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639 results on '"P Niaudet"'

2. Excessive vascular sprouting underlies cerebral hemorrhage in mice lacking αVβ8-TGFβ signaling in the brain

Author

Arnold, Thomas D, Niaudet, Colin, Pang, Mei-Fong, Siegenthaler, Julie, Gaengel, Konstantin, Jung, Bongnam, Ferrero, Gina M, Mukouyama, Yoh-suke, Fuxe, Jonas, Akhurst, Rosemary, Betsholtz, Christer, Sheppard, Dean, and Reichardt, Louis F

Subjects
Medical Physiology, Biomedical and Clinical Sciences, Neurosciences, Cerebrovascular, Brain Disorders, 2.1 Biological and endogenous factors, Cardiovascular, Analysis of Variance, Animals, Blood-Brain Barrier, Brain, Cell Count, Cerebral Hemorrhage, Endothelial Cells, Immunohistochemistry, Integrins, Mice, Microscopy, Confocal, Neovascularization, Pathologic, Signal Transduction, Transforming Growth Factor beta, Angiogenesis, CNS, Hemorrhage, Sprouting, Integrin alpha V beta 8, TGF beta, Mouse, Integrin αVβ8, TGFβ, Biological Sciences, Medical and Health Sciences, Biological sciences, Biomedical and clinical sciences, Health sciences
Abstract

Vascular development of the central nervous system and blood-brain barrier (BBB) induction are closely linked processes. The role of factors that promote endothelial sprouting and vascular leak, such as vascular endothelial growth factor A, are well described, but the factors that suppress angiogenic sprouting and their impact on the BBB are poorly understood. Here, we show that integrin αVβ8 activates angiosuppressive TGFβ gradients in the brain, which inhibit endothelial cell sprouting. Loss of αVβ8 in the brain or downstream TGFβ1-TGFBR2-ALK5-Smad3 signaling in endothelial cells increases vascular sprouting, branching and proliferation, leading to vascular dysplasia and hemorrhage. Importantly, BBB function in Itgb8 mutants is intact during early stages of vascular dysgenesis before hemorrhage. By contrast, Pdgfb(ret/ret) mice, which exhibit severe BBB disruption and vascular leak due to pericyte deficiency, have comparatively normal vascular morphogenesis and do not exhibit brain hemorrhage. Our data therefore suggest that abnormal vascular sprouting and patterning, not BBB dysfunction, underlie developmental cerebral hemorrhage.

Published
2014

3. Nephropathic cystinosis: an international consensus document

Author

Emma, Francesco, Nesterova, Galina, Langman, Craig, Labbé, Antoine, Cherqui, Stephanie, Goodyer, Paul, Janssen, Mirian C, Greco, Marcella, Topaloglu, Rezan, Elenberg, Ewa, Dohil, Ranjan, Trauner, Doris, Antignac, Corinne, Cochat, Pierre, Kaskel, Frederick, Servais, Aude, Wühl, Elke, Niaudet, Patrick, Hoff, William Van't, Gahl, William, and Levtchenko, Elena

Subjects
Biomedical and Clinical Sciences, Clinical Sciences, Kidney Disease, Rare Diseases, Pediatric, Child, Cystinosis, Fanconi Syndrome, Humans, Practice Guidelines as Topic, Societies, Medical, CTNS gene, cysteamine treatment, cystinosis, extra-renal complications, renal Fanconi syndrome, Urology & Nephrology, Clinical sciences
Abstract

Cystinosis is caused by mutations in the CTNS gene (17p13.2), which encodes for a lysosomal cystine/proton symporter termed cystinosin. It is the most common cause of inherited renal Fanconi syndrome in young children. Because of its rarity, the diagnosis and specific treatment of cystinosis are frequently delayed, which has a significant impact on the overall prognosis. In this document, we have summarized expert opinions on several aspects of the disease to improve knowledge and provide guidance for diagnosis and treatment.

Published
2014

5. Clinical and genetic heterogeneity in familial steroid-sensitive nephrotic syndrome

Author

Dorval, Guillaume, Gribouval, Olivier, Martinez-Barquero, Vanesa, Machuca, Eduardo, Tête, Marie-Josèphe, Baudouin, Véronique, Benoit, Stéphane, Chabchoub, Imen, Champion, Gérard, Chauveau, Dominique, Chehade, Hassib, Chouchane, Chokri, Cloarec, Sylvie, Cochat, Pierre, Dahan, Karin, Dantal, Jacques, Delmas, Yahsou, Deschênes, Georges, Dolhem, Phillippe, Durand, Dominique, Ekinci, Zelal, El Karoui, Khalil, Fischbach, Michel, Grunfeld, Jean-Pierre, Guigonis, Vincent, Hachicha, Mongia, Hogan, Julien, Hourmant, Maryvonne, Hummel, Aurélie, Kamar, Nassim, Krummel, Thierry, Lacombe, Didier, Llanas, Brigitte, Mesnard, Laurent, Mohsin, Nabil, Niaudet, Patrick, Nivet, Hubert, Parvex, Paloma, Pietrement, Christine, de Pontual, Loic, Noble, Claire Pouteil, Ribes, David, Ronco, Pierre, Rondeau, Eric, Sallee, Marion, Tsimaratos, Michel, Ulinski, Tim, Salomon, Rémi, Antignac, Corinne, and Boyer, Olivia

Published
2017
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21. Estimating Time to ESRD in Children With CKD

Author

Susan L. Furth, Chris Pierce, Wun Fung Hui, Colin A. White, Craig S. Wong, Franz Schaefer, Elke Wühl, Alison G. Abraham, Bradley A. Warady, Joshua Samuels, Susan Furth, Meredith Atkinson, Amy Wilson, Alejandro Quiroga, Susan Massengill, Dave Selewski, Maria Ferris, Amy Kogon, Frederick Kaskel, Marc Lande, George Schwartz, Jeffrey Saland, Victoria Norwood, Tej Matoo, Guillermo Hidalgo, Poyyapakkam Srivaths, Joann Carlson, Craig Langman, Susan Mendley, Eunice John, Kiran Upadhyay, Patricia Seo-Mayer, Larry Patterson, Rulan Parekh, Lisa Robinson, Adam Weinstein, Dmitry Samsonov, Juan Kupferman, Jason Misurac, Anil Mongia, Steffan Kiessling, Cheryl Sanchez-Kazi, Allison Dart, Sahar Fathallah, Donna Claes, Mark Mitsnefes, Tom Blydt-Hansen, Bradley Warady, Larry Greenbaum, Joseph Flynn, Craig Wong, Isidro Salusky, Ora Yadin, Katherine Dell, Randall Jenkins, Cynthia Pan, Elaine Ku, Amira Al-Uzri, Nancy Rodig, Cynthia Wong, Keefe Davis, Martin Turman, Sharon Bartosh, Colleen Hastings, Anjali Nayak, Mouin Seikaly, Nadine Benador, Robert Mak, Ellen Wood, Gary Lerner, Gina Marie Barletta, A. Anarat, A. Bakkaloglu, F. Ozaltin, A. Peco-Antic, U. Querfeld, J. Gellermann, P. Sallay, D. Drożdż, K.-E. Bonzel, A.-M. Wingen, A. Żurowska, I. Balasz, A. Trivelli, F. Perfumo, D.E. Müller-Wiefel, K. Möller, G. Offner, B. Enke, E. Wühl, C. Hadtstein, O. Mehls, F. Schaefer, S. Emre, S. Caliskan, S. Mir, S. Wygoda, K. Hohbach-Hohenfellner, N. Jeck, G. Klaus, G. Ardissino, S. Testa, G. Montini, M. Charbit, P. Niaudet, A. Caldas-Afonso, A. Fernandes-Teixeira, J. Dušek, M.C. Matteucci, S. Picca, A. Mastrostefano, M. Wigger, U.B. Berg, G. Celsi, M. Fischbach, J. Terzic, J. Fydryk, T. Urasinski, R. Coppo, L. Peruzzi, K. Arbeiter, A. Jankauskiené, R. Grenda, M. Litwin, R. Janas, and T.J. Neuhaus

Subjects
medicine.medical_specialty, Time Factors, Adolescent, Databases, Factual, medicine.medical_treatment, 030232 urology & nephrology, Renal function, Disease, 030204 cardiovascular system & hematology, Kidney Function Tests, Risk Assessment, Article, Cohort Studies, 03 medical and health sciences, Sex Factors, 0302 clinical medicine, Renal Dialysis, Internal medicine, medicine, Humans, Renal replacement therapy, Renal Insufficiency, Chronic, Child, Proteinuria, business.industry, Age Factors, Infant, Guideline, medicine.disease, Treatment Outcome, Nephrology, Child, Preschool, North America, Disease Progression, Kidney Failure, Chronic, Observational study, medicine.symptom, business, Follow-Up Studies, Glomerular Filtration Rate, Kidney disease, Cohort study
Abstract

RATIONALE & OBJECTIVE: The KDIGO (Kidney Disease: Improving Global Outcomes) guideline on CKD presented an international classification system that ranks patients' risk for CKD progression. Few data on children informed guideline development STUDY DESIGN: Observational cohort study SETTINGS AND PARTICIPANTS: Children aged 1-18 years enrolled in the North American Chronic Kidney Disease in Children (CKiD) cohort study and the European Effect of Strict Blood Pressure Control and ACE Inhibition on the Progression of CRF in Pediatric Patients (ESCAPE) trial. PREDICTOR: Level of estimated glomerular filtration rate (eGFR) and proteinuria (urine protein-creatinine ratio (mg/mg)) at study entry OUTCOME: A composite event of renal replacement therapy, 50% reduction of estimated GFR (eGFR), or eGFR 2.0 at study entry. Six ordered stages with varying combinations of eGFR categories (60-89, 45-59, 30-44 and 15-29 ml/min/1.73m(2)) and UPCR categories (2.0) described the risk continuum. Median times to event ranged from >10 years for eGFR 45-90 and UPCR 2. Children with glomerular disease were estimated to have a 43% shorter time to event than children with nonglomerular disease. Cross-validation demonstrated risk patterns that were consistent across the ten subsample validation models. LIMITATIONS: Observational study, utilized cross validation rather than external validation CONCLUSION: CKD staged by level of eGFR and proteinuria characterizes the timeline of progression and can guide management strategies in children.

Published
2018
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22. Respiratory chain deficiency presenting as congenital nephrotic syndrome

Author

Goldenberg, Alice, Ngoc, Linh Huynh, Thouret, Marie-Christine, Cormier-Daire, Valérie, Gagnadoux, Marie-France, Chrétien, Dominique, Lefrançois, Catherine, Geromel, Vanna, Rötig, Agnès, Rustin, Pierre, Munnich, Arnold, Paquis, Véronique, Antignac, Corinne, Gubler, Marie-Claire, Niaudet, Patrick, de Lonlay, Pascale, and Bérard, Etienne

Published
2005
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29. Clonal culturing of human embryonic stem cells on laminin-521/E-cadherin matrix in defined and xeno-free environment

Author

Rodin, Sergey, Antonsson, Liselotte, Niaudet, Colin, Simonson, Oscar E., Salmela, Elina, Hansson, Emil M., Domogatskaya, Anna, Xiao, Zhijie, Damdimopoulou, Pauliina, Sheikhi, Mona, Inzunza, José, Nilsson, Ann-Sofie, Baker, Duncan, Kuiper, Raoul, Sun, Yi, Blennow, Elisabeth, Nordenskjöld, Magnus, Grinnemo, Karl-Henrik, Kere, Juha, Betsholtz, Christer, Hovatta, Outi, and Tryggvason, Karl

Published
2014
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41. Hemolytic-Uremic Syndrome in Children

Author

Boyer, Olivia and Niaudet, Patrick

Abstract

Hemolytic uremic syndrome is characterized by a triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney failure. Most cases are caused by Shiga-toxin-producing bacteria, especially Escherichia coli. Transmission occurs through ground beef and unpasteurized milk. STEC-HUS is the main cause of acute renal failure in children. Management remains supportive. Immediate outcome is most often. Atypical HUS represents about 5% of cases, has a relapsing course with more than half of the patients progressing to end-stage kidney failure. Most cases are due to variants in complement regulators of the alternative pathway. Complement inhibitors, such as eculizumab, have considerably improved the prognosis.

Published
2022
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