Activity Description / Statement of Need:
In this online CME self-learning program:
Atypical hemolytic uremic syndrome (aHUS) is a disease in which the complement system is activated in an uncontrolled manner outside the presence of coexisting disease, resulting in platelet activation, damage to endothelial cells, and a range of clinical sequelae including: which may ultimately lead to kidney failure; systemic thrombotic microangiopathy;anemia; and thrombocytopenia.The disease may arise from any of a number of genetic mutations of the complement system or the presence of anti-complement factor H. While it is estimated to occur at an equally commonly between males and females, the disease is rare with an estimated incidence of about 2 people per million in the U.S. and seven per million in Europe.
Target Audience:
The following healthcare professionals: hematologists, nephrologists, and primary care physicians; physician assistants, nurse practitioners, nurses, and pharmacists specializing in hematology and transplant medicine; and any other healthcare professionals with an interest in or who may clinically encounter patients with aHUS.
This activity is supported by an educational grant from Alexion
This activity is free of charge
Release Date: July 10, 2019 — Expiration Date: July 10, 2021
Faculty: Ramy Hanna, MD
Agenda
Introduction
Epidemiology
Current statistics
Risk and causes
Pathophysiology and diagnosis, future complications [Learning objectives #1 and #2]
Clinical features
Microangiopathic hemolytic anemia, thrombocytopenia, acute kidney injury, abdominal pain, fatigue, nausea/vomiting
Diagnostic methods
Clinical presentation, screening for mutations and antibodies to complement proteins, screening for Shiga toxin-producing E. coli
Treatment [Learning objectives #3 and #4]
Present therapy
Eculizumab
Plasma exchange or infusion
Renal or combination renal-hepatic transplantation
Supportive care
Investigational therapies
Ravulizumab
Patient case(s)
Summary, conclusions, and best practice recap
Learning Objectives
By the end of the session the participant will be able to:
Describe the pathophysiology of aHUS such that it might informs pairing with present treatment mechanisms
Describe aHUS diagnostic methods, differential diagnosis, and the benefits of earlier diagnosis
Describe available therapies used for treatment of aHUS and summarize the literature supporting use of those therapies
Design an evidence-based treatment plan for a patient with aHUS
