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Treatment of childhood nephrotic syndrome
Insights
Optimal treatment for childhood nephrotic syndrome involves specific prednisone courses for steroid-responsive cases and intensive protocols for steroid-resistant focal segmental glomerulosclerosis. Further research is needed for other agents.
Area of Science:
- Pediatric Nephrology
- Clinical Therapeutics
- Glomerular Diseases
Background:
- Nephrotic syndrome is a significant kidney disorder in children.
- Steroid therapy is a cornerstone, but optimal protocols and management of resistant cases require clarification.
Purpose of the Study:
- To review current treatment strategies for childhood nephrotic syndrome.
- To highlight optimal steroid regimens for steroid-responsive nephrotic syndrome.
- To discuss management of steroid-resistant nephrotic syndrome, particularly focal segmental glomerulosclerosis.
Main Methods:
- Review of controlled studies on steroid therapy duration.
- Analysis of treatment protocols for focal segmental glomerulosclerosis.
- Brief review of emerging therapies like ACE inhibitors and cyclosporine.
Main Results:
- Longer prednisone courses are optimal for initial episodes of steroid-responsive nephrotic syndrome.
- Shorter courses are effective for relapses.
- Intensive methylprednisolone and alkylating agent therapy may improve outcomes in focal segmental glomerulosclerosis, pending controlled trials.
Conclusions:
- Treatment duration for prednisone in steroid-responsive nephrotic syndrome should be tailored to initial episodes versus relapses.
- Intensive protocols show promise for focal segmental glomerulosclerosis but require validation.
- The roles of ACE inhibitors and cyclosporine in childhood nephrotic syndrome are currently undefined.
Abstract:
This review examines selected aspects of the treatment of the nephrotic syndrome in children. Particular attention has been paid to two groups of nephrotic children. First, children with steroid-responsive nephrotic syndrome are discussed. Recently, a series of controlled studies have provided important information regarding the optimal duration of steroid therapy. Initial episodes of the nephrotic syndrome are best treated with "long" courses of prednisone therapy (6 wk of high-dose daily prednisone followed by 6 wk of alternate-day prednisone). In contrast, relapses do as well with "short" courses (about 2 wk of daily prednisone and 2 wk of alternate-day therapy). Some children who are steroid responsive require high doses of prednisone to remain in remission. These patients may require alkylating agent therapy. The most common cause of steroid-resistant nephrotic syndrome is focal segmental glomerulosclerosis. Over the past 10 yr, these patients have been treated with an intensive protocol involving multiple infusions of high-dose methylprednisolone and, in many cases, oral alkylating agent therapy. Current experience with this treatment is presented. The protocol appears to improve the outcome in children with focal segmental glomerulosclerosis, although it is believed that it is essential that these observations be confirmed by a controlled trial. There is also interest in the use of angiotensin-converting enzyme inhibitors and cyclosporine in the treatment of childhood nephrotic syndrome. The experience with these agents is briefly reviewed, but the current data are inadequate to indicate their role(s) in this condition.