Hemolytic Uremic Syndrome.

ABSTRACT

Context Hemolytic uremic syndrome (HUS) is a severe acute disease, sometimes with long-term sequelae. The diarrhoea-unrelated forms are particularly associated with a poor prognosis. The aim of this paper is to review current evidence regarding etiology and management, and explore methods by which the outcome may be optimized. Evidence acquisition An internet search of Medline, Medscape, MDConsult and Cochrane databases for publications related to HUS from 1998 onwards was performed. A review of articles pertaining to etiopathogenesis and management was undertaken. Results: HUS is now classified according to cause. New assays and gene studies allow exact diagnosis of many of the atypical forms. Post-exposure prevention of diarrhoea associated HUS with vaccines and toxin-binding agents, remains in the experimental stages. Specific directed therapies aimed at replacing deficient factors can improve the outcome of atypical HUS. Conclusions: Supportive care remains the cornerstone of management of HUS. The infection-unrelated forms should in addition be treated rapidly with plasma therapy. Efforts should be made to make an exact etiological diagnosis in all patients, as long-term treatment and prognosis is affected. Prevention of diarrhea-associated HUS by improving sanitation and proper attention to food hygiene is a practical goal.