Cochrane review: Corticosteroid therapy for nephrotic syndrome in children
Authors: EM Hodson , JF Knight , NS Willis , JC Craig 
Published: December 2006

Background

In nephrotic syndrome protein leaks from the blood to the urine through the glomeruli resulting in hypoproteinaemia and generalised oedema. While the majority of children with nephrotic syndrome respond to corticosteroids, 70% experience a relapsing course. Corticosteroid usage has reduced the mortality rate to around 3%, however they have known serious adverse effects.

Objectives

To determine the benefits and harms of corticosteroid regimens in preventing relapse in children with steroid sensitive nephrotic syndrome (SSNS).

Search strategy

We searched the Cochrane Central Register of Controlled Trials (CENTRAL), Cochrane Renal Group Specialised Register, MEDLINE and EMBASE without language restriction, reference lists of articles, abstracts from conference proceedings and contact with known investigators. Date of most recent search: October 2004

Selection criteria

Randomised controlled trials performed in children (three months to 18 years) in their initial or subsequent episode of SSNS, comparing different durations, total doses or other dose strategies using any corticosteroid agent, with outcome data at six months or more.

Data collection and analysis

Two reviewers independently assessed trial quality and extracted data. Statistical analyses were performed using a random effects model and results expressed as relative risk (RR) with 95% confidence intervals (CI).Meta‐regression was used to explore potential between‐study differences due to baseline risk of relapse, study quality and interventions.

Main results

Nineteen trials were identified. Six trials comparing two months of prednisone with three months or more in the first episode showed longer duration significantly reduced the risk of relapse at 12 to 24 months (RR 0.70; 95% CI 0.58 to 0.84). There was an inverse linear relationship between treatment duration and risk of relapse (RR = 1.26 ‐ 0.112 duration; P = 0.03). There was a significant reduction in the number of frequent relapsers and the mean relapse rate/patient/year. Deflazacort was significantly more effective in maintaining remission than prednisone in children who frequently relapsed (RR 0.44; 95% CI 0.25 to 0.78). There were no increases in adverse events.

Authors' conclusions

Children in their first episode of SSNS should be treated for at least three months with an increase in benefit being demonstrated for up to seven months of treatment For a baseline risk for relapse following the first episode of 60% with two months of prednisone, daily prednisone for four weeks followed by alternate‐day therapy for six months would reduce the number of children relapsing by 33%. Deflazacort deserves further study for frequent relapsers.

Plain lanquage summary

Corticosteroid drugs for several months after a child has nephrotic syndrome can reduce repeat episodes.

Nephrotic syndrome is a condition where the kidneys leak protein from the blood into the urine. Most children who experience this syndrome have repeat episodes. When it is untreated, children can often die from infections. Corticosteroid drugs are used to reduce these infections, but the drugs can also have serious side effects. The review of trials found that using corticosteroids for several months after the first episode has an increasing ability to reduce the risk of relapses, without an increase in serious side effects.