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Published on: August 9, 2014
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Kidney function and mortality in childhood nephrotic syndrome
Cal H Robinson1,2,3, Bess-Joan Ogome2, Nithiakishna Selvathesan1,2
1Department of Paediatrics, Division of Nephrology, The Hospital for Sick Children, Toronto, Ontario, Canada.
Summary
Children with steroid-sensitive nephrotic syndrome have low risks of kidney failure, chronic kidney disease (CKD), and death. However, children with steroid-resistant nephrotic syndrome face significantly higher risks, though these rates are declining.
Area of Science:
- Pediatric Nephrology
- Chronic Kidney Disease Epidemiology
- Childhood Kidney Diseases
Background:
- Nephrotic syndrome is a prevalent childhood kidney disease.
- Frequent relapses, acute kidney injuries, and immunosuppressive treatments can progress to chronic kidney disease (CKD).
Purpose of the Study:
- To determine the incidence of kidney failure, CKD, cancer, and mortality in children with idiopathic nephrotic syndrome.
- To compare outcomes between steroid-sensitive and steroid-resistant nephrotic syndrome.
Main Methods:
- A systematic literature search was conducted across five major databases (MEDLINE, EMBASE, CENTRAL, Web of Science, ClinicalTrials.gov) from 1970-2024.
- Included studies enrolled children with idiopathic nephrotic syndrome and reported incidence of kidney failure, CKD, cancer, or mortality.
- Meta-analyses were performed to calculate the incidence rate (IR) of outcomes, following PRISMA guidelines.
Main Results:
- 229 studies involving 32,712 children were included, with a median follow-up of 5.1 years.
- For steroid-sensitive nephrotic syndrome, incidence rates per 1000 person-years were: kidney failure 2.65, CKD 3.80, and mortality 2.30.
- For steroid-resistant nephrotic syndrome, incidence rates per 1000 person-years were significantly higher: kidney failure 33.34, CKD 46.90, and mortality 12.52. Cancer incidence was low (16 cases).
Conclusions:
- Children with steroid-sensitive nephrotic syndrome have low incidence rates (<4 per 1000 person-years) for kidney failure, CKD, and death.
- Steroid-resistant nephrotic syndrome is associated with substantially higher rates of CKD and death, although these are decreasing over time.
- Study limitations include moderate-to-high risk of bias and variability in outcome definitions and surveillance.
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