Congenital nephrotic syndrome

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.