An international consensus approach to the management of atypical hemolytic uremic syndrome in children
Reference: Loirat C, Fakhouri F, Ariceta G, et al. Pediatr Nephrol. (2016). doi: 10.1007/s00467-015-3076-8
Core Recommendations:
- 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.
- 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.
- 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.
- 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.
- 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.