarin, Giovanni Maria Rossi, Enrico Fiaccadori, Angelo Ferrantelli, Renato Alberto Sinico, Mario Cozzolino, Maurizio Gallieni, Lino Cirami, Francesco Scolari, Augusto Vaglio, Federico Alberici
Summary: Rituximab (RTX) may be a treatment option for primary focal segmental glomerular sclerosis (FSGS), especially in steroid-dependent patients with 24-hour proteinuria <5 g and those who previously responded to RTX. The optimal long-term management for responders is unclear, with some patients experiencing sustained remission and others requiring RTX retreatment, either preemptively or after rising proteinuria.
KIDNEY INTERNATIONAL REPORTS
(2022)
Article
Biochemistry & Molecular Biology
Lorena Longaretti, Piera Trionfini, Valerio Brizi, Christodoulos Xinaris, Caterina Mele, Matteo Breno, Elena Romano, Roberta Giampietro, Giuseppe Remuzzi, Ariela Benigni, Susanna Tomasoni
Summary: A study on a patient with familial steroid-resistant FSGS identified a PAX2 mutation, suggesting a dual effect of the mutation in the onset of FSGS. Fixing the mutation improved the functionality of podocytes, providing insight into the development of proteinuria in FSGS.
Article
Immunology
Ibrahim Batal, Pascale Khairallah, Astrid Weins, Nicole K. Andeen, Michael B. Stokes
Summary: Primary focal segmental glomerulosclerosis (FSGS) is a disease characterized by diffuse podocyte foot process effacement and nephrotic syndrome. It can recur after transplantation and is associated with immunogenetic factors. This study retrospectively investigated a cohort of kidney allograft recipients with primary FSGS and found a higher frequency of HLA-A30 antigen in patients with primary FSGS compared to healthy controls and deceased kidney donors. The study also revealed an association between donor HLA-A30 and recurrent FSGS in transplant patients.
FRONTIERS IN IMMUNOLOGY
(2023)
Review
Urology & Nephrology
Ke Sun, Qionghong Xie, Chuan-Ming Hao
Summary: This study focuses on the potential sources and mechanism of podocyte supplementation in FSGS. Parietal epithelial cells (PECs) have gained attention for their role in the progression of FSGS, as both influencing factors in glomerulosclerosis lesions and their repair abilities are being debated. Other resident glomerular cells are also significant in the progression of the disease.
Article
Urology & Nephrology
Marijke Stryckers, Stefaan Van Oevelen, Priyanka Koshy, Ben Sprangers, Amaryllis H. Van Craenenbroeck
Summary: We present a case of nephrotic syndrome in a 38-year-old man shortly after initiation of guselkumab for plaque psoriasis treatment. Renal biopsy revealed focal segmental glomerulosclerosis (FSGS). The clinical course strongly suggests drug-induced FSGS, as the nephrotic syndrome resolved after discontinuation of the drug without relapse (2 years of follow-up). To the best of our knowledge, this is the first report of FSGS lesions associated with the use of an interleukin-23 inhibitor.
CLINICAL KIDNEY JOURNAL
(2023)
Article
Urology & Nephrology
Fang Li, Yili Fang, Qiyuan Zhuang, Meichu Cheng, Desmond Moronge, Hao Jue, Oded Meyuhas, Xiaoqiang Ding, Zhigang Zhang, Jian-Kang Chen, Huijuan Wu
Summary: The phosphorylation of ribosomal protein S6 (rpS6) plays a crucial role in podocyte hypertrophy and loss during the pathogenesis of focal segmental glomerulosclerosis (FSGS). Inhibiting rpS6 phosphorylation can effectively attenuate podocyte hypertrophy and depletion, thereby reducing the formation of FSGS lesions.
KIDNEY INTERNATIONAL
(2022)
Article
Chemistry, Multidisciplinary
Qiong-Dan Hu, Hong-Lian Wang, Jian Liu, Tao He, Rui-Zhi Tan, Qiong Zhang, Hong-Wei Su, Fahsai Kantawong, Hui-Yao Lan, Li Wang
Summary: Btg2 plays a pathogenic role in FSGS by promoting podocyte injury through a Smad3-dependent epithelial-mesenchymal transition pathway.
Article
Medicine, General & Internal
Da Woon Kim, Hakeong Jeon, Sungmi Kim, Wanhee Lee, Hyo Jin Kim, Harin Rhee, Sang Heon Song, Eun Young Seong
Summary: FSGS is a common primary glomerular disorder that can be caused by pembrolizumab, an immune checkpoint inhibitor used for advanced melanoma. This case study highlights the importance of monitoring renal function and considering kidney biopsy in patients treated with PD-1 inhibitors who develop renal injury or unexplained proteinuria. Treatment based on guidelines can lead to resolution of nephrotic syndrome in such cases.
Article
Biochemistry & Molecular Biology
Hui Zhang, Qing-Qing Dong, Hua-Pan Shu, Yu-Chi Tu, Qian-Qian Liao, Li-Jun Yao
Summary: This study explored the mechanism of curcumin (CUR) against focal segmental glomerulosclerosis (FSGS) through network pharmacological methods and experimental verification. CUR was found to attenuate apoptosis, oxidative stress damage, and epithelial-mesenchymal transition (EMT) in FSGS by repressing the AKT signaling pathway.
ARCHIVES OF BIOCHEMISTRY AND BIOPHYSICS
(2023)
Article
Medicine, General & Internal
Ruiqiang Wang, Yunqi Wu, Dongyue An, Pupu Ma, Yuanyuan Guo, Lin Tang
Summary: This case report presents a case of acromegaly complicated with focal segmental glomerulosclerosis (FSGS). Surgery reduced the effects of GH, while low doses of glucocorticoids and immunosuppressants effectively slowed the progression of kidney disease, at least in reducing urinary protein levels.
FRONTIERS IN MEDICINE
(2021)
Article
Urology & Nephrology
Daniela Verzola, Michela Saio, Daniela Picciotto, Francesca Viazzi, Elisa Russo, Leda Cipriani, Annalisa Carta, Francesca Costigliolo, Gabriele Gaggero, Gennaro Salvidio, Pasquale Esposito, Giacomo Garibotto, Laura Poggi
Summary: The study found that an increased level of cell senescence, particularly the upregulation of p16(INK4A) in tubules, is an independent predictor for progression to end-stage renal disease in adult FSGS patients.
AMERICAN JOURNAL OF NEPHROLOGY
(2021)
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, 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.
