Congenital nephrotic syndrome

Abstract

CNS (Congenital nephrotic syndrome) is a disorder characterized by the presence of a nephrotic syndrome in the first three months of life. Different pathologies can cause this syndrome. In general, we can distinguish primary forms (sporadic and hereditary) and secondary forms (acquired and associated with other syndromes). The most common form is the Finnish CNS (CNF, congenital nephrotic syndrome of the Finnish type), a hereditary form whose name derives from the fact that the highest incidence is described in that country (1.2:10,000). The pathogenesis, the clinical picture, the diagnostic criteria, the therapy and the outcome are described in details. Proceedings of the International Course on Perinatal Pathology (part of the 10th International Workshop on Neonatology · October 22nd-25th, 2014) · Cagliari (Italy) · October 25th, 2014 · The role of the clinical pathological dialogue in problem solving Guest Editors: Gavino Faa, Vassilios Fanos, Peter Van Eyken

Keywords

• nephrotic syndrome
• newborn
• proteinuria
• hypoalbuminemia
• hyperlipidemia
• edema
• hypercoagulability
• hypergammaglobulinemia