Abstract
BackgroundHereditary Angioedema (HAE) is a rare autosomal dominant condition characterized by episodic angioedema, which may be triggered by invasive procedures and surgery. C1 inhibitor (C1 INH) was approved in the United States and Canada in 2009 and 2010, respectively, for the treatment of acute attacks. Most recently in April 2013, it was approved in Europe for short-term prophylaxis (STP), prior to medical, dental, or surgical procedures, to prevent HAE attacks in both children and adults. Currently, C1 INH is not approved in Canada or the United States for STP of HAE attacks. Our objective was to demonstrate the effectiveness of C1 INH as a short-term prophylactic treatment for patients with Type I HAE undergoing invasive surgical procedures.MethodsA retrospective chart review between 1997-2013 was performed at one Canadian Tertiary Care Allergy and Asthma Clinic affiliated with The Ottawa Hospital, in Ottawa, Canada. The standard dose of C1 INH for STP was 10 or 20 U/kg.ResultsIn all 24 procedures, there were no post-procedure HAE attacks after short-term prophylactic administration of C1 INH.ConclusionsIn this retrospective chart review at one tertiary care Allergy and Clinical Immunology Clinic, short-term prophylactic use of C1 INH was found to be effective at preventing post-procedure HAE attacks, in patients diagnosed with Type I HAE.
Highlights
Hereditary Angioedema (HAE) is a rare autosomal dominant condition characterized by episodic angioedema, which may be triggered by invasive procedures and surgery
Short-term prophylaxis (STP) is used to prevent attacks surrounding known triggers, while long-term prophylaxis is reserved for patients who suffer from frequent HAE attacks
The majority of surgical procedures (22/24) were treated with a short-term prophylactic C1 C1 inhibitor (INH) dose of 1000 U, 1500 U, or 2000 U, which corresponded to a dose of 10 or
Summary
Hereditary Angioedema (HAE) is a rare autosomal dominant condition characterized by episodic angioedema, which may be triggered by invasive procedures and surgery. Most recently in April 2013, it was approved in Europe for short-term prophylaxis (STP), prior to medical, dental, or surgical procedures, to prevent HAE attacks in both children and adults. C1 INH is not approved in Canada or the United States for STP of HAE attacks. Our objective was to demonstrate the effectiveness of C1 INH as a short-term prophylactic treatment for patients with Type I HAE undergoing invasive surgical procedures. Short-term prophylaxis (STP) is used to prevent attacks surrounding known triggers (surgeries, invasive dental procedures, stressful life events), while long-term prophylaxis is reserved for patients who suffer from frequent HAE attacks
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Schedule a callMore From: Allergy, asthma, and clinical immunology : official journal of the Canadian Society of Allergy and Clinical Immunology
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