Autosomal dominant polycystic kidney disease (ADPKD) is one of the most frequent genetic diseases, affecting an estimated 12.5 million people worldwide.  Despite its importance, approaches to the diagnosis, evaluation, prevention and treatment of the renal and extrarenal manifestations of ADPKD vary widely and no widely accepted practice guidelines exist for this disease.  As such, KDIGO held a Controversies Conference on ADPKD in Edinburgh, United Kingdom on January 17-19, 2014.  Drs. Vicente E. Torres (Mayo Clinic, USA) and Olivier Devuyst (University of Zurich, Switzerland) co-chaired this conference.

The aim of this KDIGO conference was to gather a global panel of multi-disciplinary clinical expertise that identified key issues relevant to the progression factors, mortality, co-morbidity, as well as patient support and health economical issues of ADPKD.  The objective of this conference was to assess our current state of knowledge related to the evaluation, management and treatment of ADPKD, to summarize the outstanding knowledge gaps, and to propose a research agenda to resolve standing controversial issues.  This conference served to inform whether there is sufficient evidence base for the development of a guideline on this topic and helped pave the way to harmonize and standardize the care of ADPKD patients.

Conference Scope of Work

Conference Agenda

Conference Roster

Breakout Session Discussion Questions

Executive Summary

Full Conference Report


Will introduction of tolvaptan change clinical practice in autosomal dominant polycystic kidney disease?
Author: Shigeo Horie

Plenary Session Presentations

Conference Overview & Objectives
Presenters: Olivier Devuyst & Vicente Torres (Conference Co-Chairs)

Evolution of Imaging-­Based Diagnosis
Presenter: York Pei

Present & Future Role of Molecular Genetic Diagnostics
Presenter: Peter Harris

Pre-Implantation Genetic Diagnosis and Childhood Diagnostic Evaluation
Presenter: Carsten Bergmann

Patient Perspective: Counseling & Diagnostic Implications
Presenter: Dwight Odland

What Endpoints Should Be Used in Clinical Trials for ADPKD?
Presenter: Arlene Chapman

Innovations in Evaluation & Management of Renal Complications in ADPKD
Presenter: Shigeo Horie

Unique Features of Hypertension in ADPKD
Presenter: Robert Schrier

Are Prevention of Decline in Renal Function & Cardiovascular Complications Interconnected in ADPKD?
Presenter: Tevfik Ecder

Patient Perspective: Dietary Management, Lifestyle Adaptations, Psychological & Social Support
Presenter: Tess Harris

Living Donor Evaluation, Unique Aspects of the Evaluation, and Management of ADPKD Transplant Recipients
Presenter: Klemens Budde

Choice of Modality & Management of Dialysis in ADPKD Patients
Presenter: Ahsan Alam

Evaluation & Management of Polycystic Liver Disease
Presenter: Joost Drenth

Screening & Management of Intracranial Aneurysms and Other Cardiovascular Associations
Presenter: Yves Pirson