Review
Immunology
Manuela Colucci, Julie Oniszczuk, Marina Vivarelli, Vincent Audard
Summary: B cells play a crucial pathogenic role in idiopathic nephrotic syndrome by altering immune homeostasis and affecting podocyte structure and functions through the production of antibodies, expression of activating surface molecules, and release of specific cytokines. Despite their ability to protect against infections, dysregulated B cells are associated with various autoimmune diseases.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Chemistry, Medicinal
Li Lin, Weiming Wang, YiFan Wu, JingYuan Xie, Xiao Li, XiaoXia Pan, Wen Zhang, Jing Xu, YiKai Cai, Hong Ren, Nan Chen
Summary: The study demonstrates that RTX is highly effective in treating minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS), and recommends RTX consolidation therapy to significantly reduce the risk of relapse and reinforce long-term remission.
DRUG DESIGN DEVELOPMENT AND THERAPY
(2021)
Article
Biochemistry & Molecular Biology
Natalia Chebotareva, Anatoliy Vinogradov, Alexander G. Brzhozovskiy, Daria N. Kashirina, Maria I. Indeykina, Anna E. Bugrova, Marina Lebedeva, Sergey Moiseev, Evgeny N. Nikolaev, Alexey S. Kononikhin
Summary: The aim of this study was to determine the proteomic profile of urine from patients with FSGS and MCD. The results showed significant differences in protein profiles between patients with FSGS group 2 and FSGS group 1/MCD. Several proteins were identified as potential biomarkers for these diseases, including apolipoprotein A-IV, hemopexin, and components of the complement system.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Article
Cardiac & Cardiovascular Systems
Bowen Li, Qingbo Liu, Xihui Chen, Tangdong Chen, Wenhui Dang, Jing Zhao, Guangbin Cui, Kun Chen, Yuanming Wu
Summary: This study describes a patient with symmetrically distributed idiopathic atrial calcifications and suggests that these pathologic calcifications may be related to ABCC6 dysfunction leading to reduced plasma PPi content.
FRONTIERS IN CARDIOVASCULAR MEDICINE
(2022)
Review
Urology & Nephrology
Luigi Cirillo, Gianmarco Lugli, Valentina Raglianti, Fiammetta Ravaglia, Elisa Buti, Samuela Landini, Francesca Becherucci
Summary: This review summarizes current approaches in the diagnosis of podocytopathies, focusing on strategies aimed at elucidating the etiology underlying the histological picture. By integrating traditional concepts with new data, improvements in diagnostic and therapeutic strategies, as well as the management of disease recurrence after kidney transplantation, can be achieved.
CLINICAL KIDNEY JOURNAL
(2022)
Article
Urology & Nephrology
Yumin Jiang, Ting Wang, Wei Yu, Feng Wu, Ruixue Guo, Huangmin Li, Yiding Zhang, Ge Yan, Li Wang, Zhanzheng Zhao
Summary: In this study, clinical data and gut microbiota were used to differentiate idiopathic membranous nephropathy (IMN) from minimal change disease (MCD). The results showed significant differences in clinical data and gut microbiota composition between the two groups. The differential gut microbiota may contribute to kidney injury, and a noninvasive classifier combining clinical data and gut microbiota information was constructed with high discriminating efficacy.
Review
Rheumatology
Mrinalini Dey, Gyorgy Nagy, Elena Nikiphorou
Summary: Despite improved treatment for RA, around 30% of patients continue to experience symptoms, referred to as 'difficult-to-treat' (D2T) RA. Comorbidities are recognized as significant contributors to D2T disease and are perpetuated by the D2T state.
Article
Urology & Nephrology
Takaya Ozeki, Brenda W. Gillespie, Maria Larkina, Shoichi Maruyama, Fadhl Alakwaa, Matthias Kretzler, Laura H. Mariani
Summary: This study compared the baseline characteristics and complete remission rates of adult nephrotic syndrome patients in North America and Japan. The North American cohort had more FSGS and a higher prevalence of family history of kidney disease, while the Japanese cohort had older patients and more severe NS with better response to immunosuppressive therapy. FSGS, hypertension, and lower eGFR were predictors of poor treatment response in both cohorts.
Article
Medicine, General & Internal
Franziska Schauer, Federica Casetti, Dimitra Kiritsi
Summary: A 23-year-old male patient presented with recurrent painful erosions in the urethral outlet area. Further examination revealed gingival erosions. Diagnosis of mucous membrane pemphigoid (MMP) was confirmed. Treatment led to clinical remission of symptoms.
FRONTIERS IN MEDICINE
(2022)
Article
Urology & Nephrology
Hillarey K. Stone, Bin Huang, Chen Chen, Qing Ma, Michael R. Bennett, Prasad Devarajan
Summary: The study validates a urinary biomarker risk score to predict steroid resistance in adult patients with nephrotic syndrome at initial diagnosis. The risk score showed optimal sensitivity and specificity in distinguishing steroid sensitive from resistant individuals, with an AUC of 0.79.
KIDNEY INTERNATIONAL REPORTS
(2023)
Article
Pediatrics
Ellen Go, Mira van Veenendaal, Cedric Manlhiot, Rayfel Schneider, Brian W. McCrindle, Rae S. M. Yeung
Summary: This study describes the disease course of a small proportion of patients with both Kawasaki disease and systemic juvenile idiopathic arthritis, characterized by refractory Kawasaki disease, high prevalence of coronary artery dilatation, and shared immunopathology potentially linking the two conditions.
FRONTIERS IN PEDIATRICS
(2021)
Article
Medicine, General & Internal
Natalia Chebotareva, Venzsin Cao, Anatoliy Vinogradov, Igor Alentov, Natalia Sergeeva, Alexey Kononikhin, Sergey Moiseev
Summary: This study evaluated the differences in the levels of anti-CD40 and anti-ubiquitin carboxyl-terminal hydrolase L1 (anti-UCH-L1) antibodies between patients with podocytopathies and other glomerulopathies. The results showed that the levels of anti-CD40 antibodies were higher in patients with minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS), while the levels of anti-UCH-L1 antibodies were higher in MCD patients. These antibodies may serve as potential factors for differential diagnosis and treatment prognosis.
FRONTIERS IN MEDICINE
(2023)
Article
Immunology
Heng Li, Lefeng Wang, Xiayu Li, Wenqing Chen, Ying Zhang, Jianghua Chen
Summary: This study found that a significant proportion of patients with minimal change disease (MCD) had high serum immunoglobulin E (IgE) levels. High serum IgE levels were associated with delayed remission time and increased risk of relapse in MCD patients.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Medicine, General & Internal
Sophia Lionaki, Evangelos Mantios, Ioanna Tsoumbou, Smaragdi Marinaki, George Makris, George Liapis, Chrysovalantis Vergandis, Ioannis Boletis
Summary: The study found that nearly 46% of adult MCD patients experienced at least one relapse, although renal progression was rare. Younger age of onset was identified as an independent risk factor for relapse in adult MCD patients.
JOURNAL OF CLINICAL MEDICINE
(2021)
Article
Urology & Nephrology
Ai-bo Qin, Xiao-juan Yu, Su-xia Wang, Fu-de Zhou, Ming-hui Zhao
Summary: Patients with mercury-associated minimal change disease (M-MCD) have similar clinicopathological features with primary minimal change disease (P-MCD), but with less severe foot process effacement. Mercury detoxification therapy is highly effective for M-MCD patients and can be considered as a primary choice in clinical practice, showing a lower relapse rate compared to patients with P-MCD.
Editorial Material
Urology & Nephrology
Rutger J. Maas, Tom Nijenhuis, Johan van der Vlag
KIDNEY INTERNATIONAL REPORTS
(2022)
Article
Pathology
Meyke Hermsen, Francesco Ciompi, Adeyemi Adefidipe, Aleksandar Denic, Amelie Dendooven, Byron H. Smith, Dominique van Midden, Jan Hinrich Braesen, Jesper Kers, Mark D. Stegall, Peter Bandi, Tri Nguyen, Zaneta Swiderska-Chadaj, Bart Smeets, Luuk B. Hilbrands, Jeroen A. W. M. van der Laak
Summary: This study investigated the potential of convolutional neural networks (CNNs) to quantify inflammation and chronic features in kidney transplant biopsies. CNNs were applied on whole-slide image pairs to quantify various tissue features and showed high correlation with pathologists' scores. The results suggest that CNNs can provide objective quantitative information and improve kidney transplant diagnostics.
AMERICAN JOURNAL OF PATHOLOGY
(2022)
Article
Genetics & Heredity
Yaoxian Xu, Christoph Kuppe, Javier Perales-Paton, Sikander Hayat, Jennifer Kranz, Ali T. Abdallah, James Nagai, Zhijian Li, Fabian Peisker, Turgay Saritas, Maurice Halder, Sylvia Menzel, Konrad Hoeft, Annegien Kenter, Hyojin Kim, Claudia R. C. van Roeyen, Michael Lehrke, Julia Moellmann, Thimoteus Speer, Eva M. Buhl, Remco Hoogenboezem, Peter Boor, Jitske Jansen, Cordula Knopp, Ingo Kurth, Bart Smeets, Eric Bindels, Marlies E. J. Reinders, Carla Baan, Joost Gribnau, Ewout J. Hoorn, Joachim Steffens, Tobias B. Huber, Ivan Costa, Jurgen Floege, Rebekka K. Schneider, Julio Saez-Rodriguez, Benjamin S. Freedman, Rafael Kramann
Summary: Adult kidney organoids, known as tubuloids, are derived from a distinct CD24(+) epithelial subpopulation and can be used to model autosomal dominant polycystic kidney disease (ADPKD) and study drug response. The study found similarities in gene expression between gene-edited tubuloids and ADPKD patients' tissue, demonstrating the potential of tubuloids in ADPKD disease modeling. Additionally, the approved drug for ADPKD, tolvaptan, was found to have a significant effect on cyst size in tubuloids but not in pluripotent stem cell-derived models.
Article
Transplantation
Susan T. Veissi, Bart Smeets, Joanna A. E. van Wijk, Thea van der Velden, Lambertus P. W. J. van den Heuvel, Michiel F. Schreuder
NEPHROLOGY DIALYSIS TRANSPLANTATION
(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)
Article
Oncology
Jennifer Eymael, Martijn van den Broek, Laura Miesen, Valerie Villacorta Monge, Bartholomeus T. van den Berge, Fieke Mooren, Vicky Luna Velez, Jelmer Dijkstra, Meyke Hermsen, Peter Bandi, Michiel Vermeulen, Saskia de Wildt, Brigith Willemsen, Sandrine Florquin, Roy Wetzels, Eric Steenbergen, Rafael Kramann, Marcus Moeller, Michiel F. Schreuder, Jack Fm Wetzels, Johan van der Vlag, Jitske Jansen, Bart Smeets
Summary: Scattered tubular cells (STCs) in the proximal tubule increase in number after acute kidney injury. STCs are preferentially located within the inner bends of the tubule and their number increases with age. STCs represent a transient state of dedifferentiated proximal tubule epithelial cells (PTECs) showing metabolic shift towards glycolysis, facilitating cellular survival after kidney injury.
JOURNAL OF PATHOLOGY
(2023)
Article
Urology & Nephrology
Ting Jia, Tong Xu, Bart Smeets, Eva Miriam Buhl, Marcus Johannes Moeller, Juergen Floege, Barbara Mara Klinkhammer, Peter Boor
Summary: The study suggests that PDGF-B and its receptor PDGFR-beta play a crucial role in the development and progression of FSGS by affecting the activation, proliferation, and fibrotic characteristics of PECs. The molecular crosstalk between podocytes and PECs mediated by PDGF-B and PDGFR-beta drives glomerulosclerosis and the progression of FSGS. Treatment with PDGF-B neutralizing antibody can improve kidney function and reduce the occurrence of FSGS.
JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY
(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
Urology & Nephrology
Maartje F. A. Verploegen, Marye J. Boers-Sonderen, Berber Piet, Jack F. M. Wetzels
Summary: Kidney injury in patients treated with immune checkpoint inhibitors develops gradually and may not meet the criteria for acute kidney injury. Proper classification of kidney injury can prevent the progression of chronic kidney disease and improve ongoing oncologic treatment.
Meeting Abstract
Biochemistry & Molecular Biology
Steven Podos, Carla Nester, Gerald Appel, Andrew Bomback, Koenraad Peter Bouman, Terry Cook, Erica Daina, Bradley Dixon, Craig Langman, Liz Lightstone, Jane Thanassi, Kara Rice, Mingjun Huang, Samir V. Parikh, Matthew Pickering, John Sperati, Howard Trachtman, James Tumlin, Jack Wetzels, Giuseppe Remuzzi
MOLECULAR IMMUNOLOGY
(2022)
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)