title: Spanish guidelines for the management of autosomal dominant polycystic kidney disease.
abstract: Autosomal dominant polycystic kidney disease (ADPKD) is the most frequent cause of genetic renal disease and accounts for 6-10% of patients on renal replacement therapy (RRT). Very few prospective, randomized trials or clinical studies address the diagnosis and management of this relatively frequent disorder. No clinical guidelines are available to date. This is a consensus statement presenting the recommendations of the Spanish Working Group on Inherited Kidney Diseases, which were agreed to following a literature search and discussions. Levels of evidence found were C and D according to the Centre for Evidence-Based Medicine (University of Oxford). The recommendations relate to, among other topics, the use of imaging and genetic diagnosis, management of hypertension, pain, cyst infections and bleeding, extra-renal involvement including polycystic liver disease and cranial aneurysms, management of chronic kidney disease (CKD) and RRT and management of children with ADPKD. Recommendations on specific ADPKD therapies are not provided since no drug has regulatory approval for this indication.
authors.affiliation: Molecular Biology Laboratory, Fundació Puigvert, Instituto de Investigaciones Biomédicas Sant Pau (IIB-Sant Pau), Universitat Autònoma de Barcelona, REDinREN, Instituto de Investigación Carlos III, Barcelona, Spain.
authors.forename: Elisabet
authors.initials: E
authors.lastname: Ars
authors.affiliation: Nephrology Department, Hospital de la Princesa, REDinREN, Madrid, Spain.
authors.forename: Carmen
authors.initials: C
authors.lastname: Bernis
authors.affiliation: Paediatric Nephrology, Hospital de la Santa Creu i Sant Pau, Universitat Autònoma de Barcelona, Barcelona, Spain.
authors.forename: Gloria
authors.initials: G
authors.lastname: Fraga
authors.affiliation: Nephrology Department, Hospital Reina Sofía, Murcia, Spain.
authors.forename: Víctor
authors.initials: V
authors.lastname: Martínez
authors.affiliation: Nephrology Department, Hospital Universitario de Getafe, Universidad Europea de Madrid, Madrid, Spain.
authors.forename: Judith
authors.initials: J
authors.lastname: Martins
authors.affiliation: Nephrology Department, IIS-Fundacion Jiménez Diaz, Universidad Autónoma de Madrid, IRSIN, REDinREN, Madrid, Spain.
authors.forename: Alberto
authors.initials: A
authors.lastname: Ortiz
authors.affiliation: Nephrology Department, Hospital Universitario de Gran Canaria Dr. Negrín, Universidad de Las Palmas de Gran Canaria, Las Palmas de Gran Canaria, Spain.
authors.forename: José Carlos
authors.initials: JC
authors.lastname: Rodríguez-Pérez
authors.affiliation: Nephrology Department, REDinREN, Hospital del Mar, Barcelona, Spain.
authors.forename: Laia
authors.initials: L
authors.lastname: Sans
authors.affiliation: Inherited Kidney Diseases, Nephrology Department, Fundació Puigvert, Instituto de Investigaciones Biomédicas Sant Pau (IIB-Sant Pau), Universitat Autònoma de Barcelona, REDinREN, Instituto de Investigación Carlos III, Barcelona, Spain.
authors.forename: Roser
authors.initials: R
authors.lastname: Torra
mesh_terms: D006801:Humans; D016891:Polycystic Kidney, Autosomal Dominant / Q000175:diagnosis* / Q000628:therapy*; D017582:Renal Replacement Therapy; D013030:Spain
keywords: ADPKD; autosomal dominant polycystic kidney disease; guidelines; management; recommendations