Atypical hemolytic-uremic syndrome in children

Abstract

Background: Atypical hemolytic-uremic syndrome (aHUS) is a rare progressive form of systemic thrombotic microangiopathy (TMA), which develops due to uncontrolled activation of the alternative pathway of the complement system and is characterized by a predominant lesion of the vessels of the renal microcirculatory bed.Clinical cases description: The article describes two clinical cases of aHUS, which demonstrate the difficulties of differential diagnosis of typical and atypical HUS at the initial stages of the disease.Conclusion: Given the lack of clear laboratory criteria of aHUS, it is necessary to carry out a complex and consistent differential diagnosis of various TMA options, the exclusion of which makes it possible to make a correct diagnosis more accurately.

Keywords

• atypical hemolytic-uremic syndrome
• children
• complement-mediated thrombotic microangiopathy
• acute kidney injury
• diagnosis
• clinical case