ERKNet

The European Rare Kidney Disease Reference Network

  

Thematic Area:  Atypical Hemolytic Uremic Syndrome

 
The following guidance documents are endorsed by the TMA Workgroup based on standardized reviews.

1) An international consensus approach to the management of atypical hemolytic uremic syndrome in children.   

Pediatr Nephrol  2016; 31:15-39

 

Core Recommendations:
  1. For children with a clinical diagnosis of aHUS, we propose eculizumab as first-line treatment, to avoid plasma exchange and the complications of central venous double line catheters.
  2. In such cases, confirmation of a complement mutation is not required prior to treatment initiation. Undue treatment delay may affect ultimate renal recovery and increase the risk of early progression to end-stage renal disease.
  3. When possible, eculizumab treatment should be initiated within 24–48 h of onset or admission.
    If eculizumab is not (or not immediately) available, plasma exchange (or plasma infusions if plasma exchange is not possible) should be started.
  4. While the indication of complement blockade treatment is not limited to aHUS patients with a confirmed complement mutation as this treatment is also effective in patients without complement mutation, genetic screening is required for the longer-term management of patients.
  5. Anti-CFH antibody testing is the only complement investigation urgently needed during the acute phase, as a positive result raises additional treatment options.
 
Comments by Evaluators:
  • Clear, easy to read, addresses specific management questions.
  • Authors preferred a narrative approach rather than a more quantifiable methodology
  • Little emphasis given to methodology description
  • No stakeholders other than physicians/scientists involved. 

 

 

2) Atypical hemolytic uremic syndrome and C3 glomerulopathy:  Conclusions from a KDIGO Controversies Conference.   
     Kidney Int  2017; 91:539-551

 

Core Recommendations:  

(Narrative format) 

 
Comments by Evaluators:
  • Well written and clear expert recommendations, but no systematic review
  • Methods used to search evidence are not mentioned
  • Authors identidy knowledge gaps but do not report on strengths and limitations of available body of evidence