Steroid-resistant nephrotic syndrome is a rare kidney disease involving either immune-mediated or genetic alterations of podocyte structure and function. The rare nature, heterogeneity, and slow evolution of the disorder are major obstacles to systematic genotype-phenotype, intervention, and outcome studies, hampering the development of evidence-based diagnostic and therapeutic concepts. To overcome these limitations, the PodoNet Consortium has created an international registry for congenital nephrotic syndrome and childhood-onset steroid-resistant nephrotic syndrome.

Spectrum of steroid-resistant and congenital nephrotic syndrome in children : the PodoNet registry cohort / A. Trautmann, M. Bodria, F. Ozaltin, A. Gheisari, A. Melk, M. Azocar, A. Anarat, S. Caliskan, F. Emma, J. Gellermann, J. Oh, E. Baskin, J. Ksiazek, G. Remuzzi, O. Erdogan, S. Akman, J. Dusek, T. Davitaia, O. Özkaya, F. Papachristou, A. Firszt-Adamczyk, T. Urasinski, S. Testa, R.T. Krmar, L. Hyla-Klekot, A. Pasini, Z.B. Özcakar, P. Sallay, N. Cakar, M. Galanti, J. Terzic, B. Aoun, A. Caldas Afonso, H. Szymanik-Grzelak, B.S. Lipska, S. Schnaidt, F. Schaefer. - In: CLINICAL JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY. - ISSN 1555-9041. - 10:4(2015 Apr 07), pp. 592-600.

Spectrum of steroid-resistant and congenital nephrotic syndrome in children : the PodoNet registry cohort

G. Remuzzi;
2015

Abstract

Steroid-resistant nephrotic syndrome is a rare kidney disease involving either immune-mediated or genetic alterations of podocyte structure and function. The rare nature, heterogeneity, and slow evolution of the disorder are major obstacles to systematic genotype-phenotype, intervention, and outcome studies, hampering the development of evidence-based diagnostic and therapeutic concepts. To overcome these limitations, the PodoNet Consortium has created an international registry for congenital nephrotic syndrome and childhood-onset steroid-resistant nephrotic syndrome.
WT1; children; nephrin; podocin; podocytopathies
Settore MED/14 - Nefrologia
7-apr-2015
Article (author)
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2434/333415
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