This Kidney International review brings together long-term outcome data for idiopathic nephrotic syndrome beginning in childhood, the commonest chronic glomerular disease of children. The response to initial steroids remains the strongest predictor of what follows.
Steroid-sensitive disease usually relapses and remits, and about half of children develop frequent relapses or steroid dependence. The old teaching that most grow out of it by puberty is too optimistic: roughly a quarter have at least one relapse in adulthood, most often those who were frequent relapsers or needed second-line immunosuppression. Fewer than one in ten become steroid-resistant later, and kidney failure and death are uncommon. Steroid-resistant disease is different. Up to a third of initially resistant children have a monogenic cause, and the group carries risks of growth impairment, obesity, hypertension, osteoporosis, cataracts, calcineurin inhibitor toxicity and progressive CKD; kidney failure is three to four times more likely with monogenic or multidrug-resistant disease.
For adult nephrologists and physicians, the practical point is that a history of childhood nephrotic syndrome is not closed. Relapse in adulthood should be expected in some, and the cumulative toxicity of childhood treatment needs looking for.
- Ask about childhood nephrotic syndrome in young adults presenting with oedema or proteinuria.
- Frequent relapses or steroid dependence in childhood predict relapse in adulthood.
- Screen adults treated for childhood steroid-resistant disease for hypertension, bone loss, cataracts and CKD.
- Offer genetic testing in steroid-resistant nephrotic syndrome; up to a third of initially resistant children have a monogenic cause.
- Plan a structured transition from paediatric to adult nephrology care.
Why it matters
Adult clinicians inherit both the relapses and the cumulative steroid and calcineurin inhibitor toxicity.
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