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    1 OF 49
    Hereditary angioedema: The plasma contact system out of control.
    J Thromb Haemost 2018 Jun 19. Epub 2018 Jun 19.
    Department of Clinical Chemistry and Haematology, University Medical Center Utrecht, Utrecht, the Netherlands.
    The plasma contact system contributes to thrombosis in experimental models. Even though our standard blood coagulation tests are prolonged when plasma lacks contact factors, this enzyme system appears to have a minor (if any) role in haemostasis. In this review, we will explore the clinical phenotype of C1 esterase inhibitor (C1-INH) deficiency. Read More
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    Update on the Use of C1-Esterase Inhibitor Replacement Therapy in the Acute and Prophylactic Treatment of Hereditary Angioedema.
    Clin Rev Allergy Immunol 2018 Jun 16. Epub 2018 Jun 16.
    Allergy, Asthma and Immunology, 23-00 Route 208 South, Fair Lawn, NJ, 07410, USA.
    In the vast majority of patients with hereditary angioedema (HAE), angioedema attacks are due to the quantitative or functional deficiency of C1-esterase inhibitor (C1-INH), which leads to increased vascular permeability and unregulated release of bradykinin. Exogenous administration of C1-INH is a rational way to restore the concentration and functional activity of this protein, regulate the release of bradykinin, and attenuate or prevent subcutaneous and submucosal edema associated with HAE. Recent international guidelines for the management of HAE include C1-INH as an option for acute treatment of HAE. Read More
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    Impaired Endothelial Function in Hereditary Angioedema During the Symptom-Free Period.
    Front Physiol 2018 16;9:523. Epub 2018 May 16.
    Unit of Internal Medicine, Department of Medical Sciences and Public Health, Allergy and Clinical Immunology, University of Cagliari, Cagliari, Italy.
    The presence of coronary endothelial dysfunction was previously shown in Hereditary Angioedema (HAE) patients. The aim of our study was to evaluate the effect of HAE on systemic endothelial function and whether there was a relationship among endothelial function, asymmetric dimethylarginine (ADMA) -which is a strong inhibitor of nitric oxide synthesis-, and disease severity scores. Twenty-four HAE patients (18 females, aged 47. Read More
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    Food as a trigger for abdominal angioedema attacks in patients with hereditary angioedema.
    Orphanet J Rare Dis 2018 Jun 5;13(1):90. Epub 2018 Jun 5.
    Division of Allergology, Department of Rheumatology,Immunology and Allergology, University Hospital Berne, Berne, Switzerland.
    Background: Hereditary angioedema with C1 inhibitor deficiency (C1-INH-HAE) is a rare inherited disease. In most HAE-affected subjects, defined trigger factors precede angioedema attacks. Mechanisms of how trigger factors stimulate the contact activation pathway with bradykinin generation are not well elucidated. Read More
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    The Diagnosis of Hereditary Angioedema: Family Caregivers' Experiences.
    Clin Nurs Res 2018 Jun 1:1054773818780102. Epub 2018 Jun 1.
    1 University of Almeria, Spain.
    The aim of this study was to understand the experiences of family caregivers in the process of diagnosing hereditary angioedema. An interpretive and qualitative research methodology based on Gadamer's philosophical hermeneutics was carried out. Data collection took place between May 2015 and August 2016 and included a focus group and in-depth interviews with 16 family caregivers. Read More
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    Icatibant for the treatment of hereditary angioedema with C1-inhibitor deficiency in adolescents and in children aged over 2 years.
    Expert Rev Clin Immunol 2018 Jun 24;14(6):447-460. Epub 2018 May 24.
    a Hungarian Angioedema Reference Center, 3rd Department of Internal Medicine , Semmelweis University , Budapest , Hungary.
    Introduction: Hereditary angioedema (HAE) due to C1-inhibitor deficiency (C1-INH-HAE) is a rare disorder with life-threatening complications if untreated. It begins during childhood, and reduces the patient's quality of life. Therefore, the availability of an easily administered agent to relieve unpredictable HAE episodes is indispensable for this age group. Read More
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    Targeted next-generation sequencing for the molecular diagnosis of hereditary angioedema due to C1-inhibitor deficiency.
    Gene 2018 Aug 16;667:76-82. Epub 2018 May 16.
    CeMIA SA, 31 Makrigianni street, GR-41334 Larissa, Greece; Department of Immunology & Histocompatibility, University of Thessaly, School of Health Sciences, Faculty of Medicine, 3 Panepistimiou street, Biopolis, GR-41500 Larissa, Greece. Electronic address:
    SERPING1 genotyping of subjects suspicious for hereditary angioedema due to C1-INH deficiency (C1-INH-HAE) is important for clinical practice as well as for research reasons. Conventional approaches towards the detection of C1-INH-HAE-associated SERPING1 variants are cumbersome and time-demanding with many pitfalls. To take advantage of the benefits of next-generation sequencing (NGS) technology, we developed and validated a custom NGS platform that, by targeting the entire SERPING1 gene, facilitates genetic testing of C1-INH-HAE patients in clinical practice. Read More
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    [Anaphylactic reaction to camomile tea].
    Hautarzt 2018 May 8. Epub 2018 May 8.
    Abteilung für Dermatologie, Krankenhaus Hietzing, Wolkersbergenstr. 1, 1130, Wien, Österreich.
    The case of a man with type I allergy after the intake of camomile tea is presented. About 30 min after consumption he was hospitalised with palmar pruritus, swelling of the eyelids, upper lip and nasal mucosa as well as narrowness of the throat. Hereditary angioedema was excluded. Read More
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    A transcriptomics study of hereditary angioedema attacks.
    J Allergy Clin Immunol 2018 May 4. Epub 2018 May 4.
    Department of Biomedical and Clinical Sciences, Luigi Sacco Hospital, University of Milan, Milan, Italy.
    Background: Hereditary angioedema (HAE) caused by C1-inhibitor deficiency is a lifelong illness characterized by recurrent acute attacks of localized skin or mucosal edema. Activation of the kallikrein/bradykinin pathway at the endothelial cell level has a relevant pathogenetic role in acute HAE attacks. Moreover, other pathways are involved given the variable clinical expression of the disease in different patients. Read More
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    Quantification of human complement C2 protein using an automated turbidimetric immunoassay.
    Clin Chem Lab Med 2018 May 5. Epub 2018 May 5.
    The Binding Site Group Limited, Birmingham, UK.
    Background: The measurement of complement components is clinically useful where a deficiency is suspected, or where excessive activation and consumption are present in disease. C2 deficiency carries an increased risk of developing systemic lupus erythematosus, recurrent infections and atherosclerosis. In this study, we have evaluated The Binding Site's Human Complement C2 SPAPLUS® assay. Read More
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    Epidemiology of Bradykinin-mediated angioedema: a systematic investigation of epidemiological studies.
    Orphanet J Rare Dis 2018 May 4;13(1):73. Epub 2018 May 4.
    Department of Dermatology and Allergy, Charité - Universitätsmedizin Berlin, Berlin, Germany.
    Background: Bradykinin-mediated angioedema (Bk-AE) can be life-threatening and requires specific targeted therapies. Knowledge of its epidemiology may help optimize its management.

    Methods: We systematically searched the medical literature to identify abstracts of interest indexed between 1948 and March, 2016. Read More
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    Brazilian Guidelines for Hereditary Angioedema Management - 2017 Update Part 1: Definition, Classification and Diagnosis.
    Clinics (Sao Paulo) 2018 3;73:e310. Epub 2018 May 3.
    Imunologia Clinica, Faculdade de Medicina do ABC, Santo Andre, SP, BR.
    Hereditary angioedema is an autosomal dominant disease characterized by recurrent angioedema attacks with the involvement of multiple organs. The disease is unknown to many health professionals and is therefore underdiagnosed. Patients who are not adequately diagnosed and treated have an estimated mortality rate ranging from 25% to 40% due to asphyxiation by laryngeal angioedema. Read More
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    Idiopathic Nonhistaminergic Acquired Angioedema Versus Hereditary Angioedema.
    J Allergy Clin Immunol Pract 2018 Apr 30. Epub 2018 Apr 30.
    Hungarian Angioedema Center, 3rd Department of Internal Medicine, Semmelweis University, Budapest, Hungary. Electronic address:
    Background: The mechanism of idiopathic nonhistaminergic acquired angioedema (InH-AAE) has not yet been precisely elucidated. This condition is characterized by recurrent angioedema without wheals.

    Objective: To study the clinical features of InH-AAE, and to make, for the first time, independent comparisons with hereditary angioedema of unknown origin (U-HAE), as well as with hereditary angioedema with C1-inhibitor deficiency (C1-INH-HAE). Read More
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    Cyclization of peptides with two chemical bridges affords large scaffold diversities.
    Nat Chem 2018 Apr 30. Epub 2018 Apr 30.
    Institute of Chemical Sciences and Engineering, Ecole Polytechnique Fédérale de Lausanne (EPFL), Lausanne, Switzerland.
    Successful screening campaigns depend on large and structurally diverse collections of compounds. In macrocycle screening, variation of the molecular scaffold is important for structural diversity, but so far it has been challenging to diversify this aspect in large combinatorial libraries. Here, we report the cyclization of peptides with two chemical bridges to provide rapid access to thousands of different macrocyclic scaffolds in libraries that are easy to synthesize, screen and decode. Read More
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    Suffocation due to Acute Airway Edema in a Patient with Hereditary Angioedema Highlighted the Need for Urgent Improvements in Treatment Availability in Japan.
    Intern Med 2018 Apr 27. Epub 2018 Apr 27.
    Department of Nephrology, Juntendo University Faculty of Medicine, Japan.
    A 42-year-old Japanese man with hereditary angioedema suffered accidental trauma to his jaw in Shizuoka Prefecture, Japan, which gradually caused facial edema. Since plasma-derived human C1 inhibitor (pdh C1-INH) was unavailable, he had to be transferred to Juntendo University Hospital in Tokyo. Due to his severe edema, he suffered asphyxiation leading to cardiopulmonary arrest upon arrival. Read More
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    [Allergic emergencies].
    Hautarzt 2018 May;69(5):352-363
    Klinik und Poliklinik für Dermatologie und Allergologie am Biederstein, Technische Universität München, Biedersteiner Str. 29, 80802, München, Deutschland.
    Both anaphylactic reactions and angioedema in the head and neck area can be life-threatening and require emergency treatment. Therapy needed is primarily directed by the patient's symptoms. The first measures taken should consist of immediate disruption of the allergen contact, adequate positioning of the patient, the insertion of an intravenous catheter and an emergency call. Read More
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    Clinical Features of Hereditary Angioedema in Korean Patients: A Nationwide Multicenter Study.
    Int Arch Allergy Immunol 2018 Apr 25:1-8. Epub 2018 Apr 25.
    Institute of Allergy and Clinical Immunology, Seoul National University Medical Research Center, Seoul, Republic of Korea.
    Background: Hereditary angioedema (HAE) is a genetically heterogeneous autosomal dominant disorder characterized by recurrent episodes of nonpruritic, nonpitting edema increasing after puberty. It can be fatal due to laryngeal or gastrointestinal (GI) involvement with varied and changing frequency of mortality according to studies published from the Western countries. Epidemiological and clinical data of HAE in Asian countries are sparse. Read More
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    Evaluation of avoralstat, an oral kallikrein inhibitor, in a Phase 3 hereditary angioedema prophylaxis trial: The OPuS-2 study.
    Allergy 2018 Apr 24. Epub 2018 Apr 24.
    Department of Dermatology and Allergy, Charité - Universitätsmedizin Berlin, Berlin, Germany.
    Background: Effective inhibition of plasma kallikrein may have significant benefits for patients with hereditary angioedema due to deficiency of C1 inhibitor (C1-INH-HAE) by reducing the frequency of angioedema attacks. Avoralstat is a small molecule inhibitor of plasma kallikrein. This study (OPuS-2) evaluated the efficacy and safety of prophylactic avoralstat 300 or 500 mg compared with placebo. Read More
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    [Acquired C1 esterase inhibitor deficiency via bradykinin-mediated angioedema: Four cases].
    Ann Dermatol Venereol 2018 Apr 16. Epub 2018 Apr 16.
    Dermatologie, CHRU Minjoz, 3, boulevard Alexandre-Fleming, 25030 Besançon, France.
    Background: Acquired C1-esterase inhibitor (C1-INH) deficiency angioedema (C1-INH-AAE) is a form of bradykinin-mediated angioedema. This rare disorder is due to acquired consumption of C1-INH, hyperactivation of the classic pathway of human complement, and potentially fatal recurrent angioedema symptoms. Clinical symptoms of C1-INH-AAE are very similar to those of hereditary angioedema (HAE) but usually appear after the fourth decade of life and induce abdominal pain less frequently. Read More
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    Hereditary angioedema from the patient's perspective: A follow-up patient survey.
    Allergy Asthma Proc 2018 May;39(3):212-223
    Department of Medicine, University of California San Diego, La Jolla, California.
    Background: We conducted our first patient survey at the 2013 hereditary angioedema (HAE) patient summit and learned that, despite several novel therapies, the burden of disease was high.

    Objective: To determine, from the patient's perspective, if any improvements in the current state of HAE care occurred over a two-year period between HAE patient summits.

    Methods: A patient survey was conducted at the 2015 Hereditary Angioedema Association conference by using paper surveys that aimed at understanding the current state of HAE care. Read More
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    In Vitro Fertilization Using Luteinizing Hormone-Releasing Hormone Injections Resulted in Healthy Triplets without Increased Attack Rates in a Hereditary Angioedema Case.
    Case Reports Immunol 2018 13;2018:2706751. Epub 2018 Feb 13.
    Department of Internal Medicine, Division of Allergy and Clinical Immunology, Ege University Medical Faculty, İzmir, Turkey.
    Hereditary angioedema due to C1-inhibitor deficiency (C1-INH-HAE) is a rare, autosomal dominant disorder. The management of pregnant patients with C1-INH-HAE is a challenge for the physician. Intravenous plasma-derived nanofiltered C1-INH (pdC1INH) is the only recommended option throughout pregnancy, postpartum, and breastfeeding period. Read More
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    Safety, effectiveness, and impact on quality of life of self-administration with plasma-derived nanofiltered C1 inhibitor (Berinert®) in patients with hereditary angioedema: the SABHA study.
    Orphanet J Rare Dis 2018 Apr 10;13(1):51. Epub 2018 Apr 10.
    Allergy Department, Hospital La Paz Institute for Health Research, Madrid, Spain.
    Background: Hereditary angioedema with C1 inhibitor deficiency is a disabling, potentially fatal condition characterized by recurrent episodes of swelling. Self-treatment is recommended, in order to reduce admissions to the Emergency Room and the time between the onset of the attack and the treatment, resulting in a better treatment outcome and an improved quality of life (QoL). The purpose of this study is to assess the safety, tolerability, and effect on QoL of self-administration of pnf C1-INH for IV use (Berinert®). Read More
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    Icatibant Outcome Survey in Patients with Hereditary Angioedema: Experience in Israel Compared with Other Countries.
    Isr Med Assoc J 2018 Apr;20(4):227-232
    Shire, Zug, Switzerland.
    Background: Management of patients with hereditary angioedema with C1-inhibitor deficiency (C1-INH-HAE) is evolving worldwide. Evaluating the Israeli experience may provide valuable insights.

    Objectives: To compare demographics and icatibant treatment patterns and outcomes in patients with C1-INH-HAE enrolled in the Icatibant Outcome Survey (IOS) in Israel with those in other countries. Read More
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    First Analysis of SERPING1 Gene in Patients with Hereditary Angioedema in Colombia Reveals Two Genotypic Variants in a Highly Symptomatic Individual.
    J Clin Immunol 2018 Apr 5;38(3):294-299. Epub 2018 Apr 5.
    Programa de Medicina, Facultad de Salud, Universidad Surcolombiana, Calle 9 # 14-02, Neiva, Colombia.
    Hereditary angioedema (HAE) is a heterogeneous genetic disease caused by a deficit in C1 inhibitor (C1-INH) and clinically characterized by sudden events of edema, swelling, and pruritus. Here, we describe the first SERPING1 genotyping in 22 subjects from 4 non-related families, all from southern Colombia. The previously reported heterozygous gene mutations, c. Read More
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    The complex alteration in the network of IL-17-type cytokines in patients with hereditary angioedema.
    Clin Exp Med 2018 Apr 6. Epub 2018 Apr 6.
    Dipartimento Immunologia Allergologia, Istituto Medico Europeo (ISME), Palermo, Italy.
    Hereditary angioedema (HAE) is a rare autosomic-dominant disorder characterized by a deficiency of C1 esterase inhibitor which causes episodic swellings of subcutaneous tissues, bowel walls and upper airways that are disabling and potentially life-threatening. We evaluated n = 17 patients with confirmed HAE diagnosis during attack and remission state and n = 19 healthy subjects. The samples were tested for a panel of IL (Interleukin)-17-type cytokines (IL-1β, IL-6, IL-10, granulocyte-macrophage colony stimulating factor (GM-CSF), IL-17, IL-21, IL-22, IL-23) and transforming growth factor-beta (TGF-β) subtypes. Read More
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    Functional Complement Analysis Can Predict Genetic Testing Results and Long-Term Outcome in Patients With Complement Deficiencies.
    Front Immunol 2018 21;9:500. Epub 2018 Mar 21.
    Department of Allergology, Rheumatology and Clinical Immunology, Children's Hospital Ljubljana, University Medical Centre Ljubljana, Ljubljana, Slovenia.
    Background: Prevalence of complement deficiencies (CDs) is markedly higher in Slovenian primary immunodeficiency (PID) registry in comparison to other national and international PID registries.

    Objective: The purposes of our study were to confirm CD and define complete and partial CD in registered patients in Slovenia, to evaluate frequency of clinical manifestations, and to assess the risk for characteristic infections separately for subjects with complete and partial CD.

    Methods: CD was confirmed with genetic analyses in patients with C2 deficiency, C8 deficiency, and hereditary angioedema or with repeated functional complement studies and measurement of complement components in other CD. Read More
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    Usefulness of C1 esterase inhibitor protein concentrate in the management of hereditary angioedema of oropharyngeal tissue.
    Niger J Clin Pract 2018 Apr;21(4):531-533
    Department of Emergency Medicine, Sisli Hamidiye Etfal Training and Research Hospital, University of Health Sciences, Istanbul, Turkey.
    Hereditary angioedema is an autosomal-dominant disorder caused by mutation of the gene encoding the C1 esterase inhibitor (C1-INH). It manifests as painless, nonpruritic, nonpitting episodic swelling of the subcutaneous tissues, gastrointestinal, and upper respiratory tracts in the absence of urticaria. An attack typically takes 24 h to peak and resolves over 48-72 h. Read More
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    Effectiveness of icatibant for treatment of hereditary angioedema attacks is not affected by body weight: findings from the Icatibant Outcome Survey, a cohort observational study.
    Clin Transl Allergy 2018 23;8:11. Epub 2018 Mar 23.
    7Shire, Zug, Switzerland.
    Background: Icatibant is a bradykinin B2-receptor antagonist used for the treatment of hereditary angioedema attacks resulting from C1-inhibitor deficiency. Treatment is not adjusted by body weight however the impact of body mass index (BMI) on the effectiveness of icatibant is not documented in the literature. We examined disease characteristics and icatibant treatment effectiveness in patients stratified by BMI in the Icatibant Outcome Survey, an ongoing, international, observational study monitoring the real-world safety and effectiveness of icatibant. Read More
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    Optimum Use of Acute Treatments for Hereditary Angioedema: Evidence-Based Expert Consensus.
    Front Med (Lausanne) 2017 12;4:245. Epub 2018 Mar 12.
    Honorary Consultant Immunologist, Department of Clinical Biochemistry and Immunology, Addenbrooke's Hospital, Cambridge University Hospitals NHS Foundation Trust, Cambridge, United Kingdom.
    Acute treatment of hereditary angioedema due to C1 inhibitor deficiency has become available in the last 10 years and has greatly improved patients' quality of life. Two plasma-derived C1 inhibitors (Berinert and Cinryze), a recombinant C1 inhibitor (Ruconest/Conestat alpha), a kallikrein inhibitor (Ecallantide), and a bradykinin B2 receptor inhibitor (Icatibant) are all effective. Durably good response is maintained over repeated treatments and several years. Read More
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    Extremely Delayed Diagnosis of Type II Hereditary Angioedema: Case Report and Review of the Literature.
    Mil Med 2018 Mar 26. Epub 2018 Mar 26.
    Department of Allergy and Immunology, Wilford Hall Ambulatory Surgical Center, 2200 Bergquist Drive, Lackland Air Force Base, TX 78236.
    We present a case with extremely late diagnosis of type II hereditary angioedema (HAE). Given recent advances in HAE treatment, we want to bring physician awareness to this condition and aid in earlier detection. HAE is a disorder associated with episodes of angioedema of the face, larynx, lips, abdomen, or extremities. Read More
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    C1-esterase inhibitor (Cinryze) use in the treatment of pediatric hereditary angioedema.
    Immunotherapy 2018 Jun 23;10(8):635-642. Epub 2018 Mar 23.
    ENT and Allergy Associates, Sleepy Hollow, NY, USA.
    Hereditary angioedema (HAE) is a rare genetic disease resulting in unpredictable and potentially life-threatening subcutaneous and submucosal attacks mediated by the vasoactive peptide, bradykinin. HAE often presents within the first or second decade of life, with attacks increasing in both frequency and severity over time. First-line therapies exert their action by replacing C1 inhibitor (C1-INH) or via blocking the production or function of bradykinin. Read More
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    [EFFICACY, PHARMACOKINETICS, AND SAFETY OF ICATIBANT FOR THE TREATMENT OF JAPANESE PATIENTS WITH AN ACUTE ATTACK OF HEREDITARY ANGIOEDEMA: A PHASE 3 OPEN-LABEL STUDY].
    Arerugi 2018;67(2):139-147
    Internal Medicine Department, Niigata City General Hospital.
    Background: Hereditary angioedema (HAE) is characterized by paroxysmal edema of the skin, gastrointestinal mucosa, and upper respiratory tract.

    Purpose: This study investigated icatibant, a selective bradykinin B2 receptor antagonist, as treatment for Japanese patients with an acute HAE attack.

    Methods: This was an open-label, single-arm, Phase 3 study of Japanese adults with HAE type I or II. Read More
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    A missense mutation in the plasminogen gene, within the plasminogen kringle 3 domain, in hereditary angioedema with normal C1 inhibitor.
    Biochem Biophys Res Commun 2018 03;498(1):193-198
    Institute for Molecular and Preventive Medicine, Kurfürstenstr. 10, 56068 Koblenz, Germany. Electronic address:
    Hereditary angioedema (HAE) is a genetically heterogeneous disease that is characterized by recurrent skin swelling, abdominal pain attacks, and potentially life-threatening upper airway obstruction. The two classic types, HAE types I and II, are both caused by mutations in the complement C1 inhibitor (SERPING1) gene resulting either in a quantitative or a qualitative deficiency of C1 inhibitor. In so-called HAE type III, in contrast, patients show normal C1 inhibitor measurements in plasma ('HAE with normal C1 inhibitor'). Read More
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    Hereditary angioedema education in otolaryngology residencies: survey of program directors.
    Int Forum Allergy Rhinol 2018 Mar 15. Epub 2018 Mar 15.
    Department of Otolaryngology-Head and Neck Surgery, West Virginia University School of Medicine, Morgantown, WV.
    Background: The objective of this work was to assess resident education regarding contemporary management of hereditary angioedema using a web-based survey.

    Methods: An 11-item, multiple-choice, electronic questionnaire was sent to all 106 accredited otolaryngology training programs in November 2016. Questions focused on resident education, management principles, and formalized assessment. Read More
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    Hereditary angioedema due to C1-inhibitor deficiency in Macedonia: clinical characteristics, novel SERPING1 mutations and genetic factors modifying the clinical phenotype.
    Ann Med 2018 May 15;50(3):269-276. Epub 2018 Mar 15.
    b University Clinic of Respiratory and Allergic Diseases Golnik , Golnik , Slovenia.
    Objective: Hereditary angioedema due to C1 inhibitor deficiency (C1-INH-HAE) is a rare disease, characterized by swellings. We aimed to characterize on a clinical and molecular basis C1-INH-HAE patients in the Republic of Macedonia.

    Results: All 15 patients from six unrelated families were diagnosed with C1-INH-HAE type I, with a mean age of symptom onset of 11 years and an average delay of diagnosis of seven years. Read More
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    Hereditary Angioedema: The Economics of Treatment of an Orphan Disease.
    Front Med (Lausanne) 2018 16;5:22. Epub 2018 Feb 16.
    Allergy Division, Internal Medicine, University of Texas Southwestern Medical Center, Dallas, TX, United States.
    This review will discuss the cost burden of hereditary angioedema on patients, healthcare systems, and society. The impact of availability of and access to novel and specific therapies on morbidity, mortality, and the overall burden of disease will be explored along with potential changes in treatment paradigms to improve effectiveness and reduce cost of treatment. The prevalence of orphan diseases, legislative incentives to encourage development of orphan disease therapies and the impact of orphan disease treatment on healthcare payment systems will be discussed. Read More
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    Specialist Advice Support for Management of Severe Hereditary Angioedema Attacks: A Multicenter Cluster-Randomized Controlled Trial.
    Ann Emerg Med 2018 Mar 2. Epub 2018 Mar 2.
    AP-HP, Urgences-Samu 93, hôpital Avicenne, Université Paris 13, Inserm U942, Bobigny, France; Inserm U942.
    Study Objective: Hereditary angioedema is a rare disease associated with unpredictable, recurrent attacks of potentially life-threatening edema. Management of severe attacks is currently suboptimal because emergency medical teams are often unaware of new specific treatments. The objective of this trial is to test whether a dedicated national telephone care-management strategy would reduce resource use during severe hereditary angioedema attacks. Read More
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    Coagulation factor XII in thrombosis and inflammation.
    Blood 2018 Apr 26;131(17):1903-1909. Epub 2018 Feb 26.
    Institute of Clinical Chemistry and Laboratory Medicine, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
    Combinations of proinflammatory and procoagulant reactions are the unifying principle for a variety of disorders affecting the cardiovascular system. The factor XII-driven contact system starts coagulation and inflammatory mechanisms via the intrinsic pathway of coagulation and the bradykinin-producing kallikrein-kinin system, respectively. The biochemistry of the contact system in vitro is well understood; however, its in vivo functions are just beginning to emerge. Read More
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    Evaluation of infectious and non-infectious complications in patients with primary immunodeficiency.
    Cent Eur J Immunol 2017 30;42(4):336-341. Epub 2017 Dec 30.
    Research Center for Immunodeficiencies, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran.
    Introduction: Primary immunodeficiency diseases (PIDs) are a heterogeneous group of genetic immune disorders. PID patients suffer from a variety of complications. The aim of this study was to determine the infectious and non-infectious complications among PID patients. Read More
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    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:
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    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
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    Hereditary and Acquired Angioedema: Heterogeneity of Pathogenesis and Clinical Phenotypes.
    Int Arch Allergy Immunol 2018 26;175(3):126-135. 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
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