ERKNet

The European Rare Kidney Disease Reference Network

  

Thematic Area:  Gitelman Syndrome

 
The following guidance documents have been adopted based on standardized reviews and are followed in all ERKNet centers:

Gitelman syndrome: consensus and guidance from a Kidney Disease: Improving Global Outcomes (KDIGO) Controversies Conference.  Kidney Int 2017; 91:24-33

 

Core Recommendations:
  1. Clinical diagnosis should be confirmed by genetic testing
  2. Genetic counseling should be offered
  3. Management includes liberal salt intake, as well as Mg and K supplements
  4. Limited evidence for the use of K-sparing diuretics. If done, concomitant salt supplementation should be considered
  5. Subnormal levels for plasma K and Mg levels can be accepted and concentrations of 3.0 mmol/l and 0.6 mmol/l for K and Mg, respectively, should be targeted, but may not be realistic in some patients with severe electrolyte wasting
 
Comments by evaluators:

This document is not a guideline, but a consensus expert opinion to provide guidance for clinical practice.