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, 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
Medicine, General & Internal
Qiongxiu Zhou, Qinjie Weng, Xiaoyan Zhang, Yunzi Liu, Jun Tong, Xu Hao, Hao Shi, Pingyan Shen, Hong Ren, Jingyuan Xie, Nan Chen
Summary: The study found that the NPHS2 p.R229Q variant is associated with an increased risk of FSGS/SRNS, especially in Caucasian populations and early-onset patients.
FRONTIERS IN MEDICINE
(2022)
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
Pediatrics
George W. Burke, Jayanthi Chandar, Junichiro Sageshima, Mariella Ortigosa-Goggins, Pooja Amarapurkar, Alla Mitrofanova, Marissa J. Defreitas, Chryso P. Katsoufis, Wacharee Seeherunvong, Alexandra Centeno, Javier Pagan, Lumen A. Mendez-Castaner, Adela D. Mattiazzi, Warren L. Kupin, Giselle Guerra, Linda J. Chen, Mahmoud Morsi, Jose M. G. Figueiro, Rodrigo Vianna, Carolyn L. Abitbol, David Roth, Alessia Fornoni, Phillip Ruiz, Gaetano Ciancio, Eduardo H. Garin
Summary: Podocyte B7-1 staining in biopsies of KTRs with post-transplant FSGS identifies a subset of patients who may benefit from abatacept.
PEDIATRIC NEPHROLOGY
(2023)
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
Medicine, Research & Experimental
Taihei Suzuki, Masayuki Iyoda, Nobuhiro Kanazawa, Shohei Tachibana, Hirokazu Honda
Summary: Inhibition of PCSK9 can improve kidney disease by reducing urinary albumin and ameliorating podocytopathy through regulating lipid uptake and inflammation signaling pathways. This study suggests that targeting PCSK9 may serve as a potential therapeutic strategy for kidney disease.
LABORATORY INVESTIGATION
(2023)
Article
Biochemistry & Molecular Biology
Lilas Batool, Krithika Hariharan, Yao Xu, Mario Kassmann, Dmitry Tsvetkov, Bjoern-Oliver Gohlke, Sylvia Kaden, Manfred Gossen, Bernd Nuernberg, Andreas Kurtz, Maik Gollasch
Summary: Mutations in TRPC6 gene cause familial focal segmental glomerulosclerosis (FSGS) and are inherited dominantly. Majority of TRPC6 mutations result in missense changes affecting calcium influx; however, the underlying molecular mechanisms for cell injury and kidney pathology remain unclear. A novel TRPC6 mutation (V691Kfs*) was identified in a large kindred without FSGS despite truncated TRPC6 protein. Functional studies showed that V691Kfs* mutation leads to closure of ion-conducting pathway and fully inactivates the TRPC6 channel-specific calcium influx, suggesting a complete loss-of-function phenotype. This study emphasizes the importance of increased calcium influx through TRPC6 for podocyte cell death.
CELLULAR AND MOLECULAR LIFE SCIENCES
(2023)
Article
Plant Sciences
Xin Li, Dong Qi, Meng-ying Wang, Kai Ji, Qun-ling Xie, Yu Wang, Shi-hao Du, Hua-ying Fan
Summary: The study revealed that the levels of suPAR and uPAR in SRNS patients can help predict glucocorticoids resistance in patients with idiopathic nephrotic syndrome (INS). The combination of TNF-alpha and IFN-gamma induces podocytes to establish a steroid resistance model in vitro. SAA can improve glucocorticoids resistance in podocytes by regulating the suPAR/uPAR-alpha v beta 3 signaling pathway, ultimately decreasing podocyte apoptosis and increasing Nephrin expression.
JOURNAL OF ETHNOPHARMACOLOGY
(2021)
Review
Urology & Nephrology
Cheng Xue, Bo Yang, Jing Xu, Chenchen Zhou, Liming Zhang, Xiang Gao, Bing Dai, Shengqiang Yu, Zhiguo Mao, Changlin Mei, Chenggang Xu
Summary: Rituximab (RTX) may be an effective and relatively safe alternative for the treatment of adult frequent-relapsing or steroid-dependent nephrotic syndrome patients. Patients with minimal change disease have a higher complete remission rate with RTX, while those with focal segmental glomerulosclerosis have a lower remission rate and further investigation is needed on long-term adverse events.
CLINICAL KIDNEY JOURNAL
(2021)
Article
Urology & Nephrology
Jing Yang, Yongli Xu, Linxia Deng, Luowen Zhou, Liru Qiu, Yu Zhang, Jianhua Zhou
Summary: This study identified three cases of isolated proteinuria and FSGS caused by CUBN gene mutations, expanding the spectrum of renal manifestation and genotype of CUBN gene mutations.
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
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
Urology & Nephrology
Lan Lan, Yuxin Lin, Binfeng Yu, Yin Wang, Hong Pan, Huijing Wang, Xiaowei Lou, Xiabing Lang, Qiankun Zhang, Lie Jin, Yi Yang, Liang Xiao, Jianghua Chen, Fei Han
Summary: Rituximab has been shown to be effective and safe in adult FR/SDNS patients with minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS), maintaining disease remission and minimizing corticosteroid exposure.
AMERICAN JOURNAL OF NEPHROLOGY
(2023)
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
Urology & Nephrology
Oleh Akchurin, Zhongfang Du, Nadira Ramkellawan, Vidhi Dalal, Seung Hyeok Han, James Pullman, Anne Musch, Katalin Susztak, Kimberly J. Reidy
JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY
(2016)
Article
Urology & Nephrology
Oleh M. Akchurin, Amy J. Kogon, Juhi Kumar, Christine B. Sethna, Hoda T. Hammad, Paul J. Christos, John D. Mahan, Larry A. Greenbaum, Robert Woroniecki
Article
Pediatrics
Oleh M. Akchurin
PEDIATRIC CLINICS OF NORTH AMERICA
(2019)
Article
Urology & Nephrology
Edwin Patino, Stephen B. Doty, Divya Bhatia, Kelly Meza, Yuan-Shan Zhu, Stefano Rivella, Mary E. Choi, Oleh Akchurin
KIDNEY INTERNATIONAL
(2020)
Letter
Urology & Nephrology
John R. Lee, Jeffrey Silberzweig, Oleh Akchurin, Mary E. Choi, Vesh Srivatana, Jonathan Lin, Frank Liu, Line Malha, Michelle Lubetzky, Darshana M. Dadhania, Divya Shankaranarayanan, Daniil Shimonov, Sanjay Neupane, Thalia Salinas, Aarti Bhasin, Elly Varma, Lorenz Leuprecht, Supriya Gerardine, Perola Lamba, Parag Goyal, Eric Caliendo, Victoria Tiase, Rahul Sharma, Joel C. Park, Peter A. D. Steel, Manikkam Suthanthiran, Yiye Zhang
CLINICAL JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY
(2021)
Article
Pediatrics
Kelly Meza, Sharmi Biswas, Yuan-Shan Zhu, Anuradha Gajjar, Eduardo Perelstein, Juhi Kumar, Oleh Akchurin
Summary: The study found that TNF-alpha in pediatric CKD is positively associated with biomarkers of CKD-MBD and negatively associated with height Z-score, indicating that inflammation may contribute to the development of CKD-MBD and growth impairment in this population.
PEDIATRIC NEPHROLOGY
(2021)
Review
Pediatrics
Edwin Patino, Oleh Akchurin
Summary: Chronic kidney disease causes alterations in iron metabolism, leading to anemia and the need for iron supplementation. Therapeutic iron supplementation has pleiotropic effects on various organ systems in CKD patients, beyond traditional hematologic effects. The effects of iron supplementation depend on the administration route and the specific iron preparation used.
PEDIATRIC NEPHROLOGY
(2022)
Article
Physiology
Brent A. Momb, Edwin Patino, Oleh M. Akchurin, Mark S. Miller
Summary: Children with chronic kidney disease (CKD) often experience delayed physical development and reduced physical performance, possibly due to skeletal muscle dysfunction. However, the cellular and molecular mechanisms underlying skeletal muscle impairment in juvenile CKD are still poorly understood.
PHYSIOLOGICAL REPORTS
(2023)
Article
Pediatrics
Kelly Meza, Sharmi Biswas, Chantal Talmor, Kanza Baqai, Dmitry Samsonov, Sonia Solomon, Oleh Akchurin
Summary: Around 30% of children with chronic kidney disease do not respond to oral iron therapy for anemia. The severity of anemia at baseline is correlated with treatment response, suggesting the need for further studies to improve iron therapy outcomes and identify predictors of treatment response in children with anemia of CKD.
PEDIATRIC NEPHROLOGY
(2023)
Article
Urology & Nephrology
Brent A. Momb, Edwin Patino, Oleh M. Akchurin, Mark S. Miller
Summary: Patients with chronic kidney disease (CKD) often have compromised physical performance. In this study, it was found that untreated CKD led to a significant reduction in skeletal muscle force production, which was not solely due to muscle mass loss. Iron supplementation may help improve CKD-induced myopathy.
Article
Urology & Nephrology
Oleh Akchurin, Kelly Meza, Sharmi Biswas, Michaela Greenbaum, Alexandra P. Licona-Freudenstein, Parag Goyal, Justin J. Choi, Mary E. Choi
Summary: Patients with CKD are at higher risk of in-hospital mortality due to COVID-19, especially in elderly populations. Acute-on-chronic kidney injury increases the likelihood of in-hospital death in patients with CKD hospitalized with COVID-19.
Article
Medicine, Research & Experimental
Divya Bhatia, Kuei-Pin Chung, Kiichi Nakahira, Edwin Patino, Michelle C. Rice, Lisa K. Torres, Thangamani Muthukumar, Augustine M. K. Choi, Oleh M. Akchurin, Mary E. Choi
Article
Urology & Nephrology
Oleh Akchurin, Edwin Patino, Vidhi Dalal, Kelly Meza, Divya Bhatia, Simon Brovender, Yuan-Shan Zhu, Susanna Cunningham-Rundles, Eduardo Perelstein, Juhi Kumar, Stefano Rivella, Mary E. Choi
KIDNEY INTERNATIONAL REPORTS
(2019)
Article
Medicine, Research & Experimental
Angara Sureshbabu, Edwin Patino, Kevin C. Ma, Kristian Laursen, Eli J. Finkelsztein, Oleh Akchurin, Thangamani Muthukumar, Stefan W. Ryter, Lorraine Gudas, Augustine M. K. Choi, Mary E. Choi
Article
Physiology
Oleh Akchurin, Angara Sureshbabu, Steve B. Doty, Yuan-Shan Zhu, Edwin Patino, Susanna Cunningham-Rundles, Mary E. Choi, Adele Boskey, Stefano Rivella
AMERICAN JOURNAL OF PHYSIOLOGY-RENAL PHYSIOLOGY
(2016)