Search Results - "Decramer, Stephane"

Immunoglobulin serum levels in rituximab-treated patients with steroid-dependent nephrotic syndrome by Parmentier, Cyrielle, Delbet, Jean-Daniel, Decramer, Stéphane, Boyer, Olivia, Hogan, Julien, Ulinski, Tim

“…Background Rituximab (RTX) is efficient in steroid-dependent nephrotic syndrome (SDNS) in pediatric and adult patients. The aim of this study is to describe…”
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Consensus recommendations for diagnosis, management and treatment of Fabry disease in paediatric patients by Germain, Dominique P., Fouilhoux, Alain, Decramer, Stéphane, Tardieu, Marine, Pillet, Pascal, Fila, Marc, Rivera, Serge, Deschênes, Georges, Lacombe, Didier

“…Fabry disease (FD), a rare X‐linked disease, can be treated with bi‐monthly infusion of enzyme replacement therapy (ERT) to replace deficient α‐galactosidase A…”
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The HNF1B score is a simple tool to select patients for HNF1B gene analysis by Faguer, Stanislas, Chassaing, Nicolas, Bandin, Flavio, Prouheze, Cathie, Garnier, Arnaud, Casemayou, Audrey, Huart, Antoine, Schanstra, Joost P, Calvas, Patrick, Decramer, Stéphane, Chauveau, Dominique

“…HNF1B-related disease is an emerging condition characterized by an autosomal-dominant inheritance, a 50% rate of de novo mutations, and a highly variable…”
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Comparison of the amniotic fluid and fetal urine peptidome for biomarker discovery in renal developmental disease by Fédou, Camille, Breuil, Benjamin, Golovko, Igor, Decramer, Stéphane, Magalhães, Pedro, Muller, Françoise, Dreux, Sophie, Zürbig, Petra, Klein, Julie, Schanstra, Joost P., Buffin-Meyer, Bénédicte

“…Production of amniotic fluid (AF) is view as predominately driven by excretion of fetal urine (FU). However, the origin of AF peptides, often considered as…”
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Patterns of Clinical Response to Eculizumab in Patients With C3 Glomerulopathy by Le Quintrec, Moglie, Lapeyraque, Anne-Laure, Lionet, Arnaud, Sellier-Leclerc, Anne-Laure, Delmas, Yahsou, Baudouin, Véronique, Daugas, Eric, Decramer, Stéphane, Tricot, Leila, Cailliez, Mathilde, Dubot, Philippe, Servais, Aude, Mourey-Epron, Catherine, Pourcine, Franck, Loirat, Chantal, Frémeaux-Bacchi, Véronique, Fakhouri, Fadi

“…Cases reports and small series of patients with C3 glomerulopathy have reported variable efficacy of eculizumab. Case series of C3 glomerulopathy. Pediatric…”
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Spectrum of HNF1B Mutations in a Large Cohort of Patients Who Harbor Renal Diseases by Heidet, Laurence, Decramer, Stéphane, Pawtowski, Audrey, Morinière, Vincent, Bandin, Flavio, Knebelmann, Bertrand, Lebre, Anne-Sophie, Faguer, Stanislas, Guigonis, Vincent, Antignac, Corinne, Salomon, Rémi

“…Hepatocyte nuclear factor 1beta (HNF1beta) is a transcription factor that is critical for the development of kidney and pancreas. In humans, mutations in HNF1B…”
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Mycophenolic acid area under the concentration-time curve is associated with therapeutic response in childhood-onset lupus nephritis by Godron-Dubrasquet, Astrid, Woillard, Jean-Baptiste, Decramer, Stéphane, Fila, Marc, Guigonis, Vincent, Tellier, Stéphanie, Morin, Denis, Sordet, Maud, Saint-Marcoux, Frank, Harambat, Jérôme

“…Background Mycophenolic acid (MPA), the active compound of mycophenolate mofetil (MMF), is widely used in lupus nephritis treatment. Therapeutic drug…”
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A single-center study to evaluate the efficacy of a fetal urine peptide signature predicting postnatal renal outcome in fetuses with posterior urethral valves by Buffin-Meyer, Bénédicte, Tkaczyk, Marcin, Stańczyk, Małgorzata, Breuil, Benjamin, Siwy, Justyna, Szaflik, Krzysztof, Talar, Tomasz, Wojtera, Justyna, Krzeszowski, Waldemar, Decramer, Stéphane, Klein, Julie, Schanstra, Joost P.

“…Background Posterior urethral valves (PUVs) account for 17% of pediatric renal failure. The management of pregnancies involving fetuses with PUV is hampered by…”
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Association of kidney biopsy findings with short- and medium-term outcomes in children with moderate-to-severe IgA vasculitis nephritis by Clavé, Stéphanie, Sordet, Maud, Tsimaratos, Michel, Decramer, Stéphane, Fila, Marc, Guigonis, Vincent, Faudeux, Camille, Daniel, Laurent, Torrents, Julia, Boucekine, Mohamed, Harambat, Jérôme, Rousset-Rouvière, Caroline

“…Assessing the initial severity of immunoglobulin A vasculitis nephritis (IgAV-N) is important due to its determining effect on kidney management and outcomes…”
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To biopsy or not to biopsy: Henoch-Schönlein nephritis in children, a 5-year follow-up study by Avramescu, Marina, Lahoche, Annie, Hogan, Julien, Salomon, Rémi, Roussey, Gwenaëlle, Bacchetta, Justine, Decramer, Stéphane, Ulinski, Tim, Barbe, Coralie, Pietrement, Christine

“…Background The prognosis of Henoch-Schönlein purpura (HSP), IgA vasculitis, depends on kidney involvement. There is no consensus on the initiation of treatment…”
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Hepatocyte Nuclear Factor-1 β Controls Mitochondrial Respiration in Renal Tubular Cells by Casemayou, Audrey, Fournel, Audren, Bagattin, Alessia, Schanstra, Joost, Belliere, Julie, Decramer, Stéphane, Marsal, Dimitri, Gillet, Marion, Chassaing, Nicolas, Huart, Antoine, Pontoglio, Marco, Knauf, Claude, Bascands, Jean-Loup, Chauveau, Dominique, Faguer, Stanislas

“…AKI is a frequent condition that involves renal microcirculation impairment, infiltration of inflammatory cells with local production of proinflammatory…”
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Quality of life in children with severe forms of idiopathic nephrotic syndrome in stable remission—A cross‐sectional study by Roussel, Aphaia, Delbet, Jean‐Daniel, Micheland, Laurianne, Deschênes, Georges, Decramer, Stephane, Ulinski, Tim

“…Aim Severe forms of idiopathic nephrotic syndrome (INS) require immunosuppressive therapy: oral treatment or intravenous therapy (rituximab, RTX). The main…”
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Pediatric urogenital schistosomiasis diagnosed in France by Percheron, Lucas, Leblanc, Claire, Ulinski, Tim, Fila, Marc, Malvy, Denis, Bacchetta, Justine, Guigonis, Vincent, Debuisson, Cecile, Launay, Elise, Martinez, Edouard, Morand, Aurelie, Decramer, Stéphane, Schanstra, Joost-Peter, Berry, Antoine

“…Background Schistosomiasis affects approximately 230 million people worldwide. There is an increased incidence of schistosomiasis cases in France acquired from…”
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Cryptosporidium spp. Infection in Solid Organ Transplantation: The Nationwide “TRANSCRYPTO” Study by Lanternier, Fanny, Amazzough, Karima, Favennec, Loic, Mamzer-Bruneel, Marie-France, Abdoul, Hendy, Tourret, Jérome, Decramer, Stéphane, Zuber, Julien, Scemla, Anne, Legendre, Christophe, Lortholary, Olivier, Bougnoux, Marie-Elisabeth

“…BACKGROUNDDiarrhea is a frequent complication of solid organ transplantation. Cryptosporidiosis is classically reported in patients with acquired…”
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Gitelman-Like Syndrome Caused by Pathogenic Variants in mtDNA by Viering, Daan, Schlingmann, Karl P, Hureaux, Marguerite, Nijenhuis, Tom, Mallett, Andrew, Chan, Melanie M Y, van Beek, André, van Eerde, Albertien M, Coulibaly, Jean-Marie, Vallet, Marion, Decramer, Stéphane, Pelletier, Solenne, Klaus, Günter, Kömhoff, Martin, Beetz, Rolf, Patel, Chirag, Shenoy, Mohan, Steenbergen, Eric J, Anderson, Glenn, Bongers, Ernie M H F, Bergmann, Carsten, Panneman, Daan, Rodenburg, Richard J, Kleta, Robert, Houillier, Pascal, Konrad, Martin, Vargas-Poussou, Rosa, Knoers, Nine V A M, Bockenhauer, Detlef, de Baaij, Jeroen H F

“…Gitelman syndrome is the most frequent hereditary salt-losing tubulopathy characterized by hypokalemic alkalosis and hypomagnesemia. Gitelman syndrome is…”
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Urine in Clinical Proteomics by Decramer, Stéphane, de Peredo, Anne Gonzalez, Breuil, Benjamin, Mischak, Harald, Monsarrat, Bernard, Bascands, Jean-Loup, Schanstra, Joost P.

“…Urine has become one of the most attractive biofluids in clinical proteomics as it can be obtained non-invasively in large quantities and is stable compared…”
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Improved prenatal assessment of kidney disease using multiple ultrasound features by Buffin-Meyer, Bénédicte, Klein, Julie, Aziza, Jacqueline, Fernandez, Manon, Feuillet, Guylène, Seye, Mouhamed, Buléon, Marie, Fédou, Camille, Camus, Mylène, Burlet-Schiltz, Odile, Martinovic, Jelena, Saulnier-Blache, Jean-Sébastien, Decramer, Stéphane, Schanstra, Joost P

“…Congenital anomalies of the kidney and the urinary tract (CAKUT), often discovered in utero, cover a wide spectrum of outcomes ranging from normal postnatal…”
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Clinical outcomes in children with Henoch–Schönlein purpura nephritis without crescents by Delbet, Jean Daniel, Hogan, Julien, Aoun, Bilal, Stoica, Iulia, Salomon, Rémi, Decramer, Stéphane, Brocheriou, Isabelle, Deschênes, Georges, Ulinski, Tim

“…Background Henoch–Schönlein purpura is the most common vasculitis in children. Its long-term prognosis depends on renal involvement. The management of…”
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Definition, diagnosis and management of fetal lower urinary tract obstruction: consensus of the ERKNet CAKUT-Obstructive Uropathy Work Group by Capone, Valentina, Persico, Nicola, Berrettini, Alfredo, Decramer, Stèphane, De Marco, Erika Adalgisa, De Palma, Diego, Familiari, Alessandra, Feitz, Wout, Herthelius, Maria, Kazlauskas, Vytis, Liebau, Max, Manzoni, Gianantonio, Maternik, Michal, Mosiello, Giovanni, Schanstra, Joost Peter, Vande Walle, Johan, Wühl, Elke, Ylinen, Elisa, Zurowska, Aleksandra, Schaefer, Franz, Montini, Giovanni

“…Fetal lower urinary tract obstruction (LUTO) is associated with high mortality and postnatal morbidity caused by lung hypoplasia and impaired kidney function…”
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Hepatocyte nuclear factor‐1β shapes the energetic homeostasis of kidney tubule cells by Piedrafita, Alexis, Balayssac, Stéphane, Casemayou, Audrey, Saulnier‐Blache, Jean‐Sébastien, Lucas, Alexandre, Iacovoni, Jason S., Breuil, Benjamin, Chauveau, Dominique, Decramer, Stéphane, Malet‐Martino, Myriam, Schanstra, Joost P., Faguer, Stanislas

“…Energetic metabolism controls key steps of kidney development, homeostasis, and epithelial repair following acute kidney injury (AKI). Hepatocyte nuclear…”
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