diagnosis, prognosis, and management of ahus
DESCRIPTION
Diagnosis, Prognosis, and Management of aHUS. Program Goals. TMA Disorders Are Now Classified by Molecular Mechanism. Hereditary Disorders Resulting in TMA. Acquired Causes of TMA. Complex Mechanisms May Contribute to Acquired TMA. Effects of Uncontrolled or Excessive Complement Activation. - PowerPoint PPT PresentationTRANSCRIPT
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Diagnosis, Prognosis, and Management of aHUS
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Program Goals
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TMA Disorders Are Now Classified by Molecular Mechanism
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Hereditary Disorders Resulting in TMA
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Acquired Causes of TMA
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Complex Mechanisms May Contribute to Acquired TMA
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Effects of Uncontrolled or Excessive Complement Activation
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Complement-Mediated TMA (aHUS) Can Affect Multiple Organs,
Tissues
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Extrarenal Manifestations Are Common in aHUS
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Keys to Diagnosing aHUS
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No Role for Complement-Level Testing in the Diagnosis of aHUS
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Challenges in Diagnosing aHUS
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Complement-Amplifying Conditions May Unmask aHUS
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Complement-Amplifying Conditions Commonly Unmask
aHUS
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aHUS and Complement-Amplifying Events
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Unmasking the Diagnosis of Complement-Mediated TMA
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Management Options for aHUS Have Been Limited
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Challenges in Evaluating PI/PE Therapy
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Response to Plasma Therapy for aHUS
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Plasma Therapy for aHUS Is Clinically Inadequate
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Recommendations of French Study Group for Defining PE/PI Failure
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Potential for Significant Morbidity/Mortality Regardless of Overt Laboratory or Clinical Signs
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Eculizumab: Humanized First-in-Class Anti-C5 Antibody
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Eculizimab Dosing Schedule: Adults
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Eculizumab Multinational Clinical Program Includes Broad aHUS Patient Population
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Eculizumab Multinational, Multicenter Clinical Program of Prospective Trials in aHUS (N = 100)
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Improvement in Hematologic Markers of Complement-mediated TMA Across All Studies
at 26 weeks
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Rapid and Sustained Improvement in Hematologic Markers of Complement-mediated TMA With or
Without Identified Genetic Mutation
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Early Eculizumab Therapy Associated With High Likelihood of Eliminating Dialysis
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Prospective Trial of Eculizumab in Adults With aHUS
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Safety Profile of Eculizumab in Prospective Trial of Adults With aHUS
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Eculizumab and Serious Meningococcal Infections
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Key Benefits of Eculizumab
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Abbreviations
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Abbreviations (cont)
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Abbreviations (cont)
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References
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References (cont)
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References (cont)
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References (cont)
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References (cont)
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References (cont)
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References (cont)
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References (cont)
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References (cont)