Table of contents
Atypical hemolytic uremic syndrome
Background
Overview
Definition
aHUS is a complement-mediated disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure.
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Pathophysiology
aHUS is most commonly caused by dysregulation of the alternative complement pathway.
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Disease course
The failure of complement regulation results in aHUS, which presents with clinical manifestations of microangiopathic hemolytic anemia, thrombocytopenia, and renal failure. Extra-renal manifestations include altered consciousness, seizures, focal neurologic deficits, prodromic diarrhea, hypertension, and malaise. Disease progression may lead to ESRD.
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Prognosis and risk of recurrence
aHUS is associated with an overall mortality rate of 25%.
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Guidelines
Key sources
The following summarized guidelines for the evaluation and management of atypical hemolytic uremic syndrome are prepared by our editorial team based on guidelines from the Korean Working Group on Atypical Hemolytic Uremic Syndrome (KHWG 2016) and the British Society for Haematology (BSH 2010).
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Screening and diagnosis
Diagnostic investigations
ADAMTS13 activity: as per KHWG 2016 guidelines, obtain tests for ADAMTS13 activity to exclude TTP in all patients suspected of having aHUS.
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More topics in this section
Complement testing
Shiga toxin test
Anti-factor H antibodies
Plasma homocysteine, methionine, and MMA
Other laboratory tests
FACS analysis
Genetic testing