Spectrum of steroid-resistant and congenital nephrotic syndrome in children: the PodoNet registry cohort
A Trautmann, M Bodria, F Ozaltin… - Clinical Journal of the …, 2015 - journals.lww.com
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 …
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 …