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10.2215/CJN.06260614 http://scihub22266oqcxt.onion/10.2215/CJN.06260614 C4386250!4386250 !25635037     free     free     free Warning: file_get_contents(https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&id=25635037 &cmd=llinks): Failed to open stream: HTTP request failed! 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Spectrum of steroid-resistant and congenital nephrotic syndrome in children: the PodoNet registry cohort |pdf=|usr=}}{{25635037 }} gab.com Text Spectrum of steroid-resistant and congenital nephrotic syndrome in children: the PodoNet registry cohort JO: Clin J Am Soc Nephrol http://www.kidney.de/pget.php?&tw=1&pmid=25635037 #FOAvip 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 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. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: Since August of 2009, clinical, biochemical, genetic, and histopathologic information was collected both retrospectively and prospectively from 1655 patients with childhood-onset steroid-resistant nephrotic syndrome, congenital nephrotic syndrome, or persistent subnephrotic proteinuria of likely genetic origin at 67 centers in 21 countries through an online portal. RESULTS: Steroid-resistant nephrotic syndrome manifested in the first 5 years of life in 64% of the patients. Congenital nephrotic syndrome accounted for 6% of all patients. Extrarenal abnormalities were reported in 17% of patients. The most common histopathologic diagnoses were FSGS (56%), minimal change nephropathy (21%), and mesangioproliferative GN (12%). Mutation screening was performed in 1174 patients, and a genetic disease cause was identified in 23.6% of the screened patients. Among 14 genes with reported mutations, abnormalities in NPHS2 (n=138), WT1 (n=48), and NPHS1 (n=41) were most commonly identified. The proportion of patients with a genetic disease cause decreased with increasing manifestation age: from 66% in congenital nephrotic syndrome to 15%-16% in schoolchildren and adolescents. Among various intensified immunosuppressive therapy protocols, calcineurin inhibitors and rituximab yielded consistently high response rates, with 40%-45% of patients achieving complete remission. Confirmation of a genetic diagnosis but not the histopathologic disease type was strongly predictive of intensified immunosuppressive therapy responsiveness. Post-transplant disease recurrence was noted in 25.8% of patients without compared with 4.5% (n=4) of patients with a genetic diagnosis. CONCLUSIONS: The PodoNet cohort may serve as a source of reference for future clinical and genetic research in this rare but significant kidney disease. Twit Text FOAVip Spectrum of steroid-resistant and congenital nephrotic syndrome in children: the PodoNet registry cohort ????Clin J Am Soc Nephrol http://www.kidney.de/pget.php?&tw=1&pmid=25635037 #FOAvip Twit Text # Free Paper on # # # # # LINK http://www.kidney.de/pget.php?&tw=1&pmid=25635037 #FOAvip English Wikipedia <ref>{{Cite pmid|25635037 }}</ref> Spectrum of steroid-resistant and congenital nephrotic syndrome in children: the PodoNet registry cohort #MMPMID25635037 Trautmann A ; Bodria M ; Ozaltin F ; Gheisari A ; Melk A ; Azocar M ; Anarat A ; Caliskan S ; Emma F ; Gellermann J ; Oh J ; Baskin E ; Ksiazek J ; Remuzzi G ; Erdogan O ; Akman S ; Dusek J ; Davitaia T ; Özkaya O ; Papachristou F ; Firszt-Adamczyk A ; Urasinski T ; Testa S ; Krmar RT ; Hyla-Klekot L ; Pasini A ; Özcakar ZB ; Sallay P ; Cakar N ; Galanti M ; Terzic J ; Aoun B ; Caldas Afonso A ; Szymanik-Grzelak H ; Lipska BS ; Schnaidt S ; Schaefer F Clin J Am Soc Nephrol 2015[Apr]; 10 (4 ): 592-600 PMID25635037 show ga 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 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. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: Since August of 2009, clinical, biochemical, genetic, and histopathologic information was collected both retrospectively and prospectively from 1655 patients with childhood-onset steroid-resistant nephrotic syndrome, congenital nephrotic syndrome, or persistent subnephrotic proteinuria of likely genetic origin at 67 centers in 21 countries through an online portal. RESULTS: Steroid-resistant nephrotic syndrome manifested in the first 5 years of life in 64% of the patients. Congenital nephrotic syndrome accounted for 6% of all patients. Extrarenal abnormalities were reported in 17% of patients. The most common histopathologic diagnoses were FSGS (56%), minimal change nephropathy (21%), and mesangioproliferative GN (12%). Mutation screening was performed in 1174 patients, and a genetic disease cause was identified in 23.6% of the screened patients. Among 14 genes with reported mutations, abnormalities in NPHS2 (n=138), WT1 (n=48), and NPHS1 (n=41) were most commonly identified. The proportion of patients with a genetic disease cause decreased with increasing manifestation age: from 66% in congenital nephrotic syndrome to 15%-16% in schoolchildren and adolescents. Among various intensified immunosuppressive therapy protocols, calcineurin inhibitors and rituximab yielded consistently high response rates, with 40%-45% of patients achieving complete remission. Confirmation of a genetic diagnosis but not the histopathologic disease type was strongly predictive of intensified immunosuppressive therapy responsiveness. Post-transplant disease recurrence was noted in 25.8% of patients without compared with 4.5% (n=4) of patients with a genetic diagnosis. CONCLUSIONS: The PodoNet cohort may serve as a source of reference for future clinical and genetic research in this rare but significant kidney disease. |*Glomerulonephritis, Membranoproliferative/diagnosis/epidemiology/genetics/therapy [MESH] |*Glomerulosclerosis, Focal Segmental/diagnosis/epidemiology/genetics/therapy [MESH] |*Nephrosis, Lipoid/diagnosis/epidemiology/genetics/therapy [MESH] |Adolescent [MESH] |Age Distribution [MESH] |Age of Onset [MESH] |Biopsy [MESH] |Child [MESH] |Child, Preschool [MESH] |DNA Mutational Analysis [MESH] |Europe/epidemiology [MESH] |Female [MESH] |Genetic Markers [MESH] |Genetic Predisposition to Disease [MESH] |Humans [MESH] |Immunosuppressive Agents/therapeutic use [MESH] |Infant [MESH] |Infant, Newborn [MESH] |Kidney Transplantation [MESH] |Latin America/epidemiology [MESH] |Male [MESH] |Middle East/epidemiology [MESH] |Mutation [MESH] |Nephrotic Syndrome/*congenital/diagnosis/epidemiology/genetics/therapy [MESH] |Phenotype [MESH] |Prospective Studies [MESH] |Recurrence [MESH] |Registries [MESH] |Remission Induction [MESH] |Retrospective Studies [MESH] |Risk Factors [MESH] |Treatment Outcome [MESH]Spectrum of steroid-resistant and congenital nephrotic syndrome in chi(epmc).pdf DeepDyve Pubget Overpricing