نتایج جستجو برای: congenital nephrotic syndrome

تعداد نتایج: 719259  

Journal: :Clinical journal of the American Society of Nephrology : CJASN 2015
Agnes Trautmann Monica Bodria Fatih Ozaltin Alaleh Gheisari Anette Melk Marta Azocar Ali Anarat Salim Caliskan Francesco Emma Jutta Gellermann Jun Oh Esra Baskin Joanna Ksiazek Giuseppe Remuzzi Ozlem Erdogan Sema Akman Jiri Dusek Tinatin Davitaia Ozan Özkaya Fotios Papachristou Agnieszka Firszt-Adamczyk Tomasz Urasinski Sara Testa Rafael T Krmar Lidia Hyla-Klekot Andrea Pasini Z Birsin Özcakar Peter Sallay Nilgun Cakar Monica Galanti Joelle Terzic Bilal Aoun Alberto Caldas Afonso Hanna Szymanik-Grzelak Beata S Lipska Sven Schnaidt Franz Schaefer

BACKGROUND AND OBJECTIVES 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 th...

2010
Isabel Carvalho Graça Ferreira Eduarda Marques Mercedes Sanchez Rosete Nogueira Maria S. Faria Conceição Mota

This is a rare renal disease characterised by steroidresistant nephrotic syndrome in the first years of life with rapid progression to end-stage renal failure. Its occurrence is usually sporadic, and it has been associated with a mutation in the Wilms tumour (WT1) suppressor gene. Recently new gene defects causing isolated diffuse mesangial sclerosis, such as LAMB2 and PLCE1 mutations, have bee...

Journal: :Clinical journal of the American Society of Nephrology : CJASN 2018
Jillian K Warejko Weizhen Tan Ankana Daga David Schapiro Jennifer A Lawson Shirlee Shril Svjetlana Lovric Shazia Ashraf Jia Rao Tobias Hermle Tilman Jobst-Schwan Eugen Widmeier Amar J Majmundar Ronen Schneider Heon Yung Gee J Magdalena Schmidt Asaf Vivante Amelie T van der Ven Hadas Ityel Jing Chen Carolin E Sadowski Stefan Kohl Werner L Pabst Makiko Nakayama Michael J G Somers Nancy M Rodig Ghaleb Daouk Michelle Baum Deborah R Stein Michael A Ferguson Avram Z Traum Neveen A Soliman Jameela A Kari Sherif El Desoky Hanan Fathy Martin Zenker Sevcan A Bakkaloglu Dominik Müller Aytul Noyan Fatih Ozaltin Melissa A Cadnapaphornchai Seema Hashmi Jeffrey Hopcian Jeffrey B Kopp Nadine Benador Detlef Bockenhauer Radovan Bogdanovic Nataša Stajić Gil Chernin Robert Ettenger Henry Fehrenbach Markus Kemper Reyner Loza Munarriz Ludmila Podracka Rainer Büscher Erkin Serdaroglu Velibor Tasic Shrikant Mane Richard P Lifton Daniela A Braun Friedhelm Hildebrandt

BACKGROUND AND OBJECTIVES Steroid-resistant nephrotic syndrome overwhelmingly progresses to ESRD. More than 30 monogenic genes have been identified to cause steroid-resistant nephrotic syndrome. We previously detected causative mutations using targeted panel sequencing in 30% of patients with steroid-resistant nephrotic syndrome. Panel sequencing has a number of limitations when compared with w...

Journal: :Archives of Disease in Childhood 1973

2015
Agnes Trautmann Monica Bodria Fatih Ozaltin Alaleh Gheisari Anette Melk Marta Azocar Ali Anarat Salim Caliskan Francesco Emma Jutta Gellermann Jun Oh Esra Baskin Joanna Ksiazek Giuseppe Remuzzi Ozlem Erdogan Sema Akman Jiri Dusek Tinatin Davitaia Fotios Papachristou Agnieszka Firszt-Adamczyk Tomasz Urasinski Sara Testa Rafael T. Krmar Lidia Hyla-Klekot Andrea Pasini Peter Sallay Nilgun Cakar Monica Galanti Joelle Terzic Bilal Aoun Alberto Caldas Afonso Hanna Szymanik-Grzelak Beata S. Lipska

Background and objectives 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 th...

2007
Arvi-Matti Kuusniemi

9 Review of the literature 11 1. Congenital nephrotic syndrome of the Finnish type (CNF, NPHS1) 11

Journal: :The Turkish journal of pediatrics 2014
Münevver Kaynak-Türkmen Fulya Cengiz-Erdem Ferah Sönmez İlknur Girişgen Murat Telli Afig Berdeli

Pertussis or whooping cough is a vaccine-preventable disease that still remains a serious infection in neonates and young infants. The disease is particularly severe in infants less than three months old, who are often infected by their parents. Congenital nephrotic syndrome is a rare entity presenting within the first three months. It encompasses a heterogeneous group of entities with genetic,...

2012
Julio E Barrios Claudia Duran Botello Tania González Velásquez

INTRODUCTION Although the association of infection by toxoplasmosis with the development of nephrotic syndrome is uncommon, cases of this association have nevertheless been reported in the literature for more than two decades, not only for congenital toxoplasmosis, but also in acquired cases, and occasionally in immunocompetent patients. DEVELOPMENT A case is presented of an immunocompetent p...

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