Atypical haemolytic uraemic syndrome: a case report of a rare cause of reversible cardiomyopathy.

ABSTRACT:

Background

Atypical haemolytic uraemic syndrome (aHUS) is a life-threatening, genetic disease of complement-mediated thrombotic microangiopathy that typically presents as anaemia, thrombocytopenia, and renal failure. Cardiomyopathy is seen in up to 10% of aHUS cases, but the aetiology is not well-understood.

Case summary

A 63-year-old man recently was diagnosed with a thrombotic microangiopathy most consistent with aHUS by renal biopsy after presentation with acute renal failure requiring haemodialysis. He was started on therapy with complement inhibitor, eculizumab. Six weeks after diagnosis, he presented with progressive dyspnoea on exertion and chest pain. An echocardiogram demonstrated an acute drop in left ventricular ejection fraction to 20-25% with global hypokinesis.

SUBMITTER: Campbell CM 

PROVIDER: S-EPMC7180527 | biostudies-literature | 2020 Apr

REPOSITORIES: biostudies-literature