Evidence-based clinical practice guidelines for polycystic kidney disease 2014

Origins of the guidelinesAutosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary kidney disease, with approximately half of the patients experiencing end-stage renal disease by age 60.Bilateral cysts progressively proliferate and enlarge, even as complications such as hypertension, hepatic cysts, and intracranial aneurysms lead to more lethal events such as cyst infections and ruptured intracranial aneurysms prior to end-stage renal disease.Early-stage diagnosis and intervention are recognized as being vital.Autosomal recessive polycystic kidney disease (ARPKD) is estimated to occur in 1 in 10,000-40,000 births, with symptoms present neonatally.Due to early detection and management as well as improvements in endstage renal disease treatment, long-term survival is currently possible in patients other than neonates with severe pulmonary hypoplasia.In Japan, Clinical Guidelines for Polycystic Kidney Disease in 1995 was published by the Progressive Renal Diseases Research, Research on intractable disease, from the Ministry of Health, Labour and Welfare of Japan, followed by a 2002 revision, the ADPKD Guidelines (second edition).Both serve as protocols for daily treatment of ADPKD in Japan.However, subsequent advancements in PKD expertise led to the 2010 Clinical Guidelines for Polycystic Kidney Disease, which were aimed at physicians and other health practitioners.These events provided