Sushmita Banerjee1. 1. Department of Pediatrics, Calcutta Medical Research Institute, Kolkata, India. asban@vsnl.com
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.
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.