Article
Urology & Nephrology
Caroline Duineveld, Romy N. Bouwmeester, Kioa L. Wijnsma, F. J. Bemelman, J. W. van der Heijden, S. P. Berger, L. P. W. J. van den Heuvel, Nicole C. A. J. van de Kar, Jack F. M. Wetzels
Summary: Kidney transplantation in aHUS patients in the Netherlands is performed without eculizumab prophylaxis, and eculizumab therapy is given in case of posttransplant aHUS recurrence. The study found a recurrence rate of 23% without eculizumab prophylaxis, and eculizumab treatment effectively improved or stabilized kidney function in most patients.
KIDNEY INTERNATIONAL REPORTS
(2023)
Article
Urology & Nephrology
Mendy ter Avest, Hilbert Steenbreker, Romy N. Bouwmeester, Caroline Duineveld, Kioa L. Wijnsma, Lambertus P. W. J. van den Heuvel, Saskia M. C. Langemeijer, Jack F. M. Wetzels, Nicole C. A. J. van de Kar, Rob ter Heine, CUREiHUS Study Grp
Summary: This study investigated the effect of proteinuria on the pharmacokinetics of eculizumab. The findings suggest that patients with severe proteinuria have a higher risk of underexposure to eculizumab.
CLINICAL JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY
(2023)
Article
Urology & Nephrology
Gema Ariceta, Bradley P. Dixon, Seong Heon Kim, Gaurav Kapur, Teri Mauch, Stephan Ortiz, Marc Vallee, Andrew E. Denker, Hee Gyung Kang, Larry A. Greenbaum
Summary: Ravulizumab rapidly improved hematologic and kidney parameters in complement inhibitor-naive children with atypical hemolytic uremic syndrome, showing no unexpected safety concerns.
KIDNEY INTERNATIONAL
(2021)
Article
Urology & Nephrology
Gianluigi Ardissino, Donata Cresseri, Francesca Tel, Antenore Giussani, Stefania Salardi, Martina Sgarbanti, Bice Strumbo, Sara Testa, Valentina Capone, Samantha Griffini, Elena Grovetti, Massimo Cugno, Mirco Belingheri, Chiara Tamburello, Evangeline Millicent Rodrigues, Michela Perrone, Massimo Cardillo, Grazia Corti, Dario Consonni, Lucrezia Furian, Silvana Tedeschi, Piergiorgio Messa, Claudio Beretta
Summary: This study presents experience with KTx in aHUS patients, showing that prophylactic use of Eculizumab can significantly reduce relapse rates. Tailored timing of Eculizumab doses based on classical complement pathway activity for each patient has also shown positive results.
JOURNAL OF NEPHROLOGY
(2021)
Article
Immunology
Cristina Casas Gonzalez, Veronica Lopez-Jimenez, Teresa Vazquez-Sanchez, Elena Vazquez-Sanchez, Mercedes Cabello, Domingo Hernandez-Marrero
Summary: The atypical hemolytic-uremic syndrome (aHUS) is a disease characterized by non-immune hemolytic anemia, thrombocytopenia, and acute renal failure. The use of eculizumab has improved the prognosis of aHUS and prevented recurrence after kidney transplantation.
TRANSPLANTATION PROCEEDINGS
(2022)
Article
Urology & Nephrology
Massimo Cugno, Valentina Capone, Samantha Griffini, Elena Grovetti, Giulia Pintarelli, Luigi Porcaro, Emilio Clementi, Gianluigi Ardissino
Summary: This study reports on the experience with eculizumab maintenance treatment in patients with aHUS. The interval between eculizumab doses was adjusted based on CCP activity, with the goal of maintaining CCP activity below 30% to prevent relapse. Patients who received eculizumab regularly at tailored intervals showed no relapse during the observation period, and there was an inverse correlation between CCP activity and eculizumab circulating levels.
JOURNAL OF NEPHROLOGY
(2022)
Article
Urology & Nephrology
Esra Baskin, Kibriya Fidan, Bora Gulhan, Kaan Gulleroglu, Nur Canpolat, Alev Yilmaz, Gonul Parmakiz, Birsin Z. Ozcakar, Fatih Ozaltin, Oguz Soylemezoglu
Summary: This study retrospectively analyzed the outcome of pediatric patients with aHUS who discontinued eculizumab treatment. The study found that eculizumab can be safely discontinued with close monitoring in a selected group of patients, and if recurrence occurs, restarting eculizumab immediately can achieve complete remission.
JOURNAL OF NEPHROLOGY
(2022)
Article
Urology & Nephrology
Jean-Michel Halimi, Imad Al-Dakkak, Katerina Anokhina, Gianluigi Ardissino, Christoph Licht, Wai H. Lim, Annick Massart, Franz Schaefer, Johan Vande Walle, Eric Rondeau
Summary: This analysis of the Global aHUS Registry provides further insights into the potential relationship between aHUS and malignant hypertension. The study finds that patients with aHUS and malignant hypertension have a higher need for renal replacement therapy and a higher prevalence of pathogenic variants/anti-complement factor H antibodies. Additionally, the study suggests that eculizumab is effective in treating aHUS with or without malignant hypertension.
JOURNAL OF NEPHROLOGY
(2023)
Article
Urology & Nephrology
Miguel Ernandes Neto, Lucas de Moraes Soler, Halita Vieira Gallindo Vasconcelos, Hong Si Nga, Ariane Moyses Bravin, Julio Cesar Andriotti Borges, Rodrigo Costa Goncalves, Rodrigo Brum Von Kriiger, Raquel Martins Quinino, Viviane Brandao Bandeira de Mello Santana, Maria Izabel de Holanda, Maria Helena Vaisbich, Alice Pignaton Naseri, Gianna Mastroianni Kirsztajn, Lilian Monteiro Pereira Palma, Luis Gustavo Modelli Andrade
Summary: This study analyzed the risk of aHUS relapse in Brazilian patients after eculizumab interruption due to drug shortage. It was found that the cumulative incidence of aHUS relapse at 397 days was 58%, and baseline variables were not associated with relapse rate. The interruption of eculizumab therapy was deemed unsafe due to the high rate of relapse.
JOURNAL OF NEPHROLOGY
(2021)
Article
Urology & Nephrology
Maria Helena Vaisbich, Luis Gustavo Modelli de Andrade, Precil Diego Miranda de Menezes Neves, Lilian Monteiro Pereira Palma, Maria Cristina Ribeiro de Castro, Cassiano Augusto Braga Silva, Maria Izabel Neves de Holanda Barbosa, Maria Goretti Moreira Guimaraes Penido, Oreste Angelo Ferra Neto, Roberta Mendes Lima Sobral, Silvana Maria Carvalho Miranda, Stanley de Almeida Araujo, Igor Gouveia Pietrobom, Henrique Mochida Takase, Claudia Ribeiro, Rafael Marques da Silva, Cesar Augusto Almeida de Carvalho, David Jose Barros Machado, Ana Mateus Simoes Teixeira E Silva, Andreia Ribeiro da Silva, Enzo Ricardo Russo, Flavio Henrique Soares Barros, Jarinne Camilo Landim Nasserala, Luciana Schmitt Cardon de Oliveira, Lucimary de Castro Sylvestre, Rafael Weissheimer, Sueli Oliveira Nascimento, Gilson Bianchini, Fellype de Carvalho Barreto, Valeria Soares Pigozzi Veloso, Patricia Marques Fortes, Vinicius Sardao Colares, Jaelson Guilhem Gomes, Andre Falcao Pedrosa Leite, Pablo Girardelli Mendonca Mesquita, Osvaldo Merege Vieira-Neto
Summary: This study has analyzed the first Brazilian registry for atypical hemolytic uremic syndrome (aHUS), revealing that the disease predominantly affects young adult women with renal involvement. Pediatric patients have lower hemoglobin and platelet levels and higher LDH levels compared to adults. The most common pathogenic variants were found in CFH and the CFHR1-3 deletion.
CLINICAL KIDNEY JOURNAL
(2022)
Article
Urology & Nephrology
Lilian Monteiro Pereira Palma, Renato George Eick, Gustavo Coelho Dantas, Michele Karen dos Santos Tino, Maria Izabel de Holanda
Summary: This study is the largest case series of aHUS in Brazil involving a wide range of patients for which eculizumab was the main treatment. Although eculizumab was started later than advised in the guidelines, most patients were able to stop dialysis at variable intervals. Discontinuation of eculizumab was associated with a 30% relapse of aHUS.
CLINICAL KIDNEY JOURNAL
(2021)
Article
Pediatrics
Kazuki Tanaka, Brigitte Adams, Alvaro Madrid Aris, Naoya Fujita, Masayo Ogawa, Stephan Ortiz, Marc Vallee, Larry A. Greenbaum
Summary: The study demonstrated that pediatric patients with aHUS who switched from chronic eculizumab to ravulizumab treatment experienced stable kidney and hematologic parameters, with low occurrence of adverse events.
PEDIATRIC NEPHROLOGY
(2021)
Article
Immunology
Shirley Pollack, Israel Eisenstein, Adi Mory, Tamar Paperna, Ayala Ofir, Hagit Baris-Feldman, Karin Weiss, Nora Veszeli, Dorottya Csuka, Revital Shemer, Fabian Glaser, Zoltan Prohaszka, Daniella Magen
Summary: The study identified a rare novel homozygous activating deletion in the C3 gene associated with atypical hemolytic uremic syndrome (aHUS), highlighting the critical role of the disrupted C3-TED domain in the disease mechanism.
FRONTIERS IN IMMUNOLOGY
(2021)
Article
Pediatrics
Melissa Muff-Luett, Keia R. Sanderson, Rachel M. Engen, Rima S. Zahr, Scott E. Wenderfer, Cheryl L. Tran, Sheena Sharma, Yi Cai, Susan Ingraham, Erica Winnicki, Donald J. Weaver, Tracy E. Hunley, Stefan G. Kiessling, Meredith Seamon, Robert Woroniecki, Yosuke Miyashita, Nianzhou Xiao, Abiodun A. Omoloja, Sarah J. Kizilbash, Asif Mansuri, Mahmoud Kallash, Yichun Yu, Ashley K. Sherman, Tarak Srivastava, Carla M. Nester
Summary: This study investigated the broader use and outcomes of pediatric patients exposed to eculizumab, revealing a significant number of patients receiving off-label indications for C5 blockade. Although adverse events related to the medication were low, the cohort mortality rate was relatively high. Prospective studies in homogeneous disease cohorts are needed to confirm the role of C5 blockade in kidney outcomes.
PEDIATRIC NEPHROLOGY
(2021)
Review
Urology & Nephrology
Kultigin Turkmen, Ismail Baloglu, Hakan Ozer
Summary: The complement system is crucial in the pathogenesis of C3 glomerulopathy (C3GP) and atypical hemolytic uremic syndrome (aHUS), with recent research suggesting common abnormalities in the control of the alternative complement system between the two diseases. This overlap may impact treatment strategies for both conditions.
INTERNATIONAL UROLOGY AND NEPHROLOGY
(2021)
Review
Rheumatology
Samuel Deshayes, Nicolas Martin Silva, Valerie Chatelet, Sylvain Chantepie, Moglie Le Quintrec, Francois Comoz, Frank Bridoux, Marie-Agnes Dragon-Durey, Achille Aouba
CLINICAL RHEUMATOLOGY
(2018)
Article
Oncology
Jacques Dantal, Emmanuel Morelon, Lionel Rostaing, Eric Goffin, Anabelle Brocard, Isabelle Tromme, Nilufer Broeders, Veronique del Marmol, Valerie Chatelet, Anne Dompmartin, Michele Kessler, Andreas Serra, Gunther F. L. Hofbauer, Nassim Kamar, Claire Pouteil-Noble, Jean Kanitakis, Adeline Roux, Evelyne Decullier, Sylvie Euvrard
JOURNAL OF CLINICAL ONCOLOGY
(2018)
Article
Urology & Nephrology
Valerie Chatelet, Thierry Lobbedez, Jerome Harambat, Sahar Bayat-Makoei, Francois Glowacki, Cecile Vigneau
NEPHROLOGIE & THERAPEUTIQUE
(2018)
Article
Surgery
Valerie Chatelet, Philippe Gatault, Marc Hazzan, Dany Anglicheau, Guy Launoy, Thierry Lobbedez, Bruno Moulin
TRANSPLANT INTERNATIONAL
(2019)
Article
Urology & Nephrology
Benedicte Driollet, Florian Bayer, Valerie Chatelet, Marie-Alice Macher, Remi Salomon, Bruno Ranchin, Gwenaelle Roussey, Annie Lahoche, Florentine Garaix, Stephane Decramer, Elodie Merieau, Marc Fila, Ariane Zaloszyc, Georges Deschenes, Linda Valeri, Ludivine Launay, Cecile Couchoud, Karen Leffondre, Jerome Harambat
KIDNEY INTERNATIONAL
(2019)
Article
Hematology
Renaud Prevel, Claire Roubaud-Baudron, Samuel Gourlain, Matthieu Jamme, Karine Peres, Ygal Benhamou, Lionel Galicier, Elie Azoulay, Pascale Poullin, Francois Provot, Eric Maury, Claire Presne, Mohamed Hamidou, Samir Saheb, Alain Wynckel, Aude Servais, Stephane Girault, Yahsou Delmas, Valerie Chatelet, Jean-Francois Augusto, Christiane Mousson, Pierre Perez, Jean-Michel Halimi, Tarik Kanouni, Alexandre Lautrette, Anne Charvet-Rumpler, Christophe Deligny, Dominique Chauveau, Agnes Veyradier, Paul Coppo, Augusto Jean-Francois, Azoulay Elie, Barbay Virginie, Benhamou Ygal, Bordessoule Dominique, Charasse Christophe, Charvet-Rumpler Anne, Chauveau Dominique, Choukroun Gabriel, Coindre Jean-Philippe, Coppo Paul, Corre Elise, Delmas Yahsou, Deschenes Georges, Devidas Alain, Dossier Antoine, Fain Olivier, Fakhouri Fadi, Fremeaux-Bacchi Veronique, Galicier Lionel, Grange Steven, Guidet Bertrand, Halimi Jean-Michel, Hamidou Mohamed, Herbrecht Raoul, Hie Miguel, Jacobs Frederic, Joly Berangere, Kanouni Tarik, Kaplanski Gilles, Lautrette Alexandre, Le Guern Veronique, Loirat Chantal, Moulin Bruno, Mousson Christiane, Ojeda Uribe Mario, Ouchenir Abdelkader, Parquet Nathalie, Peltier Julie, Pene Frederic, Perez Pierre, Poullin Pascale, Pouteil-Noble Claire, Presne Claire, Provot Francois, Rondeau Eric, Saheb Samir, Schlemmer Benoit, Seguin Amelie, Servais Aude, Stepanian Alain, Vernant Jean-Paul, Veyradier Agnes, Vigneau Cecile, Wynckel Alain, Zunic Patricia
Article
Urology & Nephrology
Julien Zuber, Marie Frimat, Sophie Caillard, Nassim Kamar, Philippe Gatault, Florent Petitprez, Lionel Couzi, Noemie Jourde-Chiche, Valerie Chatelet, Raphael Gaisne, Dominique Bertrand, Jamal Bamoulid, Magali Louis, Rebecca Sberro Soussan, David Navarro, Pierre-Francois Westeel, Luc Frimat, Charlotte Colosio, Antoine Thierry, Joseph Rivalan, Laetitia Albano, Nadia Arzouk, Emilie Cornec-Le Gall, Guillaume Claisse, Michelle Elias, Khalil El Karoui, Sophie Chauvet, Jean-Philippe Coindre, Jean-Philippe Rerolle, Leila Tricot, Johnny Sayegh, Cyril Garrouste, Christophe Charasse, Yahsou Delmas, Ziad Massy, Maryvonne Hourmant, Aude Servais, Chantal Loirat, Fadi Fakhouri, Claire Pouteil-Noble, Marie-Noelle Peraldi, Christophe Legendre, Eric Rondeau, Moglie Le Quintrec, Veronique Fremeaux-Bacchi
JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY
(2019)
Review
Urology & Nephrology
Angelique Thuillier Lecouf, Ludivine Videloup, Sandrine Letrecher, Clemence Bechade, Marie Recorbet, Valerie Chatelet
NEPHROLOGIE & THERAPEUTIQUE
(2020)
Article
Transplantation
Mathilde Beaumier, Clemence Bechade, Olivier Dejardin, Mathilde Lassalle, Cecile Vigneau, Nathalie Longlune, Ludivine Launay, Cecile Couchoud, Maxence Ficheux, Thierry Lobbedez, Valerie Chatelet
NEPHROLOGY DIALYSIS TRANSPLANTATION
(2020)
Editorial Material
Urology & Nephrology
Maryvonne Hourmant, Valerie Chatelet
NEPHROLOGIE & THERAPEUTIQUE
(2020)
Article
Surgery
Elodie Bailly, Simon Ville, Gilles Blancho, Emmanuel Morelon, Jamal Bamoulid, Sophie Caillard, Valerie Chatelet, Paolo Malvezzi, Jerome Tourret, Vincent Vuiblet, Dany Anglicheau, Dominique Bertrand, Philippe Grimbert, Fadi Haidar, Marc Hazzan, Nassim Kamar, Pierre Merville, Christiane Mousson, Vincent Pernin, Claire Pouteil-Noble, Raj Purgus, Johnny Sayegh, Pierre-Francois Westeel, Benedicte Sautenet, Philippe Gatault, Matthias Buchler
TRANSPLANT INTERNATIONAL
(2020)
Article
Endocrinology & Metabolism
Helene Lazareth, Neila Talbi, Nassim Kamar, Charlene Levi, Bruno Moulin, Sophie Caillard, Luc Frimat, Jonathan Chemouny, Valerie Chatelet, Clement Vachey, Renaud Snanoudj, Thibaud Lefebvre, Alexandre Karras, Laurent Gouya, Caroline Schmitt, Herve Puy, Nicolas Pallet
MOLECULAR GENETICS AND METABOLISM
(2020)
Article
Environmental Sciences
Latame Adoli, Maxime Raffray, Valerie Chatelet, Cecile Vigneau, Thierry Lobbedez, Fei Gao, Florian Bayer, Arnaud Campeon, Elsa Vabret, Laetitia Laude, Jean-Philippe Jais, Eric Daugas, Cecile Couchoud, Sahar Bayat
Summary: This study aims to understand the potential sex disparities and their causes in kidney transplantation access. It will use both quantitative and qualitative research methods to compare the differences in access to kidney transplantation between women and men at different levels, and explore the views of patients and nephrologists on the disease and transplantation.
INTERNATIONAL JOURNAL OF ENVIRONMENTAL RESEARCH AND PUBLIC HEALTH
(2022)
Meeting Abstract
Surgery
Julien Zuber, Sophie Caillard, Marie Frimat, Nassim Kamar, Philippe Gatault, Valerie Chatelet, Antoine Thierry, Dominique Bertrand, Lionel Couzi, Laetitia Albano, Claire Pouteil-Noble, Marie Noelle Peraldi, Christophe Legendre, Eric Rondeau, Moglie Le Quintrec, Veronique Fremeaux Bacchi
TRANSPLANT INTERNATIONAL
(2019)
Article
Urology & Nephrology
Mathilde Lemoine, Arnaud Francois, Steven Grange, Marion Rabant, Valerie Chatelet, David Cassiman, Emilie Cornec-Le Gall, Damien Ambrosetti, Georges Deschenes, Jean-Francois Benoist, Dominique Guerrot
KIDNEY INTERNATIONAL REPORTS
(2018)