Published in:
Open Access
01-08-2016 | Guideline
Evidence-based clinical practice guidelines for polycystic kidney disease 2014
Authors:
Shigeo Horie, Toshio Mochizuki, Satoru Muto, Kazushige Hanaoka, Yoshimitsu Fukushima, Ichiei Narita, Kikuo Nutahara, Ken Tsuchiya, Kazuhiko Tsuruya, Koichi Kamura, Saori Nishio, Tatsuya Suwabe, Yoshifumi Ubara, Eiji Ishimura, Koichi Nakanishi, Keiichi Furukawa, Kenjiro Kimura, Seiichi Matsuo
Published in:
Clinical and Experimental Nephrology
|
Issue 4/2016
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Excerpt
Autosomal 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 end-stage renal disease treatment, long-term survival is currently possible in patients other than neonates with severe pulmonary hypoplasia. …
