Abstract

A REVIEW ON ATYPICAL HEMOLYTIC UREMIC SYNDROME (aHUS)

*Dr. Subash Chandran M. P., Dr. Julia, Anju David Raj, Jeslin John, Pooja Sasi, Rizwana Muhammed

Abstract

Atypical hemolytic uremic syndrome (aHUS) is a rare complementmediated kidney disease that primarily affects kidney function. aHUS is mostly related to gene mutations, coupled with a triggering event. The pathogenesis revolves around the inherited defects in complement genes and autoantibodies against complement regulatory proteins. The syndrome has a high mortality rate therefore has to be diagnosed early also to limit target organ damage. Diagnosis includes a complete blood count (CBC) test. It will measure the red blood cell and platelet count since its low counts can be a sign of aHUS. Treatment mainly involved plasma-based therapy prior to the availability of Eculizimab. Eculizumab has revolutionised the management of aHUS.

Keywords: aHUS, complement regulatory proteins, Eculizumab, autoantibodies, CBC.