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

Vicky Brocklebank | Zendy; Edwin K.S. Wong | Zendy; R Fielding | Zendy; Timothy H.J. Goodship | Zendy; David Kavanagh | Zendy

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

Author(s) -

Vicky Brocklebank,

Edwin K.S. Wong,

R Fielding,

Timothy H.J. Goodship,

David Kavanagh

Publication year - 2014

Publication title -

clinical kidney journal

Language(s) - English

Resource type - Journals

SCImago Journal Rank - 1.033

H-Index - 40

eISSN - 2048-8513

pISSN - 2048-8505

DOI - 10.1093/ckj/sfu032

Subject(s) - shigella flexneri , medicine , atypical hemolytic uremic syndrome , shigella dysenteriae , shigella , penetrance , eculizumab , shiga toxin , cd46 , haemolytic uraemic syndrome , complement system , microbiology and biotechnology , mutation , dysentery , immunology , virology , genetics , gene , biology , antibody , escherichia coli , phenotype , pathology

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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