Macrovascular involvement in a child with atypical hemolytic uremic syndrome
2014
Background
Atypical hemolytic uremic syndrome(aHUS) is a disorder of the
complement systemwhich leads to
thrombotic microangiopathy. It is caused by either acquired or hereditary defects in the activation or regulation of the
alternative complement pathwayand is therefore considered to be a disease of local
complementdysregulation in microvasculature with predominantly renal involvement. However, extrarenal manifestations are observed in approximately one-fifth of aHUS patients, with the myocardium and central nervous system (CNS) being involved most often. Additionally, there have been a few reports of aHUS with cerebral artery stenoses or periphereal
gangrene, suggesting the possibility of ‘macrovascular’ involvement in aHUS.
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