Polycystic kidney disease

Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited renal disorder and monogenic cause of hypertension. ADPKD should be considered in any patient presenting with bilateral renal cysts particularly if there is associated hypertension. Although treatment remains mainly supportive (particularly during the progression of chronic kidney disease), effective management of associated hypertension and other cardiovascular risk is important. Several potential therapies have emerged from research into the molecular biology of ADPKD, and one drug, tolvaptan, is now licensed for the treatment of later stages of ADPKD. The practical management of patients with this disorder can be challenging and may involve a coordinated multidisciplinary team approach to the medical and surgical complications associated with ADPKD. This chapter will briefly outline the current understanding of ADPKD and consider some of the practical dilemmas faced when caring for these patients.