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Laboratory Approaches for Assessing Contact System Activation.

Sandra C Christiansen1, Bruce L Zuraw2

  • 1Department of Medicine, University of California, 9500 Gilman Drive, Mailcode 0732, La Jolla, CA 92093, USA.

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|July 9, 2017
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Summary

Hereditary angioedema (HAE) involves swelling attacks due to C1 inhibitor deficiency or normal levels. This review covers lab tests for contact system activation, aiding HAE diagnosis.

Keywords:
BradykininC1 inhibitorC1 inhibitor complexesC4Contact systemHereditary angioedemaVascular permeability

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Area of Science:

  • Immunology
  • Genetics
  • Pathophysiology

Background:

  • Hereditary angioedema (HAE) is a rare autosomal dominant disorder.
  • Characterized by recurrent, unpredictable swelling attacks (subcutaneous and mucosal).
  • Attacks can be severe, painful, disfiguring, and life-threatening.

Purpose of the Study:

  • To review laboratory parameters for detecting contact system activation.
  • To discuss the implications for diagnosing HAE and other bradykinin-mediated angioedemas.

Main Methods:

  • Review of literature on laboratory diagnostics for HAE.
  • Analysis of contact system activation markers.
  • Evaluation of diagnostic implications for various angioedema types.

Main Results:

  • HAE types I and II are linked to C1 inhibitor deficiency.
  • HAE with normal C1 inhibitor levels is also recognized.
  • Contact activation is implicated in the swelling attacks of HAE.

Conclusions:

  • Laboratory detection of contact system activation is crucial for HAE diagnosis.
  • Understanding these markers aids in differentiating HAE subtypes.
  • This knowledge supports the diagnosis of bradykinin-mediated angioedemas.