Idiopathisches nephrotisches Syndrom im Kindesalter

Although idiopathic nephrotic syndrome in children fulfils the criteria of an orphan disease, it is also one of the most common glomerulopathies in childhood. Clinically, the first episode of the disease is usually characterized by edema and sudden weight gain due to fluid retention. The diagnosis is made based on extensive proteinuria and hyperalbuminemia. Following the first episode a chronic disease develops in most cases, which is characterized by relapses of the underlying disease. The heterogeneity of the disease in terms of patient age at first episode, etiology, histology and response to standard glucocorticoid treatment influences the differential diagnostics and the treatment. As the prognosis of idiopathic steroid-sensitive nephrotic syndrome is generally good in terms of renal function, in addition to the efficacy the potential spectrum of side effects should also be taken into account when planning treatment.