Atypical haemolytic uraemic syndrome associated with a CD46 mutation triggered by Shigella flexneri.

Vicky Brocklebank1, Edwin K S Wong2, Rick Fielding1

  • 1Renal Services Centre , Freeman Hospital , Newcastle upon Tyne , UK.

We present a case of haemolytic uraemic syndrome (HUS) triggered by Shigella flexneri. Of the Shigella species, only S. dysenteriae type 1 is said to produce Shiga toxin and consequently cause HUS. Investigation of the complement system in this patient revealed a CD46 mutation. In individuals with mutations in complement genes incomplete penetrance of atypical HUS (aHUS) is seen, suggesting that a trigger, such as infection, is required for disease to manifest. In an era of complement modulatory therapy for aHUS it is important to be alert to unusual presentations of diarrhoeal-associated disease.

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