Hereditary C1 Inhibitor Deficiency Before Invasive Dental, Medical, or Surgical Procedures

In patients with hereditary C1 inhibitor deficiency, known triggers such as invasive procedures and significant stressful events carry a heightened risk of precipitating an attack. Short-term prophylaxis in these situations is a defined and clinically important strategy.

Clinical scenario: A patient with hereditary C1 inhibitor deficiency is facing an upcoming invasive dental, medical, or surgical procedure — or an anticipated stressful life event — and requires a pre-procedural prophylactic approach to reduce attack risk.
The short-term prophylactic approach in this setting involves an intravenous blood-derived product. Specific selection criteria, timing considerations, and the full clinical decision framework are contained in the complete protocol below.
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References

DOI: 10.1016/j.jaip.2020.08.046

Short-term prophylaxis is indicated when patients are at increased risk of having an attack associated with known triggers such as invasive dental or medical procedures or stressful life events.

FFP can be used in the event that the treating physician cannot obtain pdC1INH and there is insufficient time for a course of anabolic androgens.

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