Congenital anomalies of the kidney and urinary tract (CAKUT) are the commonest cause of paediatric chronic kidney disease, accounting for 20-30% of prenatal anomalies (birth prevalence about 3.83 per 1000), with kidney dysplasia/hypoplasia and lower urinary tract obstruction the main drivers of CKD.
About a fifth of cases are monogenic (notably HNF1B and PAX2), so the review argues for targeted genetic testing in severe, syndromic, familial or bilateral disease. Kidney damage progresses slowly, but the peak need for kidney replacement therapy falls in adolescence and the average start is around age 31, and these patients carry raised cardiovascular, metabolic and neurocognitive risk.
The practice shift is to manage CAKUT as a planned life-course condition: targeted genetics where it changes management, proactive CKD and cardiometabolic care through childhood and adolescence, and structured transition into adult nephrology timed to that adolescent-to-young-adult window of rising kidney-failure risk.
- CAKUT is the commonest cause of paediatric CKD (20-30% of prenatal anomalies).
- About a fifth of cases are monogenic, often HNF1B or PAX2.
- Kidney replacement need peaks in adolescence; average start is around age 31.
- Offer targeted genetic testing in severe, syndromic, familial or bilateral disease.
- Plan proactive CKD, cardiometabolic care and structured adult transition.
Why it matters
It reframes a paediatric diagnosis as a decades-long trajectory that adult nephrology must be ready to receive.
Don't overread it
This is a narrative review; the care model is consensus guidance rather than a tested intervention.
Read the rest in the app
You have read your two free briefings this month. The app carries all 27 specialties, every morning, free — and this finding is waiting in it.
Scan to keep reading on your phone. No account needed to start.
Tomorrow morning, before your first patient
One edition a day for nephrology, written by the desk, every claim tied to its paper. Six minutes.
Get the app — free