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The nephrotic syndrome is characterized by a combination of pathological lab values and clinical symptoms, i. e. pronounced proteinuria (usually more than 3 - 3,5 g protein/24 h), hypoalbuminemia, edema and hyperlipidemia. The causes of the nephrotic syndrome are manifold and can be inherited or acquired, chronically persistent or reversible. The frequency of the causes of the nephrotic syndrome is different among children and adults. While in adults acquired forms are common, genetic forms are more abundant among children. All diseases related to nephrotic proteinuria have in common that the causal defect affects the glomerular podocyte in a primary or secondary manner. This review article discusses the question when nowadays genetic diagnostics should be considered.