Hereditary C1 inhibitor deficiency
ICD-10 D84.1 · ICD-11 4A00.14
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.
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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