A review in Kidney International (22 September 2026) follows congenital anomalies of the kidney and urinary tract (CAKUT) from prenatal detection into adult life. CAKUT accounts for 20-30% of prenatal anomalies, with a birth prevalence of about 3.8 per 1,000, and kidney dysplasia or hypoplasia and lower urinary tract obstruction are the commonest causes of CKD in children.
About a fifth of cases have a genetic cause — notably HNF1B and PAX2 variants and copy number variants, especially in syndromic disease. Kidney decline is usually slow: the need for kidney replacement therapy peaks in adolescence, but the average age at starting it is about 31. Adults with CAKUT carry raised cardiovascular and metabolic risk, neurocognitive impairment and a small increase in malignancy.
The authors argue for genetic testing in severe, syndromic, familial or bilateral disease and in idiopathic CKD, and for care that follows the patient across the transition from paediatric to adult services.
For the adult nephrologist, the practical point is that CAKUT is often an adult disease with a paediatric origin — and the young adult with 'unexplained' CKD may have it.
- Ask young adults with unexplained CKD about childhood urinary tract infections, reflux or antenatal renal findings
- Consider genetic testing in bilateral or cystic dysplasia, syndromic features, family history or idiopathic CKD
- Screen HNF1B-related disease for diabetes, hypomagnesaemia and raised liver enzymes
- Manage cardiovascular and metabolic risk actively in adults with CAKUT
- Plan a structured transition from paediatric to adult nephrology
Why it matters
Many patients with congenital kidney disease reach kidney failure as adults, under clinicians who may not know the diagnosis.
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