Evaluation and management of steroid-unresponsive nephrotic syndrome.
OBJECTIVE
Idiopathic nephrotic syndrome in children is commonly associated with minimal change disease and response to steroid therapy. Steroid-unresponsive nephrotic syndrome is often characterized by persistent proteinuria and progression to chronic kidney disease. Focal segmental glomerulosclerosis is the leading cause of steroid-unresponsive nephrotic syndrome in childhood. There is no uniformed consensus as to the treatment of steroid-unresponsive nephrotic syndrome. Advances in the pathogenesis, genetics and biomarkers or surrogate markers may be useful for the diagnosis and identification of patients with steroid-unresponsive nephrotic syndrome, severity of disease, progression and response to therapy.


RESULTS
This review is intended to describe some of the recent changes in the epidemiology of steroid-unresponsive nephrotic syndrome, in particular focal segmental glomerulosclerosis, its pathogenesis and alternative therapies.


CONCLUSIONS
Recent studies in both children and adults have shown an increase in the incidence of focal segmental sclerosis as a cause of steroid-unresponsive nephrotic syndrome. Advances in the pathogenesis and noninvasive methods of diagnosis may allow for the identification of steroid-responsive patients.
Del Rio
Marcela
M
Division of Pediatric Nephrology, Children's Hospital at Montefiore, Bronx, New York 10467, USA. mdelrio@montefiore.org
Kaskel
Frederick
F
D002648:Child; D003524:Cyclosporins / Q000627:therapeutic use; D005923:Glomerulosclerosis, Focal Segmental / Q000150:complications; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D009173:Mycophenolic Acid / Q000031:analogs & derivatives / Q000627:therapeutic use; D009404:Nephrotic Syndrome / Q000175:diagnosis* / Q000188:drug therapy* / Q000209:etiology; D016032:Randomized Controlled Trials as Topic; D020123:Sirolimus / Q000627:therapeutic use; D016559:Tacrolimus / Q000627:therapeutic use; D016896:Treatment Outcome
D003524:Cyclosporins; D007166:Immunosuppressive Agents; D009173:Mycophenolic Acid; D020123:Sirolimus; D016559:Tacrolimus