Consensus document on autosomal dominant polycystic kindey disease from the Spanish Working Group on Inherited Kindey Diseases. Review 2020

Autosomal dominant polycystic kidney disease (ADPKD) is the most frequent cause of genetic renal disease and accounts for 6-10% of patients on kidney replacement therapy (KRT). Very few prospective, randomized trials or clinical studies address the diagnosis and management of this relatively frequen...

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Detalles Bibliográficos
Autores: Ars, Elisabet, Bernis, Carmen, Fraga, Gloria, Furlano, Mónica, Martínez, Víctor, Martins, Judith, Ortiz, Alberto, Pérez-Gómez, Maria Vanessa, Rodríguez-Pérez, José Carlos, Sans Atxer, Laia, Torra, Roser
Tipo de recurso: artículo
Estado:Versión publicada
Fecha de publicación:2022
País:España
Institución:Universitat Pompeu Fabra
Repositorio:Repositorio Digital de la UPF
OAI Identifier:oai:repositori.upf.edu:10230/56503
Acceso en línea:http://hdl.handle.net/10230/56503
http://dx.doi.org/10.1016/j.nefroe.2022.11.011
Access Level:acceso abierto
Palabra clave:ADPKD
Autosomal dominant polycystic kidney disease
Consensus
Management
Progression
Recommendations
Descripción
Sumario:Autosomal dominant polycystic kidney disease (ADPKD) is the most frequent cause of genetic renal disease and accounts for 6-10% of patients on kidney replacement therapy (KRT). 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 revised consensus statement from the previous 2014 version, 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 mostly are 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 KRT and management of children with ADPKD. Recommendations on specific ADPKD therapies are provided as well as the recommendation to assess rapid progression.