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Autosomal Dominant Polycystic Kidney Disease: A Canadian Expert Consensus

Why is this review important

This consensus review was done to develop evidence-informed recommendations for Canadian nephrologists to guide optimal management of adult patients with ADPKD.

Publication information:

This publication appeared in the Canadian Journal of Kidney Health and Disease

For the original publication, please visit this link or download directly here .

What are the key messages

  1. Based on existing data, genetic testing is only required for a subset of patients with ADPKD, and it is not needed prior to initiating treatment options.
  2. Although the gold standard for imaging is MRI, other less costly and more readily available imaging modalities should be used in routine clinical practice for diagnosis and determination of risk of progression.
  3. Blood pressure targets and ADPKD-specific treatment options are recommended for patients who fulfill specific criteria.
  4. We recommend treatment with tolvaptan for patients who fulfill the enrollment criteria of the Tolvaptan Efficacy and Safety in Management of Autosomal Dominant Polycystic Kidney Disease and its Outcomes (TEMPO) 3:4 study: 18 to 50 years of age, Cockcroft-Gault GFR >60 mL/min, and TKV >750 mL. In the absence of Cockcroft-Gault GFR, Chronic Kidney Disease Epidemiology Collaboration (CKD-EPI) >45 mL/min may be used, and in the absence of TKV, ultrasound (US) kidney length (KL) >16.5 cm may be used.
  5. We suggest treatment with tolvaptan for patients who, according to the Mayo Classification, are classified as 1D or 1E with eGFR in CKD stage 3 or higher. Treatment with tolvaptan should be considered for patients who are classified as 1C and are younger than 50 years or have other risk factors for rapid progression.

This text is an excerpt of the original publication