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Haemolytic Uraemic Syndrome

Haemolytic Uraemic Syndrome. Robson WLM. Paediatric Drugs 2000;2(4):243-252.


In an overview of hemolytic uremic syndrome (HUS) associated with E. coli O157:H7 infections, this report describes the history, epidemiology, laboratory identification, pathogenesis, clinical manifestations and management of this condition. Further, the prognosis, mortality estimates and long-term sequelae are discussed. Specifically, factors associated with a poor prognosis of HUS include young age, severe hemorrhagic colitis with rectal prolapse, neurological symptoms not caused by hyponatremia, multiple extrarenal system involvement, prolonged anuria, and an elevated white blood cell count. However, steady improvements in supportive care have improved the mortality rate from 100% to less than 5%. About 75% of children recover without any significant long-term sequelae. About 5% of affected children will sustain enough renal damage to eventually lead to end-stage renal disease (ESRD), chronic dialysis, and kidney transplantation. This typically occurs years after recovery from HUS. Proteinuria occurs in about 30% and hypertension in about 15% of children. A further 5% will have neurological sequelae such as paresis or a seizure disorder. Attention deficit hyperactivity disorder is reported more frequently in those who experience significant neurological involvement with the acute illness. Type 1 diabetes mellitus has been reported as a late complication in some patients who recover from pancreatitis associated with HUS in the acute phase. Gallstones are reported in 10% of HUS patients and are presumed to develop as a result of hemolytic anemia and parenteral nutrition. Due to the potential for long-term or late complications, lifetime follow-up is required for every child who experiences typical HUS.