Affiliations: Department of Community Pediatrics, Alder Hey
Children's' Hospital NHS Foundation Trust, Eaton Road, Liverpool, UK | Department of Pediatrics, Doncaster and Bassetlaw NHS
Foundation Trust, Doncaster, UK
Note: [] Correspondence: Dr. Michael O. Ogundele, Department of
Community Pediatrics, Alder Hey Children's' Hospital NHS Foundation Trust,
Eaton Road, Liverpool, UK. Tel.: +44 1512933565; Fax: +44 1512525085; E-mail: m.ogundele@nhs.net
Abstract: The hemolytic uremic syndrome (HUS) is characterized by the triad of
thrombocytopenia, micro-angiopathic hemolytic anemia and acute renal failure.
This review provides a comprehensive overview of the existing literature on the
epidemiology, etiology, pathogenesis, clinical presentation, diagnosis and
management of HUS. The ongoing controversy regarding the role of antibiotics
during the acute enteric phase of the classical HUS and the challenges involved
in the management of the atypical diseases are highlighted. The most common
cause of HUS is systemic uptake of toxins, and develops in 5–10% of
children exposed to verocytotoxin-producing Escherichia coli O157:H7.
Children below the age of 5 years are the most susceptible hosts and the main
route of spread is by fecal-oral transmission. Other infective agents and
complement genetic abnormalities are responsible for 10% of cases, referred
to as atypical HUS. The characteristic pathologic findings in HUS are renal
endothelial swelling, thrombi in the arterioles and capillaries, and
subendothelial fibrin deposits. After 30 years of discovering Shiga toxins and
over 15 years after associating it with the development of HUS, there are
neither specific predictive factors to identify the most susceptible patients
after an infection with E. coli 0157:H7 nor specific treatment for HUS
once it has become established. Treatment of HUS is mainly supportive. The
introduction of specific toxin neutralizing agents has proven disappointing in
clinical practice. Recent trials of E. coli O157:H7 O-specific conjugated
polysaccharide vaccines appear promising in preventing the development of HUS.
