{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T12:16:17Z","timestamp":1791288977112,"version":"4.3.0"},"reference-count":45,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2016,8,23]],"date-time":"2016-08-23T00:00:00Z","timestamp":1471910400000},"content-version":"vor","delay-in-days":22,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Internal Medicine Journal"],"published-print":{"date-parts":[[2016,8]]},"abstract":"<jats:p>Autosomal\u2010dominant polycystic kidney disease (<jats:styled-content style=\"fixed-case\">ADPKD<\/jats:styled-content>) is the most common genetic renal disease in adults, affecting one in every 1000 Australians. It is caused by loss\u2010of\u2010function heterozygous mutations in either <jats:italic>\n<jats:styled-content style=\"fixed-case\">PKD1<\/jats:styled-content>\n<\/jats:italic> or <jats:italic>\n<jats:styled-content style=\"fixed-case\">PKD2<\/jats:styled-content>\n<\/jats:italic>, which encode the proteins, polycystin\u20101 and polycystin\u20102 respectively. The disease hallmark is the development of hundreds of microscopic fluid\u2010filled cysts in the kidney during early childhood, which grow exponentially and continuously through life at varying rates (between 2% and 10% per year), causing loss of normal renal tissue and up to a 50% lifetime risk of dialysis\u2010dependent kidney failure. Other systemic complications include hypertensive cardiac disease, hepatic cysts, intracranial aneurysms, diverticular disease and hernias. Over the last two decades, advances in the genetics and pathogenesis of this disease have led to novel treatments that reduce the rate of renal cyst growth and may potentially delay the onset of kidney failure. New evidence indicates that conventional therapies (such as angiotensin inhibitors and statins) have mild attenuating effects on renal cyst growth and that systemic levels of vasopressin are critical for promoting renal cyst growth in the postnatal period. Identifying and integrating patient\u2010centred perspectives in clinical trials is also being advocated. This review will provide an update on recent advances in the clinical management of <jats:styled-content style=\"fixed-case\">ADPKD<\/jats:styled-content>.<\/jats:p>","DOI":"10.1111\/imj.13143","type":"journal-article","created":{"date-parts":[[2016,8,24]],"date-time":"2016-08-24T00:30:47Z","timestamp":1471998647000},"page":"883-892","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":29,"title":["Recent advances in autosomal\u2010dominant polycystic kidney disease"],"prefix":"10.1111","volume":"46","author":[{"given":"G. K.","family":"Rangan","sequence":"first","affiliation":[{"name":"Department of Renal Medicine Westmead Hospital, Western Sydney Local Health District  Sydney New South Wales Australia"},{"name":"Centre for Transplant and Renal Research, Westmead Institute\u2009for Medical Research University of Sydney  Sydney New South Wales Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M. C.","family":"Tchan","sequence":"additional","affiliation":[{"name":"Department of Genetic Medicine Westmead Hospital, Western Sydney Local Health District  Sydney New South Wales Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A.","family":"Tong","sequence":"additional","affiliation":[{"name":"Centre for Kidney Research The Children's Hospital at Westmead  Sydney New South Wales Australia"},{"name":"Sydney School of Public Health University of Sydney  Sydney New South Wales Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A. T. Y.","family":"Wong","sequence":"additional","affiliation":[{"name":"Centre for Transplant and Renal Research, Westmead Institute\u2009for Medical Research University of Sydney  Sydney New South Wales Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"B. 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