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

      , 1 , 2 , 3 , 4 , 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 , 9 , 13 , 14 , 15 , 16 , 17 , 17 , 18 , 19 , 20 , 13 , 21 , 9 , 13 , 22 , 23 , 24 , 25 , 26 , 13 , 21 , 13 , 27 , 26 , 28 , 29 , 30 , 18 , 31 , 32 , 33 , 13 , 34 , 13 , 34 , 35 , 36 , 37 , 3 , 38 , 26 , 7 , 39 , 40 , 7 , 41 , 42 , 43 , 44 , 45 , 7 , 46 , 47 , 3 , 48

      Allergy, Asthma, and Clinical Immunology : Official Journal of the Canadian Society of Allergy and Clinical Immunology

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          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) http://www.haecanada.com 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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          Most cited references 70

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          Randomized controlled trial of screening for hepatocellular carcinoma.

          Screening for hepatocellular carcinoma (HCC) has been conducted for over 20 years, but there is no conclusive evidence that screening may reduce HCC mortality. The aim of this study was to assess the effect of screening on HCC mortality in people at increased risk. This study included 18,816 people, aged 35-59 years with hepatitis B virus infection or a history of chronic hepatitis in urban Shanghai, China. Participants were randomly allocated to a screening (9,373) or control (9,443) group. Controls received no screening and continued to use health-care facilities. Screening group participants were invited to have an AFP test and ultrasonography examination every 6 months. Screening was stopped in December 1997; by that time screening group participants had been offered five to ten times. All participants were followed up until December 1998. The primary outcome measure was HCC mortality. The screened group completed 58.2 percent of the screening offered. When the screening group was compared to the control group, the number of HCC was 86 versus 67; subclinical HCC being 52 (60.5%) versus 0; small HCC 39 (45.3%) versus 0; resection achieved 40 (46.5%) versus 5 (7.5%); 1-, 3,-, and 5-year survival rate 65.9%, 52.6%, 46.4% versus 31.2%, 7.2%, 0, respectively. Thirty-two people died from HCC in the screened group versus 54 in the control group, and the HCC mortality rate was significantly lower in the screened group than in controls, being 83.2/100,000 and 131.5/100,000, respectively, with a mortality rate ratio of 0.63 (95%CI 0.41-0.98). Our finding indicated that biannual screening reduced HCC mortality by 37%.
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            Tranexamic acid: a review of its use in surgery and other indications.

             K Goa,  C J Dunn (1999)
            Tranexamic acid is a synthetic derivative of the amino acid lysine that exerts its antifibrinolytic effect through the reversible blockade of lysine binding sites on plasminogen molecules. Intravenously administered tranexamic acid (most commonly 10 mg/kg followed by infusion of 1 mg/kg/hour) caused reductions relative to placebo of 29 to 54% in postoperative blood losses in patients undergoing cardiac surgery with cardiopulmonary bypass (CPB), with statistically significant reductions in transfusion requirements in some studies. Tranexamic acid had similar efficacy to aprotinin 2 x 10(6) kallikrein inhibitory units (KIU) and was superior to dipyridamole in the reduction of postoperative blood losses. Transfusion requirements were reduced significantly by 43% with tranexamic acid and by 60% with aprotinin in 1 study. Meta-analysis of 60 trials showed tranexamic acid and aprotinin, unlike epsilon-aminocaproic acid (EACA) and desmopressin, to reduce significantly the number of patients requiring allogeneic blood transfusions after cardiac surgery with CPB. Tranexamic acid was associated with reductions relative to placebo in mortality of 5 to 54% in patients with upper gastrointestinal bleeding. Meta-analysis indicated a reduction of 40%. Reductions of 34 to 57.9% versus placebo or control in mean menstrual blood loss occurred during tranexamic acid therapy in women with menorrhagia; the drug has also been used to good effect in placental bleeding, postpartum haemorrhage and conisation of the cervix. Tranexamic acid significantly reduced mean blood losses after oral surgery in patients with haemophilia and was effective as a mouthwash in dental patients receiving oral anticoagulants. Reductions in blood loss were also obtained with the use of the drug in patients undergoing orthotopic liver transplantation or transurethral prostatic surgery, and rates of rebleeding were reduced in patients with traumatic hyphaema. Clinical benefit has also been reported with tranexamic acid in patients with hereditary angioneurotic oedema. Tranexamic acid is well tolerated; nausea and diarrhoea are the most common adverse events. Increased risk of thrombosis with the drug has not been demonstrated in clinical trials. Tranexamic acid is useful in a wide range of haemorrhagic conditions. The drug reduces postoperative blood losses and transfusion requirements in a number of types of surgery, with potential cost and tolerability advantages over aprotinin, and appears to reduce rates of mortality and urgent surgery in patients with upper gastrointestinal haemorrhage. Tranexamic acid reduces menstrual blood loss and is a possible alternative to surgery in menorrhagia, and has been used successfully to control bleeding in pregnancy.
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              Hereditary angioedema: new findings concerning symptoms, affected organs, and course.

              Hereditary angioedema (HAE) due to C1 inhibitor deficiency is clinically characterized by relapsing skin swellings, abdominal pain attacks, and life-threatening upper airway obstruction. Our aim was to examine a temporal and spatial pattern of the edema episodes by evaluating the long-term course of hereditary angioedema in order to establish a specific swelling pattern. Data were generated from 221 patients with C1 inhibitor deficiency by asking them about symptoms they experienced during their edema episodes. Documentation was accomplished through the use of standardized questionnaires. A total of 131110 edema episodes were observed. Clinical symptoms started at a mean age of 11.2 (SD 7.7) years. During the following cumulative 5736 years, only 370 (6.5%) symptom-free years occurred. Skin swellings, including extremity, facial, genital, and trunk swellings, and abdominal attacks occurred in 97.4% of all edema episodes of the disease. The other episodes were laryngeal edema (0.9%); edema of the soft palate (0.6%); tongue swellings (0.3%); headache episodes (0.7%); episodes affecting urinary bladder (0.3%), chest (0.2%), muscles (0.4%), joints (0.1%), kidneys (0.1%), and esophagus (0.05%), and were partly combined with other edema episodes. The per-patient analysis and the per-episode analysis revealed markedly discrepant results. On average, women had a more severe course of the disease than men. Patients with early onset of clinical symptoms were affected more severely than those with late onset. The described swelling pattern is specific for HAE and allows a tentative diagnosis based on clinical symptoms and the course of the disease.

                Author and article information

                Allergy Asthma Clin Immunol
                Allergy, Asthma, and Clinical Immunology : Official Journal of the Canadian Society of Allergy and Clinical Immunology
                BioMed Central
                28 July 2010
                : 6
                : 1
                : 24
                [1 ]Departments of Medicine and Paediatrics, University of Calgary, Calgary, Alberta, Canada
                [2 ]Department of Internal Medicine, Universita degli Studi di Milano, Ospedale L. Sacco, Milan, Italy
                [3 ]3rd Department of Internal Medicine, Faculty of Medicine, Semmelweis University, Budapest, Hungary
                [4 ]Department of Dermatology, University Hospital of the Johannes Gutenberg-University of Mainz, Mainz, Germany
                [5 ]Department of Immunology, Barts and the London NHS Trust, London, England, UK
                [6 ]University of California, San Diego, San Diego, California, USA
                [7 ]Johann Wolfgang Goethe University, Frankfurt/Main, Germany
                [8 ]Departments of Medicine and Pediatrics, Penn State University, Hershey, Pennsylvania, USA
                [9 ]Department of Medicine, University of Toronto, Toronto, Canada
                [10 ]Department of Medicine, Laval University, Quebec City, Quebec, Canada
                [11 ]Departments of Medicine and Medical Oncology, University of Alberta, Edmonton, Alberta, Canada
                [12 ]Department of Medicine, CHU de Grenoble, Grenoble, France
                [13 ]Member, Patient Advisory Committee, Canadian Hereditary Angioedema Network (CHAEN)/Réseau Canadien d'angioédème héréditaire (RCAH). 705 South Tower, 3031 Hospital Dr. NW, Calgary, Alberta, Canada
                [14 ]Portage La Prairie, Manitoba, Canada
                [15 ]Department of Pediatrics, University of British Columbia, Vancouver, British Columbia, Canada
                [16 ]Department of Medicine, Regina, Saskatchewan, Canada
                [17 ]Memorial University and Janeway Child Health Centre, St. John's, Newfoundland, Canada
                [18 ]Department of Medicine, McMaster University, Hamilton, Ontario, Canada
                [19 ]Department of Medicine, Dalhousie University, Halifax, Nova Scotia, Canada
                [20 ]Department of Medicine, University of Toronto, Oakville, Ontario, Canada
                [21 ]Ancaster, Ontario, Canada
                [22 ]St. Catharines, Ontario, Canada; Member and Chair, Patient Advisory Committee, Canadian Hereditary Angioedema Network (CHAEN)/Réseau Canadien d'angioédème héréditaire (RCAH
                [23 ]Transfusion Medicine, Ottawa Hospital, Ottawa, Ontario, Canada
                [24 ]Department of Medicine, University of Calgary, Calgary, Alberta, Canada
                [25 ]Department of Pediatrics, CHU Sainte-Justine, University of Montreal, Montreal, Quebec, Canada
                [26 ]Department of Medicine, University of British Columbia, Vancouver, British Columbia, Canada
                [27 ]Halifax, Nova Scotia, Canada
                [28 ]Brampton, Ontario, Canada
                [29 ]Queen's University, Kingston, Ontario, Canada
                [30 ]Department of Medicine, University of Manitoba, Winnipeg, Manitoba, Canada
                [31 ]University of Auckland, Auckland, New Zealand
                [32 ]Department of Internal Medicine, University of Cincinnati, Cincinnati, Ohio, USA
                [33 ]Department of Clinical and Experimental Medicine, County Hospital Ryhov, Jönköping, Sweden
                [34 ]Calgary, Alberta, Canada
                [35 ]Department of Dermatology and Allergy Centre, Odense University Hospital, Denmark
                [36 ]Hospital La Paz Health Research Institute, Madrid, Spain
                [37 ]Duke University Medical Center, Durham, North Carolina, USA
                [38 ]Heim Pal Pediatric Hospital, Budapest, Hungary
                [39 ]Dept of Medicine, Academic Medical Center, Amsterdam Area, Netherlands
                [40 ]Institute for Asthma & Allergy, Wheaton and Chevy Chase, Maryland, USA
                [41 ]4th Medical Clinic, University of Medicine and Pharmacy, Tirgu Mures, Romania
                [42 ]Hungarian Association of Angioedema Patients, Budapest, Hungary
                [43 ]Nordland Hospital, Bodo, University of Tromso, Norway
                [44 ]Department of Medicine, County Hospital Ryhov, Jonkoping, Sweden
                [45 ]Tel Hashomer, and Sackler Faculty of Medicine, Tel Aviv University, Ramat Aviv, Israel
                [46 ]Asociación Española de Angioedema Familiar por Deficiencia del inhibidor de C1 (AEDAF), Madrid, Spain
                [47 ]Institute of Pharmacology, University of Bern, Switzerland
                [48 ]Peking Union Medical College Hospital, Beijing, China
                Copyright ©2010 Bowen et al; licensee BioMed Central Ltd.

                This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.




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