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    2365 results match your criteria Angioedema Hereditary

    1 OF 48

    Use of pdC1-INH concentrate for long-term prophylaxis during pregnancy in hereditary angioedema with normal C1-INH.
    J Allergy Clin Immunol Pract 2018 Feb 10. Epub 2018 Feb 10.
    Clinical Immunology and Allergy Division, University of São Paulo, São Paulo, SP, Brazil. Electronic address:

    Plasma Prekallikrein: Its Role in Hereditary Angioedema and Health and Disease.
    Front Med (Lausanne) 2018 25;5. Epub 2018 Jan 25.
    Hematology and Oncology Division, Department of Medicine, University Hospitals Cleveland Medical Center, Case Western Reserve University, Cleveland, OH, United States.
    Plasma prekallikrein (PK) has a critical role in acute attacks of hereditary angioedema (HAE). Unlike C1 inhibitor, its levels fall during HAE attacks with resultant cleaved high-molecular-weight kininogen. Cleavage of high-molecular-weight kininogen liberates bradykinin, the major biologic peptide that promotes the edema. Read More

    Hereditary and Acquired Angioedema: Heterogeneity of Pathogenesis and Clinical Phenotypes.
    Int Arch Allergy Immunol 2018 Jan 26. Epub 2018 Jan 26.
    Department of Medicine, Division of Allergy and Clinical Immunology, University of Salerno, Baronissi, Italy.
    Recurrent angioedema (AE) without wheals is increasingly recognized as a clinical entity and a frequent cause of admission to the emergency room. The Hereditary Angioedema Working Group (HAWK) classification allowed the scientific community to go beyond the semantic confusion that dominated this topic for decades. This classification distinguishes hereditary and acquired forms of AE, either related or unrelated to C1 inhibitor deficiency. Read More

    Health-Related Quality-of-Life with Subcutaneous C1-inhibitor for Prevention of Attacks of Hereditary Angioedema.
    J Allergy Clin Immunol Pract 2018 Jan 29. Epub 2018 Jan 29.
    CSL Behring, King of Prussia, PA.
    Background: Hereditary angioedema with C1-INH deficiency (C1-INH-HAE) impairs health-related quality of life (HRQoL).

    Objective: To assess HRQoL outcomes in patients self-administering subcutaneous C1-INH (C1-INH[SC]; HAEGARDA) for routine prevention of HAE attacks.

    Methods: Post-hoc analysis of data from a placebo-controlled, crossover phase III study (COMPACT). Read More

    New Treatments for Hereditary Angioedema.
    Skin Therapy Lett 2018 01;23(1):6-8
    Virginia Tech Carilion School of Medicine, Roanoke, VA, USA; Carilion Clinic Dermatology & Mohs Surgery, Roanoke, VA, USA.
    Hereditary angioedema is characterized by severe, episodic edema of the subcutaneous and mucosal tissue. The disease carries significant morbidity and mortality due to involvement of the gastrointestinal tract and upper airway. Recent advances in the treatment of hereditary angioedema include new techniques used to isolate and purify human-derived C1 inhibitor, the production of a recombinant form of C1 inhibitor, and the development of drugs that target the kallikrein-kinin pathway. Read More

    Intermittent C1-Inhibitor Deficiency Associated with Recessive Inheritance: Functional and Structural Insight.
    Sci Rep 2018 Jan 17;8(1):977. Epub 2018 Jan 17.
    "L. Sacco" Department of Biomedical and Clinical Sciences, University of Milan, via GB Grassi 74, 20157, Milan, Italy.
    C1-inhibitor is a serine protease inhibitor (serpin) controlling complement and contact system activation. Gene mutations result in reduced C1-inhibitor functional plasma level causing hereditary angioedema, a life-threatening disorder. Despite a stable defect, the clinical expression of hereditary angioedema is unpredictable, and the molecular mechanism underlying this variability remains undisclosed. Read More

    [Allergology and clinical immunology].
    Rev Med Suisse 2018 Jan;14(588-589):15-18
    Service d'immunologie et d'allergologie, Département des spécialités de médecine, HUG, 1211 Genève 14.
    Hereditary angioedema (HA) is a disabling and potentially fatal condition. The management of HA includes treatment of acute attacks, short-term prophylaxis to prevent an attack, and long-term prophylaxis to minimize the frequency and severity of recurrent attacks. In this article, we will present new therapeutic alternatives for long term prophylaxis. Read More

    The international WAO/EAACI guideline for the management of hereditary angioedema - the 2017 revision and update.
    Allergy 2018 Jan 10. Epub 2018 Jan 10.
    Department of Medicine and Pediatrics, Penn State University, Hershey, PA, USA.
    Hereditary angioedema (HAE) is a rare disease and a serious health problem, globally and for affected patients and their families. The pathophysiological background is primarily a vascular reaction to an overshooting local production of bradykinin. Evidence-based recommendations are needed to inform and guide clinical decision makers. Read More

    Exposure-Response Model of Subcutaneous C1-Inhibitor Concentrate to Estimate the Risk of Attacks in Patients With Hereditary Angioedema.
    CPT Pharmacometrics Syst Pharmacol 2018 Jan 9. Epub 2018 Jan 9.
    Clinical Pharmacology and Early Development, CSL Limited, Parkville, Australia.
    Subcutaneous C1-inhibitor (HAEGARDA, CSL Behring), is a US Food and Drug Administration (FDA)-approved, highly concentrated formulation of a plasma-derived C1-esterase inhibitor (C1-INH), which, in the phase III Clinical Studies for Optimal Management in Preventing Angioedema with Low-Volume Subcutaneous C1-inhibitor Replacement Therapy (COMPACT) trial, reduced the incidence of hereditary angioedema (HAE) attacks when given prophylactically. Data from the COMPACT trial were used to develop a repeated time-to-event model to characterize the timing and frequency of HAE attacks as a function of C1-INH activity, and then develop an exposure-response model to assess the relationship between C1-INH functional activity levels (C1-INH(f)) and the risk of an attack. The C1-INH(f) values of 33. Read More

    [Vulvar angioedema during pregnancy].
    Ned Tijdschr Geneeskd 2018 ;162(0):D1548
    Deventer Ziekenhuis, Deventer.
    Background: Angioedema is a condition of acute and extensive fluid accumulation in skin or mucosae due to increased blood vessel permeability. Angioedema can have several causes, including pregnancy.

    Case Description: A healthy 33-year-old pregnant woman had acute, substantial swelling of the labia minora with no other symptoms. Read More

    Evaluating satisfaction of patients with hereditary angioedema with their past and present treatments: Implications for future therapies.
    Allergy Asthma Proc 2018 Jan;39(1):74-80
    Background: Ever-expanding armamentarium of treatments for hereditary angioedema (HAE) are associated with various adverse effects, issues with vascular access, or lack of self-administration.

    Objective: To understand patients' impressions and confidence in their past and present treatments, and identifying adverse events while also directly asking patients to reveal their hope for the future of HAE management and treatments.

    Methods: After institutional review board approval, all subjects with laboratory-confirmed HAE were mailed a survey that they completed and returned to the researchers, and data were collected and entered into a secure online web application for surveys. Read More

    Disease Severity, Activity, Impact, and Control and How to Assess Them in Patients with Hereditary Angioedema.
    Front Med (Lausanne) 2017 4;4:212. Epub 2017 Dec 4.
    Department of Dermatology and Allergy, Charité - Universitätsmedizin Berlin, Berlin, Germany.
    Hereditary angioedema (HAE) is a group of rare, potentially life-threatening, and frequently debilitating diseases characterized by recurrent, and often with an unpredictable onset, of swelling attacks. HAE is heterogeneous, with considerable differences between its subtypes, patients, and even within the same patient over time. During the past few years, several new on demand and prophylactic therapies have become available for HAE, allowing for individualized treatment. Read More

    [Hereditary angioedema by C1 inhibitor-deficit: Diagnostic and therapeutic challenges. Case report].
    Rev Alerg Mex 2017 Oct-Dec;64(4):493-498
    Centro de Neumología y Alergias, San Pedro Sula, Cortés, Honduras.
    Background: Hereditary angioedema is a disease which manifests itself with episodes of spontaneous edema on skin, mucosal and airway. Treatment includes acute and prophylactic approach to minimize the attacks and severity. In many parts of the world, androgen derivatives, antifibrinolytic and fresh frozen plasma are the therapies available for prophylaxis. Read More

    Investigational drugs in phase I and phase II clinical trials for hereditary angioedema.
    Expert Opin Investig Drugs 2018 Jan 15;27(1):87-103. Epub 2017 Dec 15.
    a Hungarian Angioedema Center, 3rd Department of Internal Medicine , Semmelweis University , Budapest , Hungary.
    Introduction: Hereditary angioedema (HAE) with C1-inhibitor deficiency (C1-INH-HAE) is a rare bradykinin-mediated disease characterized by recurrent subcutaneous and/or submucosal angioedematous attacks (HAE attacks), which occur unpredictably. The recurrent HAE attacks do not respond to conventional treatments, and may evolve into a life-threatening condition; therefore, special therapy is required. Areas covered: The agents used so far for the acute management of HAE attacks act by blocking the release of bradykinin, or its binding to its receptor. Read More

    Immunological Rare Diseases.
    Adv Exp Med Biol 2017 ;1031:497-509
    Department of Clinical and Biological Sciences, Turin University, Turin, Italy.
    The immune system is delegated to defend the body from attacks from outside or inside. Many diseases can affect immune system reducing its ability to defend self or inducing an abnormal response against external or internal antigens. Rare diseases affecting immune system present some issue in common with other rare diseases and some peculiarities due to the huge variability in the disease's expression. Read More

    Pharmacological Management of Hereditary Angioedema with C1-Inhibitor Deficiency in Pediatric Patients.
    Paediatr Drugs 2017 Dec 7. Epub 2017 Dec 7.
    3rd Department of Internal Medicine, Hungarian Angioedema Center, Semmelweis University, Kútvölgyi street 4, Budapest, 1125, Hungary.
    Hereditary angioedema with C1-inhibitor deficiency (C1-INH-HAE) is a form of bradykinin-mediated angioedema. It is a rare disorder with an onset during childhood in most instances. Therefore, familiarity with the options for the management of pediatric cases is indispensable. Read More

    The Search for Biomarkers in Hereditary Angioedema.
    Front Med (Lausanne) 2017 22;4:206. Epub 2017 Nov 22.
    Department of Clinical Chemistry and Haematology, University Medical Center Utrecht, Utrecht University, Utrecht, Netherlands.
    The unpredictable nature of attacks of tissue swelling in hereditary angioedema requires the identification of reliable biomarkers to monitor disease activity as well as response to therapy. At present, one can assess a C4 level (by ELISA) to assist in diagnosis but neither C4 nor C1 inhibitor levels reflect clinical course or prognosis. We will here review a collection of plasma proteins involved in blood coagulation, fibrinolysis, and innate immunity (Figure 1). Read More

    Outcomes of long term treatments of type I hereditary angioedema in a Turkish family.
    An Bras Dermatol 2017 Sep-Oct;92(5):655-660
    Dermatovenereology Clinic, Ataturk Training and Research Hospital - Ankara, Turkey.
    Background: Hereditary angioedema is a rare autosomal dominantly inherited immunodeficiency disorder characterized by potentially life-threatening angioedema attacks.

    Objective: We aimed to investigate the clinical and genetic features of a family with angioedema attacks.

    Methods: The medical history, clinical features and C1-INH gene mutation of a Turkish family were investigated and outcomes of long-term treatments were described. Read More

    Genetic Determinants of C1 Inhibitor Deficiency Angioedema Age of Onset.
    Int Arch Allergy Immunol 2017 9;174(3-4):200-204. Epub 2017 Nov 9.
    Department of Immunology and Histocompatibility, Faculty of Medicine, School of Health Sciences, University of Thessaly, Larissa, Greece.
    Background: In view of the large heterogeneity in the clinical presentation of hereditary angioedema due to C1 inhibitor deficiency (C1-INH-HAE), great efforts are being made towards detecting measurable biological determinants of disease severity that can help to improve the management of the disease. Considering the central role that plasma kallikrein plays in bradykinin production, we investigated the contribution of the functional polymorphism KLKB1-428G/A to the disease phenotype.

    Methods: We studied 249 C1-INH-HAE patients from 114 European families, and we explored possible associations of C1-INH-HAE clinical features with carriage of KLKB1-428G/A, combined or not with that of the functional F12-46C/T polymorphism. Read More

    Hereditary Angioedema with Normal C1 Inhibitor and F12 Mutations in 42 Brazilian Families.
    J Allergy Clin Immunol Pract 2017 Nov 8. Epub 2017 Nov 8.
    Division of Clinical Immunology, Faculdade de Medicina ABC, Santo André, SP, Brazil. Electronic address:
    Background: Hereditary angioedema (HAE) with normal C1 inhibitor (C1-INH) is a rare condition with clinical features similar to those of HAE with C1-INH deficiency. Mutations in the F12 gene have been identified in subsets of patients with HAE with normal C1-INH, mostly within families of European descent.

    Objectives: Our aim was to describe clinical characteristics observed in Brazilians from 42 families with HAE and F12 gene mutations (FXII-HAE), and to compare these findings with those from other populations. Read More

    Recurrent ascites: a need to evaluate for hereditary angio-oedema.
    Lancet 2017 Nov;390(10107):2119-2120
    Allergy-Immunology Department, Mureș County Hospital, Târgu Mureș, Romania; University of Medicine and Pharmacy, Târgu Mureș, Romania; Romanian Network for Hereditary Angioedema, Romania.

    Pharmacotherapy for Angiotensin-Converting Enzyme Inhibitor-Induced Angioedema: A Systematic Review.
    Otolaryngol Head Neck Surg 2018 Feb 7;158(2):232-239. Epub 2017 Nov 7.
    1 Department of Otolaryngology-Head and Neck Surgery, School of Medicine, Tulane University, New Orleans, Louisiana, USA.
    Objective Angioedema is a potentially life-threatening complication of angiotensin-converting enzyme inhibitor (ACEI) use, occurring in up to 0.5% of users. Although the pathophysiology of ACEI-induced angioedema is attributable to elevated serum bradykinin, standard management typically includes corticosteroids and antihistamines. Read More

    Hereditary angioedema: Assessing the hypothesis for underlying autonomic dysfunction.
    PLoS One 2017 6;12(11):e0187110. Epub 2017 Nov 6.
    Department of Biomedical and Clinical Sciences, University of Milan, Milan, Italy.
    Background: Attacks of Hereditary Angioedema due to C1-inhibitor deficiency (C1-INH-HAE)are often triggered by stressful events/hormonal changes.

    Objective: Our study evaluates the relationship between autonomic nervous system (ANS) and contact/complement system activation.

    Methods: Twenty-three HAE patients (6 males, mean age 47. Read More

    Comparison of the Frequency of Angioedema Attack, before and during Pregnancy, in a Patient with Type I Hereditary Angioedema.
    Intern Med 2017 Nov 1. Epub 2017 Nov 1.
    Division of Laboratory Medicine, Department of Pathology and Microbiology, Nihon University School of Medicine, Japan.
    The patient was a 38-year-old Japanese woman who had been diagnosed with hereditary angioedema type I at 7 years of age based on her family history. She had undergone four pregnancies. She gave birth to a healthy baby girl after her first pregnancy and had reported few episodes of angioedema. Read More

    Clinical Features of Hereditary and Mast Cell-mediated Angioedema Focusing on the Differential Diagnosis in Japanese Patients.
    Intern Med 2018 Feb 1;57(3):319-324. Epub 2017 Nov 1.
    Medical Corporation SHOWAKAI, Japan.
    Objective The present study was designed to identify the clinical characteristics that permit the differential diagnosis of hereditary angioedema (HAE) and mast cell-mediated angioedema (Mast-AE) during the first consultation. Methods The medical histories and laboratory data of 46 patients with HAE and 41 patients with Mast-AE were compared. Results The average age of onset in the HAE group (19. Read More

    Multiple doses of icatibant used during pregnancy.
    Allergy Rhinol (Providence) 2017 Oct;8(3):178-181
    From the Department of Allergy, Asthma, and Immunology, Penn State Milton S. Hershey Medical Center, Hershey, Pennsylvania.
    Background: Hereditary angioedema (HAE) is a life-long disease that often manifests by puberty. Treatment of attacks is essential to improve quality of life and to decrease morbidity and mortality. During pregnancy, treatment is limited because multiple treatment options, including icatibant, are not approved for use during pregnancy. Read More

    Pathogenesis of Drug Induced Non-Allergic Angioedema: A Review of Unusual Etiologies.
    Cureus 2017 Aug 23;9(8):e1598. Epub 2017 Aug 23.
    Internal Medicine, Pramukhswami Medical College, Anand,Gujarat.
    Angioedema is the swelling of mucosal and sub-mucosal tissue. Typically, it manifests as the swelling of the face, lips, and tongue. Angioedema can be severe and life threatening when it involves the respiratory tract. Read More

    Endocan: A Novel Marker of Endothelial Dysfunction in C1-Inhibitor-Deficient Hereditary Angioedema.
    Int Arch Allergy Immunol 2017 24;174(2):104-107. Epub 2017 Oct 24.
    Immunology and Allergy Unit, Yedikule Education and Training Hospital, Istanbul University, Istanbul, Turkey.
    Background: Hereditary angioedema (HAE) related to C1-inhibitor deficiency is a rare autosomal dominant disorder. Vascular cell adhesion molecules (VCAM) are known as endothelial activation markers. Endocan (also called ESM-1) is proposed as an endothelial dysfunction indicator. Read More

    Health-related quality of life in Danish children with hereditary angioedema.
    Allergy Asthma Proc 2017 Nov;38(6):440-446
    Background: The potentially life-threatening disease hereditary angioedema with C1-inhibitor deficiency (C1-INH-HAE) can have considerable impact on the health-related quality of life (HRQoL) in adult patients. Half the patients with C1-INH-HAE develop symptoms before the age of 10 years. However, the HRQoL in children with C1-INH-HAE is almost unexplored. Read More

    An open-label study to evaluate the long-term safety and efficacy of lanadelumab for prevention of attacks in hereditary angioedema: design of the HELP study extension.
    Clin Transl Allergy 2017 6;7:36. Epub 2017 Oct 6.
    Division of Clinical Immunology and Allergy, Department of Medicine, Icahn School of Medicine at Mount Sinai, 5 East 98th Street 11th Floor, New York, NY 10029 USA.
    Background: Hereditary angioedema (HAE) is characterized by recurrent attacks of subcutaneous or submucosal edema. Attacks are unpredictable, debilitating, and have a significant impact on quality of life. Patients may be prescribed prophylactic therapy to prevent angioedema attacks. Read More

    Angiotensin-converting enzyme inhibitor-induced angioedema: A review of the literature.
    J Clin Hypertens (Greenwich) 2017 Dec 10;19(12):1377-1382. Epub 2017 Oct 10.
    Department of Medicine, Division of Allergy, Immunology and Infectious Diseases, Rutgers Robert Wood Johnson Medical School, New Brunswick, NJ, USA.
    According to the National Health and Nutrition Examination Survey 2012, one third of antihypertensive prescriptions in the United States in the past decade were for angiotensin-converting enzyme inhibitors (ACEIs). An important and serious side effect of ACEIs is angioedema caused by a reduction in bradykinin degradation. In a national medical chart abstraction study conducted at the US Veterans Affairs Health Care System in 2008, 0. Read More

    Complete kinetic follow-up of symptoms and complement parameters during a hereditary angioedema attack.
    Allergy 2018 Feb 30;73(2):516-520. Epub 2017 Oct 30.
    Research Laboratory, 3rd Department of Internal Medicine, Semmelweis University, Budapest, Hungary.
    We studied the kinetics of C1-inhibitor (C1-INH) and other complement parameters in a self-limited edematous attack (EA) in a patient with hereditary angioedema due to C1-INH deficiency to better understand the pathomechanism of the evolution, course, and complete resolution of EAs. C1-INH concentration and functional activity (C1-INH), C1(q,r,s), C3, C4, C3a, C4a, C5a, and SC5b-9 levels were measured in blood samples obtained during the 96-hour observation period. The highest C1-INH, C4, and C1(q,r,s) levels were measured at baseline, and their continuous decrease was observed during the entire observation period. Read More

    Factor XII-Driven Inflammatory Reactions with Implications for Anaphylaxis.
    Front Immunol 2017 15;8:1115. Epub 2017 Sep 15.
    Institute of Clinical Chemistry and Laboratory Medicine, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
    Anaphylaxis is a life-threatening allergic reaction. It is triggered by the release of pro-inflammatory cytokines and mediators from mast cells and basophils in response to immunologic or non-immunologic mechanisms. Mediators that are released upon mast cell activation include the highly sulfated polysaccharide and inorganic polymer heparin and polyphosphate (polyP), respectively. Read More

    An ABC of the Warning Signs of Hereditary Angioedema.
    Int Arch Allergy Immunol 2017 27;174(1):1-6. Epub 2017 Sep 27.
    Faculdade de Medicina ABC, Santo Andre, Brazil.
    Hereditary angioedema (HAE) with C1 inhibitor deficiency is a genetic disorder that clinically manifests with attacks of angioedema in the subcutaneous and submucosal tissues, mainly in the extremities, abdomen, and upper airway. During attacks, vascular permeability is increased due to increased bradykinin (BK). This means that special therapies are needed for attacks that do not respond to traditional antiallergic therapies involving antihistamines, corticosteroids, and epinephrine. Read More

    Novel Therapies for Angiotensin-Converting Enzyme Inhibitor-Induced Angioedema: A Systematic Review of Current Evidence.
    J Emerg Med 2017 Nov 20;53(5):662-679. Epub 2017 Sep 20.
    Department of Pharmacy, Erlanger Health System, Chattanooga, Tennessee.
    Background: Angiotensin-converting enzyme inhibitor (ACEI)-induced angioedema can occur at any point during therapy and, when severe, can require mechanical ventilation. Standard agents for anaphylactic reactions have limited efficacy for bradykinin-mediated angioedema and, therefore, agents approved for hereditary angioedema are increasingly prescribed for these patients.

    Objective Of The Review: This systematic review critically evaluates evidence describing the off-label use of fresh frozen plasma (FFP), prothrombin complex concentrate (PCC), complement 1 esterase inhibitor (C1-INH), icatibant, and ecallantide for treatment of ACEI-induced angioedema. Read More

    Recombinant human C1 esterase inhibitor for acute hereditary angioedema attacks with upper airway involvement.
    Allergy Asthma Proc 2017 Nov 13;38(6):462-466. Epub 2017 Sep 13.
    Background: Recombinant human C1 esterase inhibitor (rhC1-INH) is approved for treatment of hereditary angioedema (HAE) in adolescents and adults. HAE attacks that involve the upper airway can be life threatening, and data on the administration of rhC1-INH for these types of attacks are currently limited.

    Objective: To evaluate the efficacy and safety of rhC1-INH for treatment of acute HAE attacks with upper airway involvement. Read More

    Hereditary angioedema in 2 sisters due to paternal gonadal mosaicism.
    J Allergy Clin Immunol Pract 2018 Jan - Feb;6(1):277-279.e1. Epub 2017 Sep 6.
    Department of Medical Genetics, Faculty of Medicine and Health Sciences, University of Antwerp and Antwerp University Hospital, Antwerp, Belgium.

    Efficacy of recombinant human C1 esterase inhibitor for the treatment of severe hereditary angioedema attacks.
    Allergy Asthma Proc 2017 Nov 5;38(6):456-461. Epub 2017 Sep 5.
    Background: Severe attacks of hereditary angioedema (HAE) are debilitating and potentially life threatening, and can increase anxiety and the use of medical resources.

    Objective: This post hoc assessment evaluated recombinant human C1 esterase inhibitor (rhC1-INH) used to treat acute severe HAE attacks.

    Methods: In a double-blind, randomized-controlled trial (RCT), patients with an HAE attack (baseline visual analog scale score of ≥50 mm, with severe attacks defined as ≥75 mm) were randomly assigned to receive rhC1-INH (50 IU/kg for patients who weighed <84 kg; 4200 IU for patients who weighed ≥84 kg) or placebo. Read More

    Health-related quality of life in relation to disease activity in adults with hereditary angioedema in Sweden.
    Allergy Asthma Proc 2017 Nov 30;38(6):447-455. Epub 2017 Aug 30.
    Background: Health-related quality of life (HR-QoL) is impaired in patients with hereditary angioedema (HAE) but has not yet been satisfactorily described.

    Objective: To study HR-QoL in patients with HAE by combining different HR-QoL instruments with disease activity assessment.

    Methods: All adults in the Swedish HAE registry were invited to take part in this questionnaire study, which used the generic HR-QoL instruments, EuroQol 5 Dimensions 5 Level (EQ-5D-5L) and the RAND Corporation Short Form 36 (RAND-36), the disease-specific Angioedema Quality of Life instrument (AE-QoL), the recently introduced Angioedema Activity Score (AAS) form, and questionnaires on sick leave and prophylactic medication. Read More

    Treatment of hereditary angioedema due to C1 inhibitor deficiency in Argentina.
    Medicina (B Aires) 2017 ;77(4):279-282
    Unidad de Alergia, Asma e Inmunología Clínica, Buenos Aires, Argentina.
    The benefits of the worldwide approval of new drugs for the treatment of acute C1-INH-HAE attacks may still not reach all patients. Identifying the current barriers in the access to medication, as well as conducting a detailed assessment of the progress in this area, is essential to achieve universal treatment. Two hundred and twenty five patients registered in the Argentina Hereditary Angioedema Patient Association (AHAEPA) were randomly selected and invited to participate in a web based questionnaire on accessibility to icatibant and pdC1-INH, self-treatment, delay to treatment, and coverage. Read More

    Angioedema.
    Dtsch Arztebl Int 2017 Jul;114(29-30):489-496
    Department of Otorhinolaryngology, Ulm University Hospital; Department of Anaesthesiology, Ulm University Hospital.
    Background: Acute angioedema of the upper airways can be life-threatening. An important distinction is drawn between mast-cell-mediated angioedema and bradykinin-mediated angioedema; the treatment of these two entities is fundamentally different.

    Methods: This review is based on pertinent articles retrieved by a selective search in PubMed and on guidelines concerning the treatment of angioedema. Read More

    Hereditary angioedema with a mutation in the plasminogen gene.
    Allergy 2018 Feb 7;73(2):442-450. Epub 2017 Sep 7.
    Department of Medical Psychology and Medical Sociology, Johannes Gutenberg University, Mainz, Germany.
    Background: Hereditary angioedema (HAE) with normal C1-INH (HAEnCI) may be linked to specific mutations in the coagulation factor 12 (FXII) gene (HAE-FXII) or functional mutations in other genes that are still unknown. We sought to identify and characterize a hitherto unknown type of HAE with normal C1-INH and without mutation in the F12 gene.

    Methods: The study comprised analysis of whole-exome sequencing, Sanger sequencing, and clinical data of patients. Read More

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