2010 International consensus algorithm for the diagnosis, therapy and management of hereditary Angioedema

Departments of Medicine and Paediatrics, University of Calgary, Calgary, Alberta, Canada. .
Allergy Asthma and Clinical Immunology (Impact Factor: 2.03). 07/2010; 6(1):24. DOI: 10.1186/1710-1492-6-24
Source: PubMed


We published the Canadian 2003 International Consensus Algorithm for the Diagnosis, Therapy, and Management of Hereditary Angioedema (HAE; C1 inhibitor [C1-INH] deficiency) and updated this as Hereditary angioedema: a current state-of-the-art review: Canadian Hungarian 2007 International Consensus Algorithm for the Diagnosis, Therapy, and Management of Hereditary Angioedema.
To update the International Consensus Algorithm for the Diagnosis, Therapy and Management of Hereditary Angioedema (circa 2010).
The Canadian Hereditary Angioedema Network (CHAEN)/Réseau Canadien d'angioédème héréditaire (RCAH) and cosponsors University of Calgary and the Canadian Society of Allergy and Clinical Immunology (with an unrestricted educational grant from CSL Behring) held our third Conference May 15th to 16th, 2010 in Toronto Canada to update our consensus approach. The Consensus document was reviewed at the meeting and then circulated for review.
This manuscript is the 2010 International Consensus Algorithm for the Diagnosis, Therapy and Management of Hereditary Angioedema that resulted from that conference.
Consensus approach is only an interim guide to a complex disorder such as HAE and should be replaced as soon as possible with large phase III and IV clinical trials, meta analyses, and using data base registry validation of approaches including quality of life and cost benefit analyses, followed by large head-to-head clinical trials and then evidence-based guidelines and standards for HAE disease management.

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Available from: Teresa Caballero
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    • "minor dental work), so long as C1 INH is immediately available and the manipulation has not previously triggered an attack. For major procedures or intubation, they recommended short-term prophylactic C1 INH 1-6 hours before the procedure [11]. Similarly, the WAO urges considering prophylactic C1 INH 1-6 hours before surgeries, especially dental and intraoral, those requiring endotracheal intubation, those manipulating the upper airway or pharynx, and for endoscopy and bronchoscopy procedures [4]. "
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    ABSTRACT: Hereditary 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. A 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. In all 24 procedures, there were no post-procedure HAE attacks after short-term prophylactic administration of C1 INH. In 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.
    Full-text · Article · Apr 2014 · Allergy Asthma and Clinical Immunology
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    • "These results highlight the difficulty in initially suspecting and then diagnosing HAE. Once a diagnosis of HAE is suspected, there are several published guidelines/consensus documents to aid the clinician in the diagnosis and management of HAE patients [18-21]. "
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    ABSTRACT: Background: Hereditary angioedema (HAE) is characterized by unpredictable attacks of debilitating subcutaneous and mucosal edema. Gastrointestinal attacks are painful, of sudden onset and often mistaken for acute abdomen leading to unnecessary surgery. The purpose of this study was to analyze symptom presentation of gastrointestinal angioedema in pediatric and adult HAE patients. Methods: Information collected during the clinical development of ecallantide for treatment of acute HAE attacks included affected anatomic location, accompanying symptoms, medical history, and pain assessments. Efficacy endpoints included Treatment Outcome Score (TOS, maximum score = 100; minimally important difference = 30), a point-in-time measure of treatment response, and time to treatment response. Results: Forty-nine percent of 521 HAE attacks only involved abdominal symptoms. The most commonly reported abdominal symptoms were distension (77%), cramping (73%) and nausea (67%). The most common pain descriptors were tender, tiring-exhausting, aching, cramping and sickening. White blood cell counts were elevated (> 10 x 10(9)/L) in 23% of attacks (mean +/- SD: 15.1 +/- 11.27 x 10(9)/L). A high proportion of patients reported a history of abdominal surgery, including appendectomy (23%), cholecystectomy (16.4%), and hysterectomy (8.2%). Mean TOS at 4 hours post ecallantide was 77 +/- 33 versus 29 +/- 65 for placebo. Median time to significant symptom resolution was 165 minutes (95% CI 136, 167) for ecallantide versus > 4 hours (95% CI 161, > 4 hours) for placebo. Anaphylactic reactions occurred in 6 of the 149 treated patients. Conclusions: HAE should be considered in the differential diagnosis of patients with recurrent discrete episodes of severe, unexplained crampy abdominal pain associated with nausea.
    Full-text · Article · Apr 2014 · BMC Gastroenterology
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    • "The management of the disease consists of the prevention and therapy of edematous attacks. The diagnosis, management, and follow-up of HAE-C1-INH patients are guided by international recommendations and consensus guidelines based on the latest scientific findings [6-8]. "
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    ABSTRACT: Hereditary angioedema (HAE) resulting from C1-inhibitor deficiency is characterized by attacks of subcutaneous and submucosal edema. Many factors have been presumed to induce edema. Our study analyzed these factors in a fairly large patient population. In the first stage of our study, we analyzed the data recorded by 92 subjects in their patient diaries over seven years. The second phase included 27 HAE patients, who had been completing the diary entry 'Trigger factors' every day for seven months whether or not they had experienced an attack. During the initial stage, 91% of the subjects described some factor possibly related to the onset of an attack. They could identify a trigger factor - most commonly (21%) mental stress - in 30% of the 3176 attacks. We found a significant (p < 0.001) difference in the distribution of the trigger factors of the edematous attacks of different locations. The 27 participants of the second phase identified 882 potential trigger factors and recorded 365 attacks. Of these, 246 (67%) occurred on days when the patients identified a potential trigger factor. The likelihood of edema-formation associated with the latter was as follows: menstruation - 63%, infection - 38%, mental stress - 26%, physical exertion - 25%, meteorological changes - 21%, fatigue - 17%. This analysis of the trigger factors explored, for the first time, their potential role in inducing HAE attacks. Our findings might open new perspectives in extending the indications for edema-prophylaxis, and could contribute to a better understanding of the pathomechanism of HAE attacks.
    Full-text · Article · Mar 2014 · Orphanet Journal of Rare Diseases
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