Hereditary angioedema | Page 3

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Z L M Hofman, A Relan, C E Hack
Hereditary angioedema (HAE) patients experience recurrent episodes of angioedema attacks that can be painful, disfiguring and even life-threatening. The disorder results from a mutation in the gene that controls the synthesis of C1-inhibitor (C1INH). C1INH is a major regulator of activation of the contact system. It is often assumed that attacks results from uncontrolled local activation of the contact system with subsequent formation of bradykinin. To evaluate the involvement of inflammatory reactions in HAE, we analysed C-reactive protein (CRP) levels...
July 2014: Clinical and Experimental Immunology
Bruce L Zuraw, Aleena Banerji, Jonathan A Bernstein, Paula J Busse, Sandra C Christiansen, Mark Davis-Lorton, Michael M Frank, Henry H Li, William R Lumry, Marc Riedl
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...
September 2013: Journal of Allergy and Clinical Immunology in Practice
Erika Kajdácsi, Péter K Jani, Dorottya Csuka, Lilian Ágnes Varga, Zoltán Prohászka, Henriette Farkas, László Cervenak
BACKGROUND: Hereditary angioedema (HAE) caused by C1-inhibitor (C1-INH) deficiency (HAE-C1-INH) is a potentially life-threatening rare disease caused by the decreased activity of C1-INH. Lack of C1-INH leads to overproduction of bradykinin, a potent vasoactive peptide. Although angioedema is induced by bradykinin, the function and activation of endothelial cells (ECs), the targets of bradykinin, have not yet been studied during HAE attacks. OBJECTIVE: We studied whether EC function is altered during HAE attacks in comparison with attack-free intervals...
June 2014: Journal of Allergy and Clinical Immunology
W Aberer, M Maurer, A Reshef, H Longhurst, S Kivity, A Bygum, T Caballero, B Bloom, N Nair, A Malbrán
BACKGROUND: Historically, treatment for hereditary angioedema (HAE) attacks has been administered by healthcare professionals (HCPs). Patient self-administration could reduce delays between symptom onset and treatment, and attack burden. The primary objective was to assess the safety of self-administered icatibant in patients with HAE type I or II. Secondary objectives included patient convenience and clinical efficacy of self-administration. METHODS: In this phase IIIb, open-label, multicenter study, adult patients were trained to self-administer a single 30-mg icatibant subcutaneous injection to treat their next attack...
March 2014: Allergy
Katherine A Altman, David R Naimi
BACKGROUND: Angioedema is a serious medical condition characterized by recurrent non-pitting tissue edema. Hereditary (HAE) forms of this disorder are potentially fatal. METHODS: PubMED, Up to Date and Cochrane Library databases were used to identify scholarly peer reviewed original research or review articles on angioedema. Search terms used were: angioedema, HAE, ACE inhibitor induced angioedema, acquired angioedema, type III HAE (now termed HAE with normal C1-INH), diagnosis of HAE, and treatment of HAE...
May 2014: Current Medical Research and Opinion
Inmaculada Martinez-Saguer, Marco Cicardi, Chiara Suffritti, Eva Rusicke, Emel Aygören-Pürsün, Hildegard Stoll, Tanja Rossmanith, Annette Feussner, Uwe Kalina, Wolfhart Kreuz
BACKGROUND: Hereditary angioedema (HAE) is a rare disease caused by C1-esterase inhibitor (C1-INH) deficiency, characterized by periodic attacks of acute edema affecting subcutaneous (SC) tissues and mucous membranes. Human C1-INH concentrate given intravenously (IV) is effective and safe, but venous access may be difficult. We compared SC and IV administration of human pasteurized C1-INH concentrate with respect to pharmacokinetics, pharmacodynamics, and safety. STUDY DESIGN AND METHODS: This was a prospective, randomized, open-label, crossover study...
June 2014: Transfusion
Laurence Bouillet, David Launay, Olivier Fain, Isabelle Boccon-Gibod, Jérôme Laurent, Ludovic Martin, Vincent Montauban, K Finck, Stéphane Bouée, Anne Gompel, Gisèle Kanny
BACKGROUND: Hereditary angioedema (HAE) is a rare and potentially life-threatening disease. New specific treatments are available. OBJECTIVE: To identify patients' features and patients' best therapeutic option. METHODS: A 1-year, multicenter, retrospective study was performed. The primary objective was to examine the clinical presentation of HAE. Secondary objectives included patient characteristics, management of HAE over 12 months, and health-related quality of life using the SF-36v2 questionnaire...
October 2013: Annals of Allergy, Asthma & Immunology
Andrea Zanichelli, Markus Magerl, Hilary Longhurst, Vincent Fabien, Marcus Maurer
BACKGROUND: Hereditary angioedema (HAE) is a rare, debilitating, and potentially life-threatening disease characterized by recurrent edema attacks. Important advances in HAE treatment have been made, including the development of new therapies for treating or preventing attacks. Nevertheless, the disease is still frequently misdiagnosed and inappropriately treated, potentially exposing patients with laryngeal attacks to the risk of asphyxiation. METHODS: The Icatibant Outcome Survey (IOS) is an international, observational study that documents the clinical outcome of HAE patients eligible for treatment with icatibant...
2013: Allergy, Asthma, and Clinical Immunology
Bruce L Zuraw, Jonathan A Bernstein, David M Lang, Timothy Craig, David Dreyfus, Fred Hsieh, David Khan, Javed Sheikh, David Weldon, David I Bernstein, Joann Blessing-Moore, Linda Cox, Richard A Nicklas, John Oppenheimer, Jay M Portnoy, Christopher R Randolph, Diane E Schuller, Sheldon L Spector, Stephen A Tilles, Dana Wallace
These parameters were developed by the Joint Task Force on Practice Parameters (JTFPP), representing the American Academy of Allergy, Asthma & Immunology (AAAAI); the American College of Allergy, Asthma & Immunology (ACAAI); and the Joint Council of Allergy, Asthma and Immunology. The AAAAI and the ACAAI have jointly accepted responsibility for establishing "A focused parameter update: Hereditary angioedema, acquired C1 inhibitor deficiency, and angiotensin-converting enzyme inhibitor-associated angioedema...
June 2013: Journal of Allergy and Clinical Immunology
Kusumam Joseph, Baby G Tholanikunnel, Anette Bygum, Berhane Ghebrehiwet, Allen P Kaplan
BACKGROUND: We have previously reported that prekallikrein expresses an active site when it is bound to high-molecular-weight kininogen (HK) and can digest HK to produce bradykinin. The reaction is stoichiometric and inhibited by C1 inhibitor (C1-INH) or corn trypsin inhibitor. Addition of heat shock protein 90 leads to conversion of prekallikrein to kallikrein in a zinc-dependent reaction. OBJECTIVE: Our goal was to determine whether these reactions are demonstrable in plasma and distinguish them from activation through factor XII...
August 2013: Journal of Allergy and Clinical Immunology
William Lumry, Michael E Manning, David S Hurewitz, Mark Davis-Lorton, David Fitts, Ira N Kalfus, Marc E Uknis
OBJECTIVES: To evaluate the use of Cinryze (nanofiltered C1-esterase inhibitor [C1 INH-nf]) for the acute management and prevention of hereditary angioedema attacks in the subgroup of children and adolescents who participated in 2 placebo-controlled and 2 open-label extension studies. STUDY DESIGN: In the acute-attack treatment studies, the efficacy of 1000 U of C1 INH-nf (with an additional 1000 U given 1 hour later if needed) was assessed based on the time to the start of symptomatic relief and the proportion of patients experiencing relief within 4 hours of therapy...
May 2013: Journal of Pediatrics
Jenny Björkqvist, Anna Sala-Cunill, Thomas Renné
Edema is tissue swelling and is a common symptom in a variety of diseases. Edema form due to accumulation of fluids, either through reduced drainage or increased vascular permeability. There are multiple vascular signalling pathways that regulate vessel permeability. An important mediator that increases vascular leak is the peptide hormone bradykinin, which is the principal agent in the swelling disorder hereditary angioedema. The disease is autosomal dominant inherited and presents clinically with recurrent episodes of acute swelling that can be life-threatening involving the skin, the oropharyngeal, laryngeal, and gastrointestinal mucosa...
March 2013: Thrombosis and Haemostasis
A Reshef, D Moldovan, K Obtulowicz, I Leibovich, E Mihaly, S Visscher, A Relan
BACKGROUND: Hereditary angioedema (HAE) is a disease characterized by recurrent tissue swelling affecting various body locations. Recent literature shows that patients with frequent attacks may benefit from long-term prophylaxis. This study evaluated the safety and prophylactic effect of weekly administrations of recombinant C1INH (rhC1INH). METHODS: Patients with a history of HAE attacks occurring ≥every 2 weeks received a once weekly administration of 50 U/kg rhC1INH...
January 2013: Allergy
Teresa Caballero, Henriette Farkas, Laurence Bouillet, Tom Bowen, Anne Gompel, Christina Fagerberg, Janne Bjökander, Konrad Bork, Anette Bygum, Marco Cicardi, Caterina de Carolis, Michael Frank, Jimmy H C Gooi, Hilary Longhurst, Inmaculada Martínez-Saguer, Erik Waage Nielsen, Krystina Obtulowitz, Roberto Perricone, Nieves Prior
BACKGROUND: There are a limited number of publications on the management of gynecologic/obstetric events in female patients with hereditary angioedema caused by C1 inhibitor deficiency (HAE-C1-INH). OBJECTIVE: We sought to elaborate guidelines for optimizing the management of gynecologic/obstetric events in female patients with HAE-C1-INH. METHODS: A roundtable discussion took place at the 6th C1 Inhibitor Deficiency Workshop (May 2009, Budapest, Hungary)...
February 2012: Journal of Allergy and Clinical Immunology
M Cicardi, K Bork, T Caballero, T Craig, H H Li, H Longhurst, A Reshef, B Zuraw
Angioedema owing to hereditary deficiency of C1 inhibitor (HAE) is a rare, life-threatening, disabling disease. In the last 2 years, the results of well-designed and controlled trials with existing and new therapies for this condition have been published, and new treatments reached the market. Current guidelines for the treatment for HAE were released before the new trials and before the new treatments became available and were essentially based on observational studies and expert opinion. To provide evidence-based HAE treatment guidelines supported by the new studies, a conference was held in Gargnano del Garda, Italy, from September 26 to 29, 2010...
February 2012: Allergy
Michael Lunn, Erin Banta
Hereditary angioedema (HAE) is a clinical disorder characterized by a deficiency of C1 esterase inhibitor (C1-INH). HAE has traditionally been divided into two subtypes. Unique among the inherited deficiencies of the complement system, HAE Types I and II are inherited as an autosomal dominant disorder. The generation of an HAE attack is caused by the depletion and/or consumption of C1-inhibitor manifested as subcutaneous or submucosal edema of the upper airway, face, extremities, or gastrointestinal tract mediated by bradykinin...
2011: Clinical Medicine Insights. Cardiology
Bruce Zuraw, Marco Cicardi, Robyn J Levy, Jan H Nuijens, Anurag Relan, Sonja Visscher, Gerald Haase, Leonard Kaufman, C Erik Hack
BACKGROUND: Hereditary angioedema (HAE) results from a genetic deficiency of C1-inhibitor. Two similar independent, randomized, saline controlled, double-blind studies were conducted to evaluate the efficacy and safety of recombinant human C1-inhibitor (rhC1INH) as a treatment of acute angioedema attacks in patients with HAE. OBJECTIVE: Analysis of pooled study results. METHODS: Patients with an eligible attack were randomized to a single intravenous dose of rhC1INH or saline...
October 2010: Journal of Allergy and Clinical Immunology
Allen P Kaplan
A functional abnormality of C1 inhibitor (C1INH) is present in types I and II hereditary angioedema (HAE), and normal C1INH may be rendered ineffective in the newly described type III HAE. C1INH inhibits factor XIIa, factor XII fragment (XIIf), kallikrein, and plasmin. Thus, in its absence, there is marked activation of the bradykinin-forming cascade resulting in severe angioedema. Factor XII may autoactivate on binding to endothelial cell surface gC1qR (receptor for the globular heads of C1q) thus initiating the cascade...
November 2010: Journal of Allergy and Clinical Immunology
Marco Cicardi, Aleena Banerji, Francisco Bracho, Alejandro Malbrán, Bernd Rosenkranz, Marc Riedl, Konrad Bork, William Lumry, Werner Aberer, Henning Bier, Murat Bas, Jens Greve, Thomas K Hoffmann, Henriette Farkas, Avner Reshef, Bruce Ritchie, William Yang, Jürgen Grabbe, Shmuel Kivity, Wolfhart Kreuz, Robyn J Levy, Thomas Luger, Krystyna Obtulowicz, Peter Schmid-Grendelmeier, Christian Bull, Brigita Sitkauskiene, William B Smith, Elias Toubi, Sonja Werner, Suresh Anné, Janne Björkander, Laurence Bouillet, Enrico Cillari, David Hurewitz, Kraig W Jacobson, Constance H Katelaris, Marcus Maurer, Hans Merk, Jonathan A Bernstein, Conleth Feighery, Bernard Floccard, Gerald Gleich, Jacques Hébert, Martin Kaatz, Paul Keith, Charles H Kirkpatrick, David Langton, Ludovic Martin, Christiane Pichler, David Resnick, Duane Wombolt, Diego S Fernández Romero, Andrea Zanichelli, Francesco Arcoleo, Jochen Knolle, Irina Kravec, Liying Dong, Jens Zimmermann, Kimberly Rosen, Wing-Tze Fan
BACKGROUND: Hereditary angioedema is characterized by recurrent attacks of angioedema of the skin, larynx, and gastrointestinal tract. Bradykinin is the key mediator of symptoms. Icatibant is a selective bradykinin B2 receptor antagonist. METHODS: In two double-blind, randomized, multicenter trials, we evaluated the effect of icatibant in patients with hereditary angioedema presenting with cutaneous or abdominal attacks. In the For Angioedema Subcutaneous Treatment (FAST) 1 trial, patients received either icatibant or placebo; in FAST-2, patients received either icatibant or oral tranexamic acid, at a dose of 3 g daily for 2 days...
August 5, 2010: New England Journal of Medicine
Marco Cicardi, Robyn J Levy, Donald L McNeil, H Henry Li, Albert L Sheffer, Marilyn Campion, Patrick T Horn, William E Pullman
BACKGROUND: Hereditary angioedema is a rare genetic disorder characterized by acute, intermittent, and potentially life-threatening attacks of edema of the skin and mucosa. We evaluated ecallantide, a newly developed recombinant plasma kallikrein inhibitor, for the treatment of acute attacks of angioedema. METHODS: In this double-blind, placebo-controlled trial, patients with hereditary angioedema presenting with an acute attack were randomly assigned, in a 1:1 ratio, to receive subcutaneous ecallantide, at a dose of 30 mg, or placebo...
August 5, 2010: New England Journal of Medicine
2016-07-06 15:57:58
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