ONE YEAR FOLLOW-UP OF CHILDREN WITH IDIOPATHIC NEPHROTIC SYNDROME

Hodson EM, Willis NS, Craig JC and members of Australian and New Zealand Paediatric Nephrology Association through the Australian Paediatric Surveillance Unit (APSU).
The Centre for Kidney Research, Childrens Hospital at Westmead, Locked Bag 4001, Westmead, NSW 2145

To determine the incidence, demography, initial management and one year follow-up of childhood nephrotic syndrome (NS) in Australia, children with newly diagnosed NS were identified through the APSU from July 1998 to June 2001. Of 135 children, with idiopathic NS, 1 year follow-up data were available on 93 children at December 2001; one child was lost to follow up. Of 92 children, 21 (23%) had steroid resistant idiopathic NS (SRINS); 11 had FSGS, 4 had minimal change, 4 had membranous glomerulonephritis and 2 had mesangial proliferative glomerulonephritis. In 72 children, with steroid sensitive NS (SSINS), 56 (78%) received daily steroids for 1-32 weeks (median 4 weeks) after remission. After daily steroids, 65 (90%) children received alternate day steroids for 1-30 weeks (median 6 weeks. Total duration of steroid therapy after remission was 2-30 weeks (median 11 weeks); 34 (47%) received steroids for 12 weeks or more. The median duration of daily steroid therapy before first remission was 10 days (range 3-66 days). At 1 year, 15 (21%) children had not relapsed and 29 children (40%) relapsed infrequently ( 1 relapse in first 6 months or < 4/year); median number of relapses 1;range 1-3. Sixteen children (22%) relapsed frequently ( relapses in first 6 months or 4/year); median number of relapses 3;range 2-6. Twelve children (17%) were steroid dependent (relapse on steroids or within 2 weeks of cessation); median number of relapses 3; range 2-5. Three children with SRINS developed chronic renal failure. Nine serious infections occurred in 8 (9%) children (2 with SRINS). No child developed any thromboses. Management of SSINS varied markedly despite data from randomised controlled trials showing that 12 weeks or more of steroid therapy reduces the risk for relapse compared with 8 weeks.

Presented at the Australian and New Zealand Society of Nephrology, Annual Meeting, Sydney Convention Centre, 2 - 4 Sept 2002 Sydney NSW.

Correspondence
Elizabeth Hodson
Centre for Kidney Research
The Childrens Hospital at Westmead
Locked Bag 4001
Westmead NSW 2145 Sydney Australia
Tel: +61 2 9845 3430
Email1:Elizabeth Hodson