Federica Casiraghi, Pamela Yossenaidy Rodriguez Ordonez, Nadia Azzollini, Marta Todeschini, Daniela Rottoli, Roberta Donadelli, Roberto Gramignoli, Ariela Benigni, Marina Noris, Giuseppe Remuzzi
Complement factor H (FH) is the main plasma regulator of the alternative pathway of complement. Genetic and acquired abnormalities in FH cause uncontrolled complement activation amplifying, with the consequent accumulation of complement components on the renal glomeruli. This leads to conditions such as C3 glomerulopathy (C3G) and atypical hemolytic uremic syndrome (aHUS). There is no effective therapy for these diseases. Half of the patients progress to end-stage renal disease and the condition recurs frequently in transplanted kidneys...
June 10, 2021: Stem Cell Research & Therapy
Małgorzata Kielar, Agnieszka Gala-Błądzińska, Paulina Dumnicka, Piotr Ceranowicz, Maria Kapusta, Beata Naumnik, Grzegorz Kubiak, Marek Kuźniewski, Beata Kuśnierz-Cabala
Currently, kidney transplantation is widely accepted as the renal replacement therapy allowing for the best quality of life and longest survival of patients developing end-stage renal disease. However, chronic transplant rejection, recurrence of previous kidney disease or newly acquired conditions, or immunosuppressive drug toxicity often lead to a deterioration of kidney allograft function over time. Complement components play an important role in the pathogenesis of kidney allograft impairment. Most studies on the role of complement in kidney graft function focus on humoral rejection; however, complement has also been associated with cell mediated rejection, post-transplant thrombotic microangiopathy, the recurrence of several glomerulopathies in the transplanted kidney, and transplant tolerance...
May 21, 2021: Biomolecules
Clara García-Carro, Ander Vergara, Sheila Bermejo, María A Azancot, Joana Sellarés, Maria José Soler
Obesity is one of the epidemics of our era. Its prevalence is higher than 30% in the U.S. and it is estimated to increase by 50% in 2030. Obesity is associated with a higher risk of all-cause mortality and it is known to be a cause of chronic kidney disease (CKD). Typically, obesity-related glomerulopathy (ORG) is ascribed to renal hemodynamic changes that lead to hyperfiltration, albuminuria and, finally, impairment in glomerular filtration rate due to glomerulosclerosis. Though not only hemodynamics are responsible for ORG: adipokines could cause local effects on mesangial and tubular cells and podocytes promoting maladaptive responses to hyperfiltration...
2021: Frontiers in Medicine
Fernando Caravaca-Fontán, Hernando Trujillo, Francisco Caravaca, Manuel Praga
No abstract text is available yet for this article.
April 29, 2021: Nephrology, Dialysis, Transplantation
Timothée Laboux, Jean-Baptiste Gibier, Nicolas Pottier, François Glowacki, Aghilès Hamroun
No abstract text is available yet for this article.
April 2021: Journal of Nephrology
Fernando Caravaca-Fontán, Montserrat Díaz-Encarnación, Virginia Cabello, Gema Ariceta, Luis F Quintana, Helena Marco, Xoana Barros, Natalia Ramos, Nuria Rodríguez-Mendiola, Sonia Cruz, Gema Fernández-Juárez, Adela Rodríguez, Ana Pérez de José, Cristina Rabasco, Raquel Rodado, Loreto Fernández, Vanessa Pérez Gómez, Ana Ávila, Luis Bravo, Natalia Espinosa, Natalia Allende, Maria Dolores Sanchez de la Nieta, Eva Rodríguez, Teresa Olea, Marta Melgosa, Ana Huerta, Rosa Miquel, Carmen Mon, Gloria Fraga, Alberto de Lorenzo, Juliana Draibe, Marta Cano-Megías, Fayna González, Amir Shabaka, Maria Esperanza López-Rubio, María Ángeles Fenollosa, Luis Martín-Penagos, Iara Da Silva, Juana Alonso Titos, Santiago Rodríguez de Córdoba, Elena Goicoechea de Jorge, Manuel Praga
INTRODUCTION: The association between a change in proteinuria over time and its impact in kidney prognosis has not been analyzed in C3 glomerulopathy. This study aims to investigate the association between the longitudinal change in proteinuria and the risk of kidney failure. METHODS: Retrospective, multicenter observational cohort study in 35 nephrology departments belonging to the Spanish Group for the Study of Glomerular Diseases (GLOSEN). Patients diagnosed with C3 glomerulopathy between 1995 and 2020 were enrolled...
March 29, 2021: Nephrology, Dialysis, Transplantation
Emma Levenson, Tara N Shepherd, Diego Aviles, Randall Craver, Abdulla Ehlayel, Gordon L Love, K'Joy Simms, Caroline Straatmann, Isa F Ashoor
The negative impact of COVID-19 on adults with underlying chronic kidney disease, including kidney transplant recipients, has been well documented. Children have a less severe presentation and better prognosis compared to adults. However, little is known regarding the spectrum of COVID-19 infection in children and adolescents with underlying autoimmune disorders necessitating solid organ transplant and long-term immunosuppressive therapy. Case Report. An adolescent male developed end-stage kidney disease secondary to microscopic polyangiitis requiring a living-donor kidney transplant...
March 27, 2021: Pediatric Transplantation
Xiao-Jie Peng, Wei-Min Zheng, Rui Fu, Yu-Hui Huang, Mei-Hui Deng, Shan-Shan Tao, Ting-Jie Wang, Chunhui Zhu
AIM: IgA nephropathy is virtually known as the most common glomerulopathy to end-stage renal failure in the world. Mycophenolate mofetil is a selective immunosuppressant widely used in organ transplantation, yet its tolerance and effectiveness in IgAN is controversial. METHODS: This is a systematic review and random-effects meta-analysis, searching PubMed, Embase, Te Cochrane Library, Science Citation Index, Ovid evidence-based medicine, Chinese Biomedical Literature and Chinese Science and Technology Periodicals...
July 2021: Clinical and Experimental Nephrology
B Karthikeyan, M Edwin Fernando, N D Srinivasaprasad, S Sujit, K Thirumal Valavan, Anila A Kurien
Introduction: Collapsing glomerulopathy (CG) is a distinct morphologic pattern of proliferative renal parenchymal injury. It differ from focal segmental glomerulosclerosis (FSGS) by clinicopathologic pattern and its adverse outcome. The clinical significance of CG in renal allograft biopsies is not yet clear due to scant data and less occurrence of CG in renal transplant recipients. We conducted this single-center retrospective study to evaluate the prevalence, clinicopathological features, and outcome of post renal transplant CG...
September 2020: Indian Journal of Nephrology
Aleksandar Senev, Elisabet Van Loon, Evelyne Lerut, Jasper Callemeyn, Maarten Coemans, Vicky Van Sandt, Dirk Kuypers, Marie-Paule Emonds, Maarten Naesens
Transplant glomerulopathy is established as a hallmark of chronic antibody-mediated rejection in kidney transplant patients with donor-specific HLA antibodies (HLA-DSA). The clinical importance of transplant glomerulopathy in the absence of HLA-DSA is not well established. To help define this, 954 patients (encompassing 3744 biopsies) who underwent kidney transplantation 2004-2013 were studied with retrospective high-resolution HLA genotyping of both donors and recipients. The risk factors, histopathological appearance and prognosis of cases with transplant glomerulopathy in the absence of HLA-DSA were compared to those cases with HLA-DSA, and the impact of the PIRCHE-II score and eplet mismatches on development of transplant glomerulopathy evaluated...
March 3, 2021: Kidney International
Yassamine Bentata
Parvovirus B19 infection is s new viral threat in post-kidney transplantation. It is a viral infection often acquired from the donor, occurring in young transplant patients during the first post-kidney transplantation months, and with a non-specific clinico-biological picture. The hallmark symptom is regenerative anaemia which may be severe, requiring blood transfusion. The C3 and C4 complement fractions are reduced and constitute an early and inexpensive diagnostic marker. Diagnosis is often delayed due to the non-specific clinico-biological picture...
February 27, 2021: Infectious Diseases
Conxita Jacobs-Cachá, Ander Vergara, Clara García-Carro, Irene Agraz, Nestor Toapanta-Gaibor, Gema Ariceta, Francesc Moreso, Daniel Serón, Joan López-Hellín, Maria José Soler
Primary or idiopathic focal segmental glomerulosclerosis (FSGS) is a kidney entity that involves the podocytes, leading to heavy proteinuria and in many cases progresses to end-stage renal disease. Idiopathic FSGS has a bad prognosis, as it involves young individuals who, in a considerably high proportion (∼15%), are resistant to corticosteroids and other immunosuppressive treatments as well. Moreover, the disease recurs in 30-50% of patients after kidney transplantation, leading to graft function impairment...
February 2021: Clinical Kidney Journal
Christof Aigner, Martina Gaggl, Gunar Stemer, Michael Eder, Georg Böhmig, Renate Kain, Zoltán Prohászka, Nóra Garam, Dorottya Csuka, Raute Sunder-Plassmann, Leah Charlotte Piggott, Natalja Haninger-Vacariu, Alice Schmidt, Gere Sunder-Plassmann
BACKGROUND: Practice patterns of eculizumab use are not well described. We examined indications for, and outcomes of, eculizumab therapy in a tertiary care nephrology center. METHODS: We used the "Vienna TMA cohort" and the hospital pharmacy database at the Medical University of Vienna to identify patients that received eculizumab treatment between 2012 and 2019. We describe clinical characteristics, details of eculizumab use, and outcomes of patients with complement gene-variant mediated TMA (cTMA), secondary TMA (sTMA) and C3 glomerulopathy (C3G)...
February 18, 2021: Journal of Nephrology
Sophie Ferlicot, Matthieu Jamme, François Gaillard, Julie Oniszczuk, Aymeric Couturier, Olivia May, Anne Grünenwald, Aurélie Sannier, Anissa Moktefi, Ophélie Le Monnier, Camille Petit-Hoang, Nadine Maroun, Albane Brodin-Sartorius, Arthur Michon, Hélène Dobosziewicz, Fabrizio Andreelli, Matthieu Guillet, Hassane Izzedine, Christian Richard, Manon Dekeyser, Romain Arrestier, Thomas Sthelé, Edouard Lefèvre, Alexis Mathian, Christophe Legendre, Charlotte Mussini, Marie-Christine Verpont, Nicolas Pallet, Zahir Amoura, Marie Essig, Renaud Snanoudj, Isabelle Brocheriou-Spelle, Hélène François, Xavier Belenfant, Guillaume Geri, Eric Daugas, Vincent Audard, David Buob, Ziad A Massy, Mohamad Zaidan
We report a multicentric retrospective case series of patients with COVID-19 who developed acute kidney injury and/or proteinuria and underwent a kidney biopsy in the Paris and its metropolitan area. Forty-seven patients (80.9% men) with COVID-19 who underwent a kidney biopsy between March 08 and May 19, 2020 were included. Median age was 63 years IQR [52-69]. Comorbidities included hypertension (66.0%), diabetes mellitus (27.7%), obesity (27.7%), history of chronic kidney (25.5%), cardiac (38.6%) and respiratory (27...
February 12, 2021: Nephrology, Dialysis, Transplantation
Ashwani Kumar, Raja Ramachandran, Amit Rawat, Reena Das, Charan S Rayat, Deepesh B Kenwar, Ashish Sharma, Krishan L Gupta, Ritambhra Nada
Background: Complement 3 glomerulopathy (C3G) results from dysfunction of the alternative complement pathway (ACP). No data are available on post-transplant C3G in South Asia. Methods: In this study, renal allograft biopsies of C3G patients performed from 2012 to 2017 were analysed for ACP functional assay (APFA), serum complement levels, complement factor H (CFH), complement factor B (CFB) and autoantibodies to CFH and CFB. Limited genetic screening for CFH / CFHR5 genes was carried out...
January 2021: Clinical Kidney Journal
Andrea Domingo-Gallego, Marc Pybus, Gemma Bullich, Mónica Furlano, Laia Ejarque-Vila, Laura Lorente-Grandoso, Patricia Ruiz, Gloria Fraga, Mercedes López González, Juan Alberto Piñero-Fernández, Lidia Rodríguez-Peña, Isabel Llano-Rivas, Raquel Sáez, Anna Bujons-Tur, Gema Ariceta, Guirado Lluis, Roser Torra, Elisabet Ars
BACKGROUND: Inherited kidney diseases are one of the leading causes of chronic kidney disease (CKD) that manifests before the age of 30 years. Precise clinical diagnosis of early-onset CKD is complicated due to the high phenotypic overlap, but genetic testing is a powerful diagnostic tool. We aimed to develop a genetic testing strategy to maximize the diagnostic yield for patients presenting with early-onset CKD and to determine the prevalence of the main causative genes. METHODS: We performed genetic testing of 460 patients with early-onset CKD of suspected monogenic cause using next-generation sequencing of a custom-designed kidney disease gene panel in addition to targeted screening for c...
February 3, 2021: Nephrology, Dialysis, Transplantation
Zhifeng Xu, Lu Chen, Huiling Xiang, Chun Zhang, Jing Xiong
Background: Membranous nephropathy (MN), a major cause of nephrotic syndrome, has attracted people's attention in recent years for its growing prevalence. It is the second or third leading cause of ESRD in patients with primary glomerulonephritis and is the leading glomerulopathy that recurs after kidney transplantation. Summary: MN can be classified as idiopathic membranous nephropathy (IMN) and secondary MN. The discovery of the M-type phospholipase A2 receptor (PLA2R) and thrombospondin type-1 domain-containing 7A (THSD7A) provides the new diagnostic methods and treatment strategies for IMN on the molecular level...
September 2020: Kidney Diseases
Avantee Gokhale, Jorge Chancay, Ron Shapiro, Parmjeet Randhawa, Madhav C Menon
There have been recent significant advances in short-term outcomes in renal transplantation, however, long-term allograft survival remains a challenge. With reported incidences as high of 74.5% of chronic graft loss in patients with biopsies showing transplant glomerulopathy (TG), this syndrome represents an important factor for chronic allograft complications. In this review we show an overview of the novel mechanistic insights into pathogenesis of TG, as well as a brief description of the pathology, diagnosis and newer prognostic indices within TG diagnosis...
March 2021: Clinical Transplantation
Francois Gougeon, Harsharan K Singh, Volker Nickeleit
BACKGROUND: Collapsing focal segmental glomerulosclerosis (FSGS) has various underlying etiologies and often leads to renal failure. The impact of biopsy-proven renal comorbidities in promoting collapsing glomerulopathy (CG) has not been systematically evaluated in large comparative studies. Those data are reported here. METHODS: Biopsies with the initial diagnosis of CG in native (n = 321) or transplant kidneys (n = 30) were identified in the University of North Carolina nephropathology database (1 January 2011 to 1 January 2016)...
December 28, 2020: Nephrology, Dialysis, Transplantation
Matthew R D'Costa, Andrew Bentall, Aleksandar Denic, Carrie A Schinstock, Massini A Merzkani, Walter D Park, Margaret S Ryan, Mariam P Alexander, Byron H Smith, Manish J Gandhi, Mark D Stegall
BACKGROUND: At 5 and 10 years after kidney transplantation, chronic histologic changes such as arteriolar hyalinosis and mesangial expansion are common, however, determining etiology is difficult. We compared surveillance biopsies in living donor kidney transplants (LDKTx) from HLA matched siblings (termed HLA-identical (HLA-ID)) to HLA non-ID to investigate which histologic changes were likely due to alloimmune injury and which were due to non-alloimmune injury. METHODS: We performed a retrospective, cohort study comparing HLA-ID sibling LDKTx (n=175) to HLA non-ID LDKTx (n=175; matched for age, sex and year of transplant +/- 2 years) performed at a single institution from 03/1999 to 11/2018...
December 10, 2020: Transplantation
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