title: Hepatic and renal manifestations in autosomal dominant polycystic kidney disease: a dichotomy of two ends of a spectrum.
abstract: Autosomal dominant polycystic kidney disease (ADPKD) is a multisystem disorder. It is the most common genetic cause of end-stage renal disease. One frequent extra-renal manifestation is hepatic cyst formation. The majority of ADPKD patients develop complications as a result of renal cyst formation&semi; however, a small proportion develop extensive hepatic disease with minor renal features. Both phenotypes seem to represent the spectrum of ADPKD. This review discusses the current understanding of the pathogenesis of the disease, its manifestations and the mechanisms of cyst formation. Furthermore, it focuses on monitoring the disease and the treatment options currently available.
authors: Department of Gastroenterology and Hepatology, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands.
authors: J J M
authors: JJ
authors: van Gulick
authors: T J G
authors: TJ
authors: Gevers
authors: L
authors: L
authors: van Keimpema
authors: J P H
authors: JP
authors: Drenth
mesh_terms: D006801:Humans; D006973:Hypertension / Q000209:etiology; D007676:Kidney Failure, Chronic / Q000209:etiology*; D008107:Liver Diseases / Q000209:etiology* / Q000628:therapy; D016891:Polycystic Kidney, Autosomal Dominant / Q000150:complications* / Q000175:diagnosis / Q000235:genetics / Q000628:therapy