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1

Diagnose aHUS

http://www.identifyahus.com/tma-differential-diagnosis

Severe ADAMTS13 deficiency (TTP. And atypical hemolytic uremic syndrome (aHUS) are both thrombotic microangiopathies (TMAs) but have different underlying causes. Severe ADAMTS13 deficiency occurs when the activity level of the von Willebrand factor (vWF) cleaving protein, ADAMTS13, decreases to 5%. AHUS is caused by genetic changes in, and autoantibodies to complement regulators, disrupting the delicate balance of complement pathway. 1,2,6,8,19,21,22. TTP = thrombotic thrombocytopenic purpura. Hematology...

2

About aHUS

http://www.identifyahus.com/about-ahus

Atypical hemolytic uremic syndrome (aHUS) is a disease characterized by chronic, uncontrolled complement activation leading to systemic thrombotic microangiopathy (TMA). AHUS is defined as a disease that manifests with the clinical characteristics of TMA (thrombocytopenia, microangiopathic hemolysis, and symptoms of organ dysfunction), an ADAMTS13 activity level 5% (which rules out severe ADAMTS13 deficiency [TTP]), and the absence of a positive Shiga-toxin-producing E coli. STEC) which rules out STEC-HUS.

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Site Map | Identify aHUS Home

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aHUS Resources

http://www.identifyahus.com/downloadable-resources

Is it TTP or is it aHUS? This guide provides information that is key to differentiating between aHUS and TTP, and includes a copy of the differential diagnosis pathway. ADAMTS13 Activity Testing (Flashcard). Learn how an ADAMTS13 activity assessment supports the accurate differential diagnosis of thrombotic microangiopathies (TMAs), and get important tips for accurate testing. AHUS Clinical Case Study: 20 year-old male. Take the diagnostic challenge. AHUS Clinical Case Study: 26 year-old female.

5

aHUS Interactive Case Study

http://www.identifyahus.com/interactive-case-study

Diagnosis of aHUS in a patient presenting with thrombocytopenia, microangiopathic hemolysis, and neurological symptoms. The patient case described herein is representative of physician experience and does not include actual patient data.

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