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Konrad Bork - One of the best experts on this subject based on the ideXlab platform.
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Hereditary Angioedema
Der Internist, 2019Co-Authors: Emel Aygören-pürsün, Konrad BorkAbstract:Hereditary Angioedema (HAE) encompasses a heterogeneous group of diseases with similar phenotypes but different underlying genotypes. Specific clinical signs may point to HAE as opposed to histaminergic Angioedema: the typical prolonged development of Angioedema over time, positive family history, a lack of response to antihistamines and steroids and response to bradykinin antagonists are typical signs of HAE. The different types of HAE may be associated with a severe clinical course. They are life-long conditions and are still potentially life-threatening. The quality of life of patients with HAE may be considerably impaired. Management plans should be individualized, which is facilitated by the variety of specific medicastions available.
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Hereditary Angioedema with Normal C1 Inhibitor
Immunology and Allergy Clinics of North America, 2013Co-Authors: Konrad BorkAbstract:: Until recently it was assumed that Hereditary Angioedema was a disease that results exclusively from a genetic deficiency of the C1 inhibitor. In 2000, families with Hereditary Angioedema, normal C1 inhibitor activity, and protein in plasma were described. Since then, numerous patients and families with that condition have been reported. Most of the patients were women. In many of the affected women, oral contraceptives, hormone replacement therapy containing estrogens, and pregnancies triggered the clinical symptoms. In some families mutations in the coagulation factor XII (Hageman factor) gene were detected.
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Diagnosis and treatment of Hereditary Angioedema with normal C1 inhibitor.
Allergy Asthma & Clinical Immunology, 2010Co-Authors: Konrad BorkAbstract:Until recently it was assumed that Hereditary Angioedema is a disease that results exclusively from a genetic deficiency of the C1 inhibitor. In 2000, families with Hereditary Angioedema, normal C1 inhibitor activity and protein in plasma were described. Since then numerous patients and families with that condition have been reported. Most of the patients by far were women. In many of the affected women, oral contraceptives, hormone replacement therapy containing estrogens, and pregnancies triggered the clinical symptoms. Recently, in some families mutations in the coagulation factor XII (Hageman factor) gene were detected in the affected persons.
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Hereditary Angioedema with normal C1 inhibition
Current allergy and asthma reports, 2009Co-Authors: Konrad BorkAbstract:Until recently, it was assumed that Hereditary Angioedema was a disease resulting exclusively from a genetic deficiency of the C1 inhibitor. In 2000, families with Hereditary Angioedema, normal C1 inhibitor activity, and protein in plasma were described. Since then, numerous patients and families with this condition have been reported. Most of the patients were women. In many of the affected women, oral contraceptives, hormone replacement therapy containing estrogens, and pregnancies triggered the clinical symptoms. In some families, mutations in the coagulation factor XII (Hageman factor) gene were detected in the affected persons.
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Disease expression in women with Hereditary Angioedema.
American Journal of Obstetrics and Gynecology, 2008Co-Authors: Laurence Bouillet, Konrad Bork, Anette Bygum, Hilary Longhurst, Teresa Caballero, Henriette Farkas, Isabelle Boccon-gibod, Christophe Bucher, Christian Drouet, Christian MassotAbstract:OBJECTIVE: Fluctuations in sex hormones can trigger Angioedema attacks in women with Hereditary Angioedema. Combined oral contraceptive therapies, as well as pregnancy, can induce severe attacks. The course of Angioedema may be very variable in different women. STUDY DESIGN: Within the PREHAEAT project launched by the European Union, data on 150 postpubertal women with Hereditary Angioedema were collected in 8 countries, using a patient-based questionnaire. RESULTS: Puberty worsened the disease for 62%. Combined oral contraceptives worsened the disease for 79%, whereas progestogen-only pills improved it for 64%. During pregnancies, 38% of women had more attacks, but 30% had fewer attacks. Vaginal delivery was usually uncomplicated. Attacks occurred within 48 hours in only 6% of cases. Those more severely affected during menses had more symptoms during pregnancies, suggesting a hormone-sensitive phenotype for some patients. CONCLUSION: The course of Angioedema in women with C1 inhibitor deficiency is affected by physiologic hormonal changes; consequently, physicians should take these into account when advising on management.
Bruce L. Zuraw - One of the best experts on this subject based on the ideXlab platform.
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us Hereditary Angioedema association medical advisory board 2013 recommendations for the management of Hereditary Angioedema due to c1 inhibitor deficiency
The Journal of Allergy and Clinical Immunology: In Practice, 2013Co-Authors: Bruce L. Zuraw, Sandra C. Christiansen, Michael M. Frank, Paula J. Busse, Aleena Banerji, Jonathan A Bernstein, Mark Davislorton, Henry H LiAbstract:Background The treatment of Hereditary Angioedema (HAE) has undergone dramatic changes as newer medicines have become available in recent years. Optimal care of these patients requires a comprehensive management plan. Although several consensus papers have been published concerning the diagnosis and treatment of HAE, guidelines for a comprehensive management plan have not been developed. Objective To develop state-of-the-art recommendations for the treatment and management of HAE due to C1 inhibitor (C1INH) deficiency in the United States. Methods Members of the US Hereditary Angioedema Association Medical Advisory Board began by reviewing the literature concerning treatment of HAE. Preliminary recommendations were developed based on the literature review, discussions in a face-to-face meeting, and refinements in a series of drafts. Final recommendations reflect the unanimous consensus of the medical advisory board and the US Hereditary Angioedema Association leadership. Results Recommendations are provided regarding a comprehensive care plan for HAE, including the following: development of an overall management plan, treatment of Angioedema attacks, prophylactic treatment, and patient monitoring. Conclusion A comprehensive individualized management plan developed between an expert HAE physician and the patient, in collaboration with local medical providers and emergency departments, can provide patients with the best opportunity to lead a normal life.
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Recognizing and managing Hereditary Angioedema.
Cleveland Clinic journal of medicine, 2013Co-Authors: Kevin Tse, Bruce L. ZurawAbstract:Hereditary Angioedema is a rare but life-threatening disease characterized by recurring attacks of swelling of any part of the body, without hives. Prompt recognition is critical so that treatment can be started to minimize morbidity and the risk of death. Drugs have recently become available to prevent and treat acute attacks.
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Clinical impact of peripheral attacks in Hereditary Angioedema patients.
The American journal of medicine, 2012Co-Authors: Andy Kusuma, Bruce L. Zuraw, Anurag Relan, André C. Knulst, Dumitru Moldovan, Marco Cicardi, Robyn J. Levy, J. H. Nuijens, C. Erik HackAbstract:Abstract Background Episodes of acute subcutaneous Angioedema affecting the extremities in patients with known Hereditary Angioedema are called peripheral attacks. These attacks are considered to be of limited clinical importance. Objective To evaluate the impact of peripheral attacks in patients with Hereditary Angioedema and to assess the response to treatment with recombinant human C1-inhibitor (rhC1INH). Methods Hereditary Angioedema patients with a peripheral attack included in a clinical database of rhC1INH were analyzed. Visual analog scale (VAS) scoring was used to evaluate symptom severity and response to therapy. Results Sixty-five patients with a peripheral attack were identified. VAS scores for 64 patients were available. Twenty-nine (45%) patients reported a single peripheral location of the attack, the others multiple locations. Eight patients (13%) indicated moderate (VAS 20-50 mm) and 55 (86%) severe (VAS ≥50 mm) swelling, 17 (27%) had moderate and 35 (55%) severe pain, while 8 (13%) patients reported moderate and 51 (80%) severe dysfunction for the peripheral attack. Symptom VAS scores decreased over time more rapidly in patients treated with rhC1INH than in patients treated with placebo. Onset of relief was achieved in 95% of the rhC1INH-treated patients within 4 hours, whereas only 21% of saline-treated patients had relief in the same time period. Conclusion Peripheral attacks in Hereditary Angioedema patients often are located at multiple anatomical locations and frequently have associated pain and dysfunction, in addition to swelling, as dominant symptoms. The medical need for treatment of these attacks may be underestimated. Treatment with rhC1INH constitutes a therapeutic option for acute peripheral Hereditary Angioedema attacks.
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Nanofiltered C1 Inhibitor Concentrate for Treatment of Hereditary Angioedema
The New England Journal of Medicine, 2010Co-Authors: Bruce L. Zuraw, Timothy J Craig, Paula J. Busse, Martha V. White, Joshua J. Jacobs, William R. Lumry, James R. Baker, J. Andrew Grant, D. Hurewitz, Leonard BieloryAbstract:Background Hereditary Angioedema due to C1 inhibitor deficiency is characterized by recurrent acute attacks of swelling that can be painful and sometimes life-threatening. Methods We conducted two randomized trials to evaluate nanofiltered C1 inhibitor concentrate in the management of Hereditary Angioedema. The first study compared nanofiltered C1 inhibitor concentrate with placebo for treatment of an acute attack of Angioedema. A total of 68 subjects (35 in the C1 inhibitor group and 33 in the placebo group) were given one or two intravenous injections of the study drug (1000 units each). The primary end point was the time to the onset of unequivocal relief. The second study was a crossover trial involving 22 subjects with Hereditary Angioedema that compared prophylactic twice-weekly injections of nanofiltered C1 inhibitor concentrate (1000 units) with placebo during two 12-week periods. The primary end point was the number of attacks of Angioedema per period, with each subject acting as his or her own c...
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New promise and hope for treating Hereditary Angioedema.
Expert opinion on investigational drugs, 2008Co-Authors: Bruce L. Zuraw, Sandra C. ChristiansenAbstract:Background: While there is no approved effective therapy for the treatment of acute attacks of Hereditary Angioedema in the USA, four different drugs are completing or have recently completed Phase III clinical trials. Objective: To review the clinical status and future prospects of the new therapies under development for the treatment of Hereditary Angioedema. Methods: A review was carried out of the literature and presentations at meetings on the efficacy and safety of plasma-derived C1 inhibitor, recombinant human C1 inhibitor, the kallikrein inhibitor DX-88, and the B2 bradykinin receptor antagonist HOE-140. Results/conclusion: Each of these drugs has been shown to be effective and safe for the treatment of Hereditary Angioedema; however, subtle differences in their mechanisms of action and delivery may influence how physicians and patients utilize the different drugs. The availability of effective therapy is expected to reshape the management of Hereditary Angioedema.
Alvin E Davis - One of the best experts on this subject based on the ideXlab platform.
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New treatments addressing the pathophysiology of Hereditary Angioedema
Clinical and Molecular Allergy, 2008Co-Authors: Alvin E DavisAbstract:Hereditary Angioedema is a serious medical condition caused by a deficiency of C1-inhibitor. The condition is the result of a defect in the gene controlling the synthesis of C1-inhibitor, which regulates the activity of a number of plasma cascade systems. Although the prevalence of Hereditary Angioedema is low – between 1:10,000 to 1:50,000 – the condition can result in considerable pain, debilitation, reduced quality of life, and even death in those afflicted. Hereditary Angioedema presents clinically as cutaneous swelling of the extremities, face, genitals, and trunk, or painful swelling of the gastrointestinal mucosa. Angioedema of the upper airways is extremely serious and has resulted in death by asphyxiation. Subnormal levels of C1-inhibitor are associated with the inappropriate activation of a number of pathways – including, in particular, the complement and contact systems, and to some extent, the fibrinolysis and coagulation systems. Current findings indicate bradykinin, a product of contact system activation, as the primary mediator of Angioedema in patients with C1-inhibitor deficiency. However, other systems may play a role in bradykinin's rapid and excessive generation by depleting available levels of C1-inhibitor. There are currently no effective therapies in the United States to treat acute attacks of Hereditary Angioedema, and currently available agents used to treat Hereditary Angioedema prophylactically are suboptimal. Five new agents are, however, in Phase III development. Three of these agents replace C1-inhibitor, directly addressing the underlying cause of Hereditary Angioedema and re-establishing regulatory control of all pathways and proteases involved in its pathogenesis. These agents include a nano-filtered C1-inhibitor replacement therapy, a pasteurized C1-inhibitor, and a recombinant C1-inhibitor isolated from the milk of transgenic rabbits. All C1-inhibitors are being investigated for acute Angioedema attacks; the nano-filtered C1-inhibitor is also being investigated for prophylaxis of attacks. The other two agents, a kallikrein inhibitor and a bradykinin receptor-2 antagonist, target contact system components that are mediators of vascular permeability. These mediators are formed by contact system activation as a result of C1-inhibitor consumption.
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New treatments addressing the pathophysiology of Hereditary Angioedema
Clinical and molecular allergy : CMA, 2008Co-Authors: Alvin E DavisAbstract:Hereditary Angioedema is a serious medical condition caused by a deficiency of C1-inhibitor. The condition is the result of a defect in the gene controlling the synthesis of C1-inhibitor, which regulates the activity of a number of plasma cascade systems. Although the prevalence of Hereditary Angioedema is low – between 1:10,000 to 1:50,000 – the condition can result in considerable pain, debilitation, reduced quality of life, and even death in those afflicted. Hereditary Angioedema presents clinically as cutaneous swelling of the extremities, face, genitals, and trunk, or painful swelling of the gastrointestinal mucosa. Angioedema of the upper airways is extremely serious and has resulted in death by asphyxiation.
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Hereditary Angioedema: a current state-of-the-art review, III: mechanisms of Hereditary Angioedema.
Annals of Allergy Asthma & Immunology, 2008Co-Authors: Alvin E DavisAbstract:Objective To review the available evidence on the pathophysiologic mechanism of episodes of edema in Hereditary Angioedema (HAE). Data Sources MEDLINE and PubMed were searched using the following keywords: Hereditary Angioedema, C1 inhibitor, complement system, contact system , and bradykinin . Study Selection Studies were selected based on their relevance to the pathophysiologic features of HAE. Results Early studies from the 1970s and 1980s disagreed as to whether the symptoms in HAE were mediated via complement or contact system activation. Studies have demonstrated that, in vitro, in C1 inhibitor (C1-INH)-deficient plasma, only contact system activation results in generation of a vascular permeability enhancing factor. Furthermore, individuals who express a variant C1-INH that is a normal inhibitor of contact system proteases but is deficient in the ability to inactivate complement system proteases do not develop Angioedema. The blood of patients with HAE, during attacks, contains elevated levels of cleaved high-molecular-weight kininogen and bradykinin. Last, C1-INH-deficient mice develop increased vascular permeability that is mediated via contact system activation. Conclusions Hereditary Angioedema attacks are mediated by bradykinin generated via contact system activation. The specific factors that trigger attacks remain unclear.
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Hereditary Angioedema: a current state-of-the-art review, III: mechanisms of Hereditary Angioedema.
Annals of allergy asthma & immunology : official publication of the American College of Allergy Asthma & Immunology, 2008Co-Authors: Alvin E DavisAbstract:To review the available evidence on the pathophysiologic mechanism of episodes of edema in Hereditary Angioedema (HAE). MEDLINE and PubMed were searched using the following keywords: Hereditary Angioedema, C1 inhibitor, complement system, contact system, and bradykinin. Studies were selected based on their relevance to the pathophysiologic features of HAE. Early studies from the 1970s and 1980s disagreed as to whether the symptoms in HAE were mediated via complement or contact system activation. Studies have demonstrated that, in vitro, in C1 inhibitor (C1-INH)-deficient plasma, only contact system activation results in generation of a vascular permeability enhancing factor. Furthermore, individuals who express a variant C1-INH that is a normal inhibitor of contact system proteases but is deficient in the ability to inactivate complement system proteases do not develop Angioedema. The blood of patients with HAE, during attacks, contains elevated levels of cleaved high-molecular-weight kininogen and bradykinin. Last, C1-INH-deficient mice develop increased vascular permeability that is mediated via contact system activation. Hereditary Angioedema attacks are mediated by bradykinin generated via contact system activation. The specific factors that trigger attacks remain unclear.
Jacques Hébert - One of the best experts on this subject based on the ideXlab platform.
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The International/Canadian Hereditary Angioedema Guideline
Allergy asthma and clinical immunology : official journal of the Canadian Society of Allergy and Clinical Immunology, 2019Co-Authors: Stephen Betschel, Jacquie Badiou, Karen Binkley, Rozita Borici-mazi, Jacques Hébert, Amin Kanani, Paul K. Keith, Gina Lacuesta, Susan Waserman, Bill YangAbstract:This is an update to the 2014 Canadian Hereditary Angioedema Guideline with an expanded scope to include the management of Hereditary Angioedema (HAE) patients worldwide. It is a collaboration of Canadian and international HAE experts and patient groups led by the Canadian Hereditary Angioedema Network. The objective of this guideline is to provide evidence-based recommendations, using the GRADE system, for the management of patients with HAE. This includes the treatment of attacks, short-term prophylaxis, long-term prophylaxis, and recommendations for self-administration, individualized therapy, quality of life, and comprehensive care. New to the 2019 version of this guideline are sections covering the diagnosis and recommended therapies for acute treatment in HAE patients with normal C1-INH, as well as sections on pregnant and paediatric patients, patient associations and an HAE registry. Hereditary Angioedema results in random and often unpredictable attacks of painful swelling typically affecting the extremities, bowel mucosa, genitals, face and upper airway. Attacks are associated with significant functional impairment, decreased health-related quality of life, and mortality in the case of laryngeal attacks. Caring for patients with HAE can be challenging due to the complexity of this disease. The care of patients with HAE in Canada, as in many countries, continues to be neither optimal nor uniform. It lags behind some other countries where there are more organized models for HAE management, and greater availability of additional licensed therapeutic options. It is anticipated that providing this guideline to caregivers, policy makers, patients, and advocates will not only optimize the management of HAE, but also promote the importance of individualized care. The primary target users of this guideline are healthcare providers who are managing patients with HAE. Other healthcare providers who may use this guideline are emergency and intensive care physicians, primary care physicians, gastroenterologists, dentists, otolaryngologists, paediatricians, and gynaecologists who will encounter patients with HAE and need to be aware of this condition. Hospital administrators, insurers and policy makers may also find this guideline helpful.
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the international canadian Hereditary Angioedema guideline
Allergy Asthma & Clinical Immunology, 2019Co-Authors: Stephen Betschel, Jacquie Badiou, Karen Binkley, Jacques Hébert, Amin Kanani, Paul K. Keith, Gina Lacuesta, Susan Waserman, Rozita Boricimazi, Bill YangAbstract:This is an update to the 2014 Canadian Hereditary Angioedema Guideline with an expanded scope to include the management of Hereditary Angioedema (HAE) patients worldwide. It is a collaboration of Canadian and international HAE experts and patient groups led by the Canadian Hereditary Angioedema Network. The objective of this guideline is to provide evidence-based recommendations, using the GRADE system, for the management of patients with HAE. This includes the treatment of attacks, short-term prophylaxis, long-term prophylaxis, and recommendations for self-administration, individualized therapy, quality of life, and comprehensive care. New to the 2019 version of this guideline are sections covering the diagnosis and recommended therapies for acute treatment in HAE patients with normal C1-INH, as well as sections on pregnant and paediatric patients, patient associations and an HAE registry. Hereditary Angioedema results in random and often unpredictable attacks of painful swelling typically affecting the extremities, bowel mucosa, genitals, face and upper airway. Attacks are associated with significant functional impairment, decreased health-related quality of life, and mortality in the case of laryngeal attacks. Caring for patients with HAE can be challenging due to the complexity of this disease. The care of patients with HAE in Canada, as in many countries, continues to be neither optimal nor uniform. It lags behind some other countries where there are more organized models for HAE management, and greater availability of additional licensed therapeutic options. It is anticipated that providing this guideline to caregivers, policy makers, patients, and advocates will not only optimize the management of HAE, but also promote the importance of individualized care. The primary target users of this guideline are healthcare providers who are managing patients with HAE. Other healthcare providers who may use this guideline are emergency and intensive care physicians, primary care physicians, gastroenterologists, dentists, otolaryngologists, paediatricians, and gynaecologists who will encounter patients with HAE and need to be aware of this condition. Hospital administrators, insurers and policy makers may also find this guideline helpful.
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2010 international consensus algorithm for the diagnosis therapy and management of Hereditary Angioedema
The Journal of Allergy and Clinical Immunology, 2004Co-Authors: Tom Bowen, Konrad Bork, Hilary Longhurst, Bruce L. Zuraw, Marco Cicardi, Karen Binkley, Henriette Farkas, Emel Aygoerenpursun, Timothy J Craig, Jacques HébertAbstract:Background: 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. Objective: To update the International Consensus Algorithm for the Diagnosis, Therapy and Management of Hereditary Angioedema (circa 2010). Methods: The Canadian Hereditary Angioedema Network (CHAEN)/Reseau Canadien d’angioedeme hereditaire (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. Results: This manuscript is the 2010 International Consensus Algorithm for the Diagnosis, Therapy and Management of Hereditary Angioedema that resulted from that conference. Conclusions: 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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Management of Hereditary Angioedema: a Canadian approach.
Transfusion and apheresis science : official journal of the World Apheresis Association : official journal of the European Society for Haemapheresis, 2003Co-Authors: Tom Bowen, Jacques Hébert, Bruce Ritchie, Jeanne Burnham, Mike Macsween, Richard Warrington, William H. Yang, Andrew C. Issekutz, Nick Karitsiotis, Nancy MccombieAbstract:Abstract C1 esterase inhibitor (C1-INH) deficiency is a rare disorder that lacks consensus for diagnosis therapy and management. Recognizing that Canada is behind the European approach to this disorder, we have formed the Canadian Hereditary Angioedema Society (CHAES)/Societe d'angioedeme hereditaire du Canada (SAHC) to foster knowledge of this disorder in Canada and to advance care of patients with this disorder in Canada. We here present a review of treatment of this disorder in Canada including prevention of Angioedema events and use of replacement therapy and present an algorithm for diagnosis therapy and management of C1-INH deficiency in Canada for discussion at our International Conference on Hereditary Angioedema to be held in Toronto, Canada, October 24th to 26th, 2003.
Bill Yang - One of the best experts on this subject based on the ideXlab platform.
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The International/Canadian Hereditary Angioedema Guideline
Allergy asthma and clinical immunology : official journal of the Canadian Society of Allergy and Clinical Immunology, 2019Co-Authors: Stephen Betschel, Jacquie Badiou, Karen Binkley, Rozita Borici-mazi, Jacques Hébert, Amin Kanani, Paul K. Keith, Gina Lacuesta, Susan Waserman, Bill YangAbstract:This is an update to the 2014 Canadian Hereditary Angioedema Guideline with an expanded scope to include the management of Hereditary Angioedema (HAE) patients worldwide. It is a collaboration of Canadian and international HAE experts and patient groups led by the Canadian Hereditary Angioedema Network. The objective of this guideline is to provide evidence-based recommendations, using the GRADE system, for the management of patients with HAE. This includes the treatment of attacks, short-term prophylaxis, long-term prophylaxis, and recommendations for self-administration, individualized therapy, quality of life, and comprehensive care. New to the 2019 version of this guideline are sections covering the diagnosis and recommended therapies for acute treatment in HAE patients with normal C1-INH, as well as sections on pregnant and paediatric patients, patient associations and an HAE registry. Hereditary Angioedema results in random and often unpredictable attacks of painful swelling typically affecting the extremities, bowel mucosa, genitals, face and upper airway. Attacks are associated with significant functional impairment, decreased health-related quality of life, and mortality in the case of laryngeal attacks. Caring for patients with HAE can be challenging due to the complexity of this disease. The care of patients with HAE in Canada, as in many countries, continues to be neither optimal nor uniform. It lags behind some other countries where there are more organized models for HAE management, and greater availability of additional licensed therapeutic options. It is anticipated that providing this guideline to caregivers, policy makers, patients, and advocates will not only optimize the management of HAE, but also promote the importance of individualized care. The primary target users of this guideline are healthcare providers who are managing patients with HAE. Other healthcare providers who may use this guideline are emergency and intensive care physicians, primary care physicians, gastroenterologists, dentists, otolaryngologists, paediatricians, and gynaecologists who will encounter patients with HAE and need to be aware of this condition. Hospital administrators, insurers and policy makers may also find this guideline helpful.
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the international canadian Hereditary Angioedema guideline
Allergy Asthma & Clinical Immunology, 2019Co-Authors: Stephen Betschel, Jacquie Badiou, Karen Binkley, Jacques Hébert, Amin Kanani, Paul K. Keith, Gina Lacuesta, Susan Waserman, Rozita Boricimazi, Bill YangAbstract:This is an update to the 2014 Canadian Hereditary Angioedema Guideline with an expanded scope to include the management of Hereditary Angioedema (HAE) patients worldwide. It is a collaboration of Canadian and international HAE experts and patient groups led by the Canadian Hereditary Angioedema Network. The objective of this guideline is to provide evidence-based recommendations, using the GRADE system, for the management of patients with HAE. This includes the treatment of attacks, short-term prophylaxis, long-term prophylaxis, and recommendations for self-administration, individualized therapy, quality of life, and comprehensive care. New to the 2019 version of this guideline are sections covering the diagnosis and recommended therapies for acute treatment in HAE patients with normal C1-INH, as well as sections on pregnant and paediatric patients, patient associations and an HAE registry. Hereditary Angioedema results in random and often unpredictable attacks of painful swelling typically affecting the extremities, bowel mucosa, genitals, face and upper airway. Attacks are associated with significant functional impairment, decreased health-related quality of life, and mortality in the case of laryngeal attacks. Caring for patients with HAE can be challenging due to the complexity of this disease. The care of patients with HAE in Canada, as in many countries, continues to be neither optimal nor uniform. It lags behind some other countries where there are more organized models for HAE management, and greater availability of additional licensed therapeutic options. It is anticipated that providing this guideline to caregivers, policy makers, patients, and advocates will not only optimize the management of HAE, but also promote the importance of individualized care. The primary target users of this guideline are healthcare providers who are managing patients with HAE. Other healthcare providers who may use this guideline are emergency and intensive care physicians, primary care physicians, gastroenterologists, dentists, otolaryngologists, paediatricians, and gynaecologists who will encounter patients with HAE and need to be aware of this condition. Hospital administrators, insurers and policy makers may also find this guideline helpful.