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    Whole Exome Sequencing and Molecular Modeling of a Missense Variant in That Segregates with Disease in a Family with Chronic Urticaria and Angioedema.
    Case Rep Genet 2018 22;2018:6968395. Epub 2018 Feb 22.
    Center for Individualized Medicine, Mayo Clinic, Jacksonville, FL, USA.
    Chronic urticaria is a common condition characterized by recurrent hives lasting several weeks or months and is usually idiopathic. Approximately half of the individuals with chronic urticaria will present with episodes of angioedema that can be severe and debilitating. In this report, we describe a 47-year-old Hispanic male who presented initially for an evaluation of chronic hives following hospitalization due to hive-induced anaphylaxis. Read More

    Prevalence and Clinical Characteristics of Chronic Spontaneous Urticaria in Pediatric Patients.
    Pediatr Allergy Immunol 2018 Apr 21. Epub 2018 Apr 21.
    Charité - Universitätsmedizin Berlin, Department of Dermatology and Allergy, Berlin, Germany.
    Background: Data on the prevalence and disease management of chronic urticaria (CU) and chronic spontaneous urticaria (CSU) in the pediatric population are scarce. The present study assessed the prevalence of CU and CSU, and disease management among pediatric patients (0-17 years).

    Methods: A physician-based online survey was conducted in 5 European countries (United Kingdom, Germany, Italy, France, and Spain) assessing the annual diagnosed prevalence, disease characteristics and treatment patterns in the target population. Read More

    Cervicofacial emphysema following unilateral external dacryocystorhinostomies: A case report.
    Indian J Ophthalmol 2018 May;66(5):722-724
    Haydarpasa Numune Education and Research Hospital, Eye Clinic, Istanbul, Turkey.
    Cervicofacial emphysema (CFE), mostly seen after trauma or dental procedures, is an unexpected, extremely rare condition after uncomplicated dacryocystorhinostomy (DCR). It may be misdiagnosed as angioedema or necrotizing fasciitis. In this article, we present the case of a 40-year-old female with CFE twice after uncomplicated unilateral DCR for left and right sides on different operative days. Read More

    [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

    Severe Adverse Reactions Following Ketoconazole, Fluconazole, and Environmental Exposures: A Case Report.
    Drug Saf Case Rep 2018 Apr 18;5(1):18. Epub 2018 Apr 18.
    Center for Occupational and Environmental Medicine (COEM), 7510 Northforest Drive, North Charleston, SC, 29420, USA.
    In this case report, we describe a 66-year-old man who developed multiple adverse reactions beginning at age 56 after exposure to several azole antifungal drugs including ketoconazole and fluconazole. He also had a history of more than 40 years exposure to chemicals including pesticides, wood preservatives, fertilizers, and welding chemicals. His reactions involved dehydration (requiring several liters of intravenous fluids in less than an hour to alleviate this condition), angioedema, nausea, tinnitus, hypotension, and difficulty breathing. Read More

    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

    Diagnosis, pathogenesis, and treatment of chronic spontaneous urticaria.
    Allergy Asthma Proc 2018 May;39(3):184-190
    Background: Chronic Spontaneous Urticaria (CSU) is an endogenous disorder that is strongly associated with autoimmunity, particularly with immunoglobulin G (IgG) antibody to the alpha subunit of the IgE receptor seen in 35-40% of patients. Basophils and cutaneous mast cells can be activated and lead to a late-phase-like perivascular infiltration about small venules and hive formation.

    Methods: Review of current literature. Read More

    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

    Twenty years' experience with anaphylaxis-like reactions to local anesthetics: genuine allergy is rare.
    J Allergy Clin Immunol Pract 2018 Apr 12. Epub 2018 Apr 12.
    Department of Dermatology, Venereology and Allergy, University Hospital Würzburg, Germany.
    Background: Anaphylaxis-like reactions occur within minutes after application of local anesthetics (LA), most commonly during dental interventions. Impressive symptoms including respiratory distress or loss of consciousness frequently give rise to a suspicion of allergy and may prompt patients and treating physicians to refuse future LA injections.

    Objective: Non-allergic mechanisms are responsible for the majority of LA-induced immediate-type reactions. Read More

    The protective effect of Lactobacillus and Bifidobacterium as the gut microbiota members against chronic urticaria.
    Int Immunopharmacol 2018 Apr 11;59:168-173. Epub 2018 Apr 11.
    Department of Microbiology, Faculty of Medicine, Urmia University of Medical Sciences, Urmia, Iran. Electronic address:
    Background: Chronic Urticaria is a common disorder which is defined by recurrent occurrence of wheals and sometimes angioedema. It has a notable influence on the patients' quality of life. Regulation of the immune system is one of the important roles of the gut microbiota. Read More

    Rationale and design of the comParIson Of sacubitril/valsartaN versus Enalapril on Effect on nt-pRo-bnp in patients stabilized from an acute Heart Failure episode (PIONEER-HF) trial.
    Am Heart J 2018 Apr 10;198:145-151. Epub 2018 Jan 10.
    Thrombolysis in Myocardial Infarction (TIMI) Study Group, Cardiovascular Division, Brigham and Women's Hospital, Harvard Medical School, Boston, MA.
    Objective: The objective is to assess the safety, tolerability, and efficacy of sacubitril/valsartan compared with enalapril in patients with heart failure (HF) with a reduced ejection fraction (EF) stabilized during hospitalization for acute decompensated HF.

    Background: Sacubitril/valsartan, a first-in-class angiotensin receptor-neprilysin inhibitor, improves survival among ambulatory HF patients with a reduced EF. However, there is very limited experience with the in-hospital initiation of sacubitril/valsartan in patients who have been stabilized following hospitalization for acute decompensated HF. Read More

    High-dose antihistamines for chronic spontaneous urticaria in adults.
    • Authors:
    Drug Ther Bull 2018 Apr;56(4):45-48
    Chronic spontaneous urticaria (CSU) is a common skin disease characterised by intermittent weals (hives), angioedema or both lasting for at least 6 weeks.1-3 Second-generation antihistamines are widely used to manage symptoms but are not completely effective in many patients at licensed doses.4 Some guidelines recommend off-label use of high-dose antihistamines as the next therapeutic step. Read More

    Food Allergy Point of Care Pearls.
    Immunol Allergy Clin North Am 2018 May 23;38(2):e1-e8. Epub 2018 Feb 23.
    Departments of Pediatrics and Internal Medicine, Division of Allergy and Immunology, University of Texas Southwestern Medical Center, 5323 Harry Hines Boulevard, Dallas, TX 75390-9063, USA. Electronic address:
    Food allergy should be suspected in individuals with a history of immediate reactivity following ingestion (ie, typically within 20 minutes and almost always within 2 hours) with typical symptoms of immunoglobulin E-mediated reactivity (eg, urticaria, angioedema, coughing, wheezing, vomiting). Testing for food allergy should focus on the most likely allergen to provoke the reaction based on the patient's history. Safe introduction of peanut-containing foods into the diet of an infant at high risk of developing peanut allergy at 4 to 6 months is likely to reduce the risk of peanut allergy. Read More

    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

    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

    Potential Immediate Hypersensitivity Reactions Following Immunization in Preschool Aged Children in Victoria, Australia.
    Hum Vaccin Immunother 2018 Apr 6:1-10. Epub 2018 Apr 6.
    c School of Population & Global Health , University of Melbourne , Victoria , Australia.
    Immediate hypersensitivity reactions (IHR) are rare but potentially serious adverse events following immunization (AEFI). Surveillance of Adverse Events following Vaccination in the Community (SAEFVIC) is an enhanced passive surveillance system that collects, analyses and reports information about AEFI in Victoria, Australia. We describe the incidence, timing and type of potential IHR following vaccination in preschool children reported over an 8-year period. Read More

    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. 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

    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

    Management of anaphylaxis in Spain: pediatric emergency care providers' knowledge.
    Eur J Emerg Med 2018 Apr 4. Epub 2018 Apr 4.
    Department of Pediatric Emergency, Cruces University Hospital - Basque Country University.
    Background: Acute care providers must diagnose and treat patients with anaphylaxis. The objective was to analyze Spanish pediatric emergency departments' (ED) providers' knowledge of the international recommendations for the management of anaphylaxis.

    Methods: A web-based survey including providers (both attending and residents) from seven Spanish pediatric EDs was conducted. Read More

    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

    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

    Angiotensin-Converting Enzyme Inhibitors vs. Angiotensin Receptor Blockers for the Treatment of Hypertension in Adults With Type 2 Diabetes: Why We Favour Angiotensin Receptor Blockers.
    Can J Diabetes 2018 Apr 22;42(2):118-123. Epub 2017 Dec 22.
    Division of Nephrology, Jewish General Hospital, McGill University, Montreal, Quebec, Canada. Electronic address:
    Cardiovascular disease is the principal cause of morbidity and mortality in patients with diabetes mellitus. The incidence or progression of kidney disease is also common in these patients. Several clinical trials have established the efficacy of angiotensin receptor blockers for the prevention of adverse cardiovascular and renal outcomes in this population and are summarized in this review article. Read More

    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

    Epidemiology of urticaria in Poland - nationally representative survey results.
    Postepy Dermatol Alergol 2018 Feb 20;35(1):67-73. Epub 2018 Feb 20.
    Department of Prevention of Environmental Hazards and Allergology, Medical University of Warsaw, Warsaw, Poland.
    Introduction: The prevalence of urticaria is 15-20%. Women are twice as likely to be affected.

    Aim: To present the epidemiology of urticaria and angioedema in Poland. Read More

    Angiotensin-Converting Enzyme Inhibitors in Hypertension: To Use or Not to Use?
    J Am Coll Cardiol 2018 Apr;71(13):1474-1482
    Department of Cardiology and Clinical Research, University Hospital, Bern, Switzerland.
    Most guidelines for the management of patients with cardiovascular disease recommend angiotensin-converting enzyme (ACE) inhibitors as first-choice therapy, whereas angiotensin receptor blockers (ARBs) are merely considered an alternative for ACE inhibitor-intolerant patients. The aim of this review was to compare outcomes and adverse events between ACE inhibitors and ARBs in patients. In patients with hypertension and hypertension with compelling indications, we found no difference in efficacy between ARBs and ACE inhibitors with regard to the surrogate endpoint of blood pressure and outcomes of all-cause mortality, cardiovascular mortality, myocardial infarction, heart failure, stroke, and end-stage renal disease. Read More

    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

    Angioedema as the presenting feature of systemic lupus erythematosus.
    BMJ Case Rep 2018 Mar 28;2018. Epub 2018 Mar 28.
    Department of Medicine, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Puducherry, India.
    A young female in her early 20s presented with low-grade fever for 1 month, puffiness of face and abdominal distension for 15 days. Evaluation revealed pancytopenia with normocellular marrow, hypocomplementaemia and Coomb's positive haemolytic anaemia. She had angioedema with laryngospasm and worsening facial oedema which had dramatic response to steroids. Read More

    The Frequency of Nonsteroidal Anti-Inflammatory Drug Hypersensitivity in Children with Asthma.
    Int Arch Allergy Immunol 2018 Mar 28. Epub 2018 Mar 28.
    Division of Pediatric Allergy and Immunology, Department of Children's Health and Diseases, Faculty of Medicine, Mugla Sitki Kocman University, Mugla, Turkey.
    Background: Nonsteroidal anti-inflammatory drugs (NSAIDs) are the second-most frequent drug type to cause hypersensitivity reactions in children. Asthma is one of the risk factors for NSAID hypersensitivity (NSAID-H) in adult patients. There are limited number of studies evaluating NSAID-H among children with asthma. Read More

    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

    Comparative efficacy of individual renin-angiotensin system inhibitors on major renal outcomes in diabetic kidney disease: a network meta-analysis.
    Nephrol Dial Transplant 2018 Mar 22. Epub 2018 Mar 22.
    Department of respiratory medicine, Zhangjiajie City Hospital, Zhangjiajie, Hunan, China.
    Background: Angiotensin-converting enzyme inhibitors (ACEIs) and angiotensin receptor blockers (ARBs) are two drug classes with well-documented renal protective effects. However, whether there is any difference among individual drugs remains unknown. In this study, we aimed to compare the efficacy of individual ACEIs/ARBs on major renal outcomes in adults with diabetic kidney disease (DKD). Read More

    C1-esterase inhibitor (Cinryze) use in the treatment of pediatric hereditary angioedema.
    Immunotherapy 2018 Mar 23. 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

    Validity and responsiveness of the urticaria activity and impact measure (U-AIM), a new patient-reported tool.
    Ann Allergy Asthma Immunol 2018 Mar 19. Epub 2018 Mar 19.
    Genentech, Inc., South San Francisco, CA.
    Background: Chronic spontaneous urticaria (CSU), also known as chronic idiopathic urticaria (CIU), may produce hives, itch, and angioedema. The Urticaria Activity and Impact Measure (U-AIM) is a newly developed 9-item patient-reported measure designed for use in routine clinical practice to assess CSU activity and impact over the previous 7 days.

    Objective: To evaluate validity, responsiveness, and clinically meaningful change of the U-AIM. Read More

    Analysis of related factors of orolingual angioedema after rt-PA intravenous thrombolytic therapy.
    Eur Rev Med Pharmacol Sci 2018 Mar;22(5):1478-1484
    Department of Internal Neurology, Tianjin Huanhu Hospital, Tianjin, China.
    Objective: Orolingual angioedema (OA) is a rare clinical complication with a potentially fatal risk that occurs after the intravenous application of alteplase (rt-PA) in patients with acute ischemic stroke. The purpose of this work is to investigate the related factors of OA in patients with acute ischemic stroke after the administration of intravenous thrombolytic therapy, to improve the predictive ability of OA during intravenous thrombolytic therapy, and to reduce the prevalence of complications.

    Patients And Methods: We recruited 1223 cases of patients with acute ischemic stroke that were treated in the Department of Neurology No. Read More

    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

    Small bowel angioedema from angiotensin-converting enzyme: Changes on computed tomography.
    Radiol Case Rep 2018 Feb 16;13(1):55-57. Epub 2017 Oct 16.
    Department of Medicine, Section of Digestive Diseases, West Virginia University Hospitals, 1 Medical Center Drive, Box 9161, Morgantown, WV 26506, USA.
    Intestinal angioedema is a rare side effect of angiotensin-converting enzyme inhibitors. We present a 41-year-old woman with sporadic right lower quadrant abdominal pain and diarrhea with multiple computed tomography scans demonstrating enteritis. Suspicion turned to angiotensin-converting enzyme inhibitor use as the cause for the patient's illness after an extensive negative evaluation including labs, stool studies, endoscopies, and capsule endoscopy. Read More

    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

    Refractory Abdominal Pain in a Patient with Chronic Lymphocytic Leukemia: Be Wary of Acquired Angioedema due to C1 Esterase Inhibitor Deficiency.
    Case Rep Hematol 2018 10;2018:7809535. Epub 2018 Jan 10.
    Department of Medicine, Reading Hospital, West Reading, PA, USA.
    Acquired angioedema due to C1 inhibitor deficiency (C1INH-AAE) is a rare and potentially fatal syndrome of bradykinin-mediated angioedema characterized by episodes of angioedema without urticaria. It typically manifests with nonpitting edema of the skin and edema in the gastrointestinal (GI) tract mucosa or upper airway. Edema of the upper airway and tongue may lead to life-threatening asphyxiation. Read More

    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

    Histamine-release test in angioedema patients without urticaria - a retrospective co-hort study of 404 patients.
    Eur Ann Allergy Clin Immunol 2018 Feb 19. Epub 2018 Feb 19.
    Department of Dermatology and Allergy Centre, Odense University Hospital, Odense, Den-mark.
    Summary: A subset of patients with angioedema (AE) and urticaria has histamine releasing autoanti-boies. The histamine release test (HR-test) has been used as a tool in chronic urticaria to de-fine the autoimmune subgroup and may possibly guide the clinician to a more personalized therapy, like omalizumab and cyclosporine. The prevalence and value of positive histamine releasing autoantibodies in monosymptomatic AE is sparsely described in the literature. Read More

    Consensus Statement for the Diagnosis and Treatment of Urticaria: A 2017 Update.
    Indian J Dermatol 2018 Jan-Feb;63(1):2-15
    Department of Dermatology, Krupa Shankar Skin Care Center, Mallige Hospital, Bengaluru, Karnataka, India.
    This article is developed by the Skin Allergy Research Society of India for an updated evidence-based consensus statement for the management of urticaria, with a special reference to the Indian context. This guideline includes updated definition, causes, classification, and management of urticaria. Urticaria has a profound impact on the quality of life and causes immense distress to patients, necessitating effective treatment. Read More

    Health care burden and treatment patterns in commercially insured children with chronic idiopathic/spontaneous urticaria: A real-world study in the United States.
    Allergy Asthma Proc 2018 May 7;39(3):201-211. Epub 2018 Mar 7.
    Herbert Wertheim School of Medicine, Florida International University, Miami, Florida.
    Background: Chronic idiopathic urticaria (CIU)/spontaneous urticaria (CSU) is defined by the presence of wheals, angioedema, or both for ≥6 weeks, with or without an identifiable trigger. Real-world health care data among children with CIU/CSU remain scarce.

    Objectives: To describe treatment patterns, health care resource utilization (HRU), and costs in pediatric patients with CIU/CSU (<12 years old) and to compare these with pediatric patients without CIU/CSU. Read More

    Acta Pol Pharm 2017 May;74(3):983-986
    Angiotensin-converting enzyme inhibitors (ACEI) are one of the most frequently used drugs in the therapy of cardiovascular diseases. Beneficial therapeutic effects may be accompanied by different adverse reactions including angioedema. Recently published data suggest that ACEI-induced angioedema can be life-threatening without emergent intervention, for example due to the respiratory arrest. Read More

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