Advances in podocytology and genetic techniques have expanded our understanding of the pathogenesis of hereditary steroid-resistant nephrotic syndrome (SRNS). In the past 20 years, over 45 genetic mutations have been identified in patients with hereditary SRNS. Genetic mutations on structural and functional molecules in podocytes can lead to serious injury in the podocytes themselves and in adj...

Schimke immuno-osseous dysplasia is a rare autosomal recessive multisystem disorder characterized by steroid-resistant nephrotic syndrome, immunodeficiency, and spondyloepiphy-seal dysplasia. Mutations in SWI/SNF2 related, matrix associated, actin dependent regulator of chromatin, subfamily a-like 1 (SMARCAL1) gene are responsible for the disease. The present report describes, for the f...

Idiopathic nephrotic syndrome is commonly seen in childhood between three months and 16 years of age, and const itutes 90% of all causes o f nephrot ic syndrome in childhood. It is defined as the association of nephrotic syndrome with minimal glomerular changes or nonspecific lesions, such as segmental and focal glomerular sclerosis or di ffuse mesangial p roliferat ion without any immune compl...

Abstract Background Nephrotic syndrome is characterized by proteinuria, hyperlipidemia, and edema. The annual incidence of nephrotic 2–7 cases/100,000 children. Methods results present study included 32 control, steroid-sensitive cases, steroid-resistant cases aged ≤ 17 years. Serum samples were handled to check serum albumin, creatinine, calcium, total cholesterol in SSNS SRNS. Pearson’s corre...

AMA Kuran P, Platos E, Mizerska-Wasiak M, Pańczyk-Tomaszewska M. A rare cause of steroid-resistant nephrotic syndrome – a case report. Central European Journal Immunology. 2023. doi:10.5114/ceji.2023.127534. APA Kuran, P., Platos, E., Mizerska-Wasiak, M., & Pańczyk-Tomaszewska, (2023). https://doi.org/10.5114/ceji.2023.127534 Chicago Paulina, Emilia Małgorzata and Pańczyk-Tomaszewska. "A report...

Introduction. Nephrotic syndrome (NS) is a disease of the glomeruli that occurs in childhood with frequency 12–16 per 100,000. More than 85 % children NS respond to corticosteroid therapy, approximately 10–15 remain refractory or later become resistant them. In 10–30 patients steroid-resistant nephrotic (SRNS), mutations structural genes podocytes, modifier were found, there also evidence forma...