Management of childhood nephrotic syndrome in Australia
EM Hodson, NS Willis, JC Craig
For the Australian and New Zealand Paediatric Nephrology Association
through the Australian Paediatric Surveillance Unit (APSU)
Centre for Kidney Research, The Children's Hospital at Westmead, Locked Bag 4001, Westmead, NSW 2145.
Introduction. Childhood nephrotic syndrome (NS) is a rare (1 per 100,000)
but important disease with a risk of relapse (70%), infection (10%) and thrombosis
(2%). A systematic review of randomised controlled trials has shown that daily
prednisone for 4-6 weeks, followed by 6-7 months of a tapering dose of prednisone,
reduces the risk of relapse by 60% compared with shorter courses of corticosteroid
therapy following the first episode of NS without increase in steroid toxicity.
This study was done to determine how children with their first episode of NS
are treated in Australia.
Methods. NS has been listed on the APSU card since July 1998 and it will
be listed for 3 years. Initial and one year follow up questionnaires are sent
to all doctors who report a case.
Results. Between July 1998 and August 2000, 96 new cases of idiopathic
NS in children aged 0.25 to 15 years were reported. 91% (87) children were treated
with daily prednisolone or prednisone at a dose of 60mg/m2/day or 2mg/kg/day;
70% (67) children received one dose per day, 26% (25) received two doses per
day and 4% (4) received three doses per day. In 40% (37) children, it was planned
to give corticosteroids daily for 2-3 weeks (4) or until the child achieved
remission (33). In the remaining children, the planned duration of daily corticosteroids
was 4 weeks (41%,39), 6 weeks (10), 8 weeks (4), and 12 weeks (4). Antibiotic
prophylaxis was given to 55% (53) children; of these 39 received penicillin.
Of 87 children aged 2 years or more, 28% (24) received pneumococcal vaccination;
17 were nephrotic and 18 on daily steroids when the vaccine was given. Albumin
and diuretics were given to 29% (28) children; 5 received diuretics alone and
9 albumin alone. 30% children (29) received aspirin.
Conclusions. While two-thirds of Australian children with NS received
daily prednisone for 4 weeks or more, one-third received shorter periods of
daily steroids thus increasing their risk for relapse. Better systems are required
to inform clinicians about the existing trial evidence for the use of corticosteroids
in nephrotic syndrome to assist them with their decision making.
Presented at the Royal Australasian College of Physicians Annual Scientific Meeting, Sydney, May 2001.
Correspondence
Dr Elisabeth Hodson
ElisaH@chw.edu.au