Clinical Practice Guideline Monitoring children and young people with, or at risk of developing Autosomal Dominant Polycystic Kidney Disease (ADPKD)

Clinical Practice Guideline Monitoring children and young people with, or at risk of developing Autosomal Dominant Polycystic Kidney Disease (ADPKD) - The UK Kidney Association

This guideline makes recommendations for monitoring children and young people (CYP) up to 18 years of age with, or at risk of developing Autosomal Dominant Polycystic Kidney Disease (ADPKD).

ADPKD is the commonest inherited renal disease with an incidence of around 1 in 1000 and accounts for 5-7% of adults commencing renal replacement therapy (1.2). Whilst ADPKD has traditionally been thought of as an adult disease, with established renal failure tending to occur in or after the 6th decade, there is clear evidence of earlier manifestation in children and young people (CYP), in whom hypertension and proteinuria may accelerate progression to later stages of chronic kidney disease. There is wide variation in clinical practice facing CYP with confirmed or a family history of ADPKD, with regard to a) assessment of blood pressure and urine testing for the presence of proteinuria b) ultrasound testing to evaluate presence of cysts and c) genetic counselling and testing. In order to improve quality of care and reduce variation in practice, the British Association for Paediatric Nephrology (BAPN) and the UK Renal Association (RA) in collaboration with key partners, has undertaken this work to develop best practice guidance in the area.


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