Authors: Karolis Ažukaitis Chantal Loirat Michal Malina Irina Adomaitienė Augustina Jankauskienė
Publish Date: 2013/12/19
Volume: 29, Issue: 7, Pages: 1273-1277
Abstract
Atypical hemolytic uremic syndrome aHUS is a disorder of the complement system which leads to thrombotic microangiopathy It is caused by either acquired or hereditary defects in the activation or regulation of the alternative complement pathway and is therefore considered to be a disease of local complement dysregulation in microvasculature with predominantly renal involvement However extrarenal manifestations are observed in approximately onefifth 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 aHUSWe present a child with early onset aHUS and a C3 gainoffunction mutation who developed cerebral artery stenoses leading ultimately to death due to a massive stroke 9 days after successful renal transplantation under prophylactic eculizumab treatment Similar cases described in the literature are also briefly summarizedThe disease course in our patient with aHUS confirms that dysregulated complement activation can induce arterial stenoocclusive lesions in the absence of acute episodes of HUS Further studies are required to document the frequency of such macrovascular complications and the role of eculizumab treatment in preventing their development and progression
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