Extra-renal manifestations of autosomal dominant polycystic kidney disease (ADPKD): considerations for routine screening and management.
Autosomal-dominant polycystic kidney disease (ADPKD) is a systemic disease, marked by progressive increase of bilateral renal cysts, resulting in chronic kidney disease (CKD) and often leading to end-stage renal disease (ESRD). Apart from renal cysts, patients often have extra-renal disease, involving the liver, heart and vasculature. Other less common but equally important extra-renal manifestations of ADPKD include diverticular disease, hernias, male infertility and pain. Extra-renal disease burden is often asymptomatic, but may result in increased morbidity and mortality. If the disease burden is significant, screening may prove beneficial. We review the rationale for current screening recommendations and propose some guidelines for screening and management of ADPKD patients.
Section of Nephrology, Yale University School of Medicine, New Haven, CT, USA.
Randy L
RL
Luciano
Neera K
NK
Dahl
D002318:Cardiovascular Diseases / Q000175:diagnosis*; D002493:Central Nervous System Diseases / Q000175:diagnosis*; D004066:Digestive System Diseases / Q000175:diagnosis*; D019468:Disease Management; D020022:Genetic Predisposition to Disease; D005820:Genetic Testing / Q000379:methods; D014943:Global Health; D006801:Humans; D008297:Male; D008403:Mass Screening / Q000379:methods*; D009017:Morbidity; D016891:Polycystic Kidney, Autosomal Dominant / Q000175:diagnosis* / Q000628:therapy*; D015996:Survival Rate
ADPKD; extra-renal; screening