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Nephrol Dial Transplant (2000) 15: 1897-1900
© 2000 European Renal Association-European Dialysis and Transplant Association

Editorial Comments

Familial steroid-resistant nephrotic syndromes: recent advances

Arno Fuchshuber and Otto Mehls

University Children's Hospitals of Freiburg and Heidelberg, Germany

Introduction

Nephrotic syndrome (NS) usually occurs as a sporadic disease, but familial cases have frequently been reported. The first observation in six families with 13 affected siblings was reported by Fanconi et al. [1]. Larger population studies have been published since 1970 [2–4]; whereas only some of the reported families presented with steroid-sensitive NS, the majority presented with steroid-resistant NS. In most of the patients with primary steroid-resistant NS, focal segmental glomerulosclerosis (FSGS) was demonstrated by renal biopsy. Despite uniform renal histological findings, heterogeneous pathogenesis had been suspected, but it is only during the last 5 years that several genes have been localized which have allowed a more precise classification of FSGS and steroid-resistant NS. Recently the gene for steroid-resistant NS (NPHS2) was cloned [5], which is an important step in understanding the pathogenesis of NS in general and NPHS2 in particular.

Familial idiopathic nephrotic syndrome

. . . [Full Text of this Article]

Congenital nephrotic syndrome of the Finnish type

Nephrotic syndrome and WT1 mutations

Lessons from the genetic studies in steroid resistant NS

Notes

References


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