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    1 OF 46

    Recombinant Human C1-Esterase Inhibitor to Treat Acute Hereditary Angioedema Attacks in Adolescents.
    J Allergy Clin Immunol Pract 2017 Feb 12. Epub 2017 Feb 12.
    Department of Medicine, Division of Rheumatology, Allergy, & Immunology, University of California San Diego, San Diego, Calif.
    Background: Recombinant human C1-esterase inhibitor (rhC1-INH) is efficacious and well tolerated for managing hereditary angioedema (HAE) attacks in adults. However, there are insufficient data on its efficacy and safety in adolescents.

    Objective: To evaluate the efficacy and safety profiles of rhC1-INH for acute HAE attacks in adolescents. Read More

    Structure-Guided Design of Novel, Potent, and Selective Macrocyclic Plasma Kallikrein Inhibitors.
    ACS Med Chem Lett 2017 Feb 6;8(2):185-190. Epub 2016 Dec 6.
    Pharmaron Xi'an Co. , Xi'an, Shaanxi 710018, China.
    A series of macrocyclic analogues were designed and synthesized based on the cocrystal structure of small molecule plasma kallikrein (pKal) inhibitor, 2, with the pKal protease domain. This led to the discovery of a potent macrocyclic pKal inhibitor 29, with an IC50 of 2 nM for one olefinic isomer and 42.3 nM for the other olefinic isomer. Read More

    Mutational spectrum of the SERPING1 gene in Swiss patients with Hereditary Angioedema.
    Clin Exp Immunol 2017 Feb 14. Epub 2017 Feb 14.
    Division of Hematology and Central Hematology Laboratory, Department of Internal Medicine, Kantonsspital Lucerne, and University of Berne, Switzerland.
    Hereditary angioedema with C1 inhibitor deficiency (C1-INH-HAE) is a rare autosomal dominant disease caused by mutations in the C1 inhibitor gene SERPING1. Phenotype and clinical features of the disease are very heterogeneous, varying even in the same family. Compared to HAE cohorts in other countries the genetic background of the Swiss HAE patients has not been elucidated yet. Read More

    Screening for hereditary angioedema (HAE) at 13 emergency centers in Osaka, Japan: A prospective observational study.
    Medicine (Baltimore) 2017 Feb;96(6):e6109
    aDepartment of Traumatology and Acute Critical Medicine, Osaka University Graduate School of Medicine bEmergency and Critical Care Medical Center, Osaka Police Hospital cSenri Critical Care Medical Center, Osaka Saiseikai Senri Hospital dEmergency and Critical Care Medicine, Kishiwada Tokushukai Hospital eSenshu Trauma and Critical Care Center, Rinku General Medical Center fEmergency Division, Osaka Red Cross Hospital gDepartment of Emergency Medicine, Osaka General Medical Center hOsaka Prefectural Nakakawachi Medical Center of Acute Medicine iTraumatology and Critical Care Medical Center, National Hospital Organization Osaka National Hospital jEmergency and Critical Care Medical Center, Osaka City General Hospital kOsaka Mishima Emergency Critical Care Center lDepartment of Critical Care Medical Center, Kinki University School of Medicine mDepartment of Trauma and Critical Care Medicine, Osaka City University Graduate School of Medicine, Osaka nDepartment of Internal Medicine, Kyushu University Beppu Hospital, Oita, Japan.
    Hereditary angioedema (HAE) with deficiency of C1 inhibitor (C1-INH) is an autosomal-dominant disease characterized by recurrent episodes of potentially life-threatening angioedema. The objective is to study the incidence of HAE among patients who visit the emergency department.This was a 3-year prospective observational screening study involving 13 urban tertiary emergency centers in Osaka prefecture, Japan. Read More

    Hereditary Angioedema: Implications of Management.
    South Med J 2017 Feb;110(2):101-106
    From the Department of Internal Medicine, University of Florida, Gainesville, and Wake Baptist Hospital, Salisbury, North Carolina.
    Hereditary angioedema (HAE) is a genetic condition that is characterized by frequent episodes of localized angioedema. It is a rare disorder that a primary care provider, otolaryngologist, dermatologist, or rheumatologist may encounter only occasionally. This disease is being reviewed because of the significant advances in further understanding the genetics, biology, and therapeutic management surrounding the condition. Read More

    [Angioedema and the role of bradykinins: new treatments and implications in patients with heart failure].
    G Ital Cardiol (Rome) 2016 Dec;17(12):966-972
    Dipartimento di Scienze Biomediche e Cliniche "Luigi Sacco", Università degli Studi, Ospedale Luigi Sacco, Milano.
    The definition of angioedema is an edema of subcutaneous and submucosal tissues due to increased vascular permeability and fluid extravasation. It can affect different areas, including extremities, genitals, upper airways and intestinal mucosa. The symptoms are disabling and this condition can be fatal if it involves the larynx. Read More

    Hereditary angioedema: health-related quality of life in Canadian patients as measured by the SF-36.
    Allergy Asthma Clin Immunol 2017 19;13. Epub 2017 Jan 19.
    grid.415502.7Division of Clinical Immunology and Allergy, St. Michael's Hospital, Bond Street, 4 CC Specialty Clinics, Toronto, ON M5B 1W8 Canada.
    Background: Hereditary angioedema (HAE) is a rare but serious condition characterized by recurrent spontaneous attacks of angioedema affecting superficial tissues of upper respiratory and gastrointestinal tracts. The potentially fatal and disfiguring nature of HAE impacts the health-related quality of life (HRQoL) of patients with this condition.

    Objectives: To assess the health-related quality of life of Canadian patients with HAE using the 36-item Short-Form Health Survey (SF-36v2). Read More

    Health-related quality of life with hereditary angioedema following prophylaxis with subcutaneous C1-inhibitor with recombinant hyaluronidase.
    Allergy Asthma Proc 2017 Jan 16. Epub 2017 Jan 16.
    Background: To estimate health-related quality-of-life changes in patients with hereditary angioedema due to C1-inhibitor (C1-INH) deficiency who received subcutaneous C1-INH with recombinant hyaluronidase (rHuPH20) for attack prophylaxis in a randomized, double-blind, dose-ranging, cross-over study.

    Methods: Patients with type I/II hereditary angioedema received 1000 U of C1-INH with 24,000 U of rHuPH20 or 2000 U of C1-INH with 48,000 U of rHuPH20 every 3-4 days for 8 weeks and then crossed over for another 8-week period. The study was terminated early as a precaution related to non-neutralizing antibodies to rHuPH20. Read More

    Therapeutic complement inhibition: a promising approach for treatment of neuroimmunological diseases.
    Expert Rev Neurother 2017 Jan 17. Epub 2017 Jan 17.
    d Consultant neurologist , Gloucestershire Hospitals NHS Foundation Trust Ringgold standard institution , Great Western Road, Gloucester , United Kingdom of Great Britain and Northern Ireland.
    Introduction: Autoimmunity is an important cause of disease both in the central and peripheral nervous systems. Aetiologies and clinical manifestations are complex and heterogeneous. Inappropriate control of complement activation at inappropriate sites has been recognized as a major determinant in several neurological conditions, including Guillain-Barré syndrome and neuromyelitis optica. Read More

    Glucocorticoid receptor gene polymorphisms in hereditary angioedema with C1-inhibitor deficiency.
    Orphanet J Rare Dis 2017 Jan 10;12(1). Epub 2017 Jan 10.
    Hungarian Angioedema Center, 3rd Department of Internal Medicine, Semmelweis University, Kútvölgyi street 4, H-1125, Budapest, Hungary.
    Background: Hereditary angioedema caused by C1-inhibitor deficiency (C1-INH-HAE) is a rare, autosomal dominant disorder. C1-INH-HAE is characterized by edema-formation, which may occur in response to stress. The individual's response to stress stimuli is partly genetically determined. Read More

    Novel usage of fresh frozen plasma in hereditary angioedema.
    Clin Ter 2016 Nov-Dec;167(6):182-184
    Department of Emergency and Trauma, Hospital Ampang.
    Hereditary angioedema (HAE) is a rare and potentially life threatening autosomal dominant disease characterized by recurrent episodes of cutaneous and mucosal oedema. It results from reduced expression or loss of function of CI-esterase inhibitors (C1-INH). As opposed to the more common histamine-mediated angioedema, HAE does not respond well to conventional treatments with anti-histamines, steroids and adrenaline. Read More

    Peptide Macrocycle Inhibitor of Coagulation Factor XII with Subnanomolar Affinity and High Target Selectivity.
    J Med Chem 2017 Feb 20;60(3):1151-1158. Epub 2017 Jan 20.
    Institute of Chemical Sciences and Engineering, Ecole Polytechnique Fédérale de Lausanne (EPFL) , CH-1015 Lausanne, Switzerland.
    Factor XII (FXII) is a plasma protease that has emerged in recent years as a potential target to treat or prevent pathological thrombosis, to inhibit contact activation in extracorporeal circulation, and to treat the swelling disorder hereditary angioedema. While several protein based inhibitors with high affinity for activated FXII (FXIIa) were developed, the generation of small molecule inhibitors has been challenging. In this work, we have generated a potent and selective FXIIa inhibitor by optimizing a peptide macrocycle that was recently evolved by phage display (Ki = 0. Read More

    Experimental protocol of dental procedures In patients with hereditary angioedema: the role of anxiety and the use of nitrogen oxide.
    Oral Implantol (Rome) 2016 Apr-Jun;9(2):49-53. Epub 2016 Nov 13.
    Department of Systems Medicine, Medical School, University of Rome "Tor Vergata", Rome, Italy.
    Hereditary angioedema (HAE) is a rare disease, little known to the medical and dental community, but with a growing rate of hospitalization over the years. HAE is due to a deficit/dysfunction of C1 esterase inhibitor which leads to an increase in vascular permeability and the appearance of edemas widespread in all body areas. The airways are the most affected and laryngeal swelling, which can occur, it is dangerous for the patient's life, is also a sensitive spot in our daily practice, therefore, it is also important to be aware of all the signs of this disease. Read More

    Hereditary Angioedema as a Metabolic Liver Disorder: Novel Therapeutic Options and Prospects for Cure.
    Front Immunol 2016 30;7:547. Epub 2016 Nov 30.
    Immunology and Allergy Unit, Campbelltown Hospital and Western Sydney University , Sydney, NSW , Australia.
    Hereditary angioedema (HAE) is a rare autosomal dominant disorder caused by mutations of the SERPING1 or the Factor 12 genes. It is potentially fatal, particularly if not identified at an early stage. Apart from androgens, which are contraindicated in children and in pregnant women, a range of effective, albeit very expensive treatments have recently become available for HAE patients. Read More

    Management of Children With Hereditary Angioedema Due to C1 Inhibitor Deficiency.
    Pediatrics 2016 Nov;138(5)
    University of California Medical Center, San Diego, California.
    Hereditary angioedema (HAE) is a potentially life-threatening inherited disease characterized by attacks of skin swelling, severe abdominal pain, and upper airway swelling. Attacks typically begin in childhood, but the appropriate diagnosis is often missed. Attacks do not respond to epinephrine, antihistamines, or glucocorticoids. Read More

    Comparing acquired angioedema with hereditary angioedema (types I/II): findings from the Icatibant Outcome Survey.
    Clin Exp Immunol 2016 Dec 9. Epub 2016 Dec 9.
    Shire, Zug, Switzerland.
    Icatibant is used to treat acute hereditary angioedema with C1 inhibitor deficiency types I/II (C1-INH-HAE types I/II) and has shown promise in angioedema due to acquired C1 inhibitor deficiency (C1-INH-AAE). Data from the Icatibant Outcome Survey (IOS) were analysed to evaluate the effectiveness of icatibant in the treatment of patients with C1-INH-AAE and compare disease characteristics with those with C1-INH-HAE types I/II. Key medical history (including prior occurrence of attacks) was recorded upon IOS enrolment. Read More

    Subcutaneous administration of human C1 inhibitor with recombinant human hyaluronidase in patients with hereditary angioedema.
    Allergy Asthma Proc 2016 Nov;37(6):489-500
    Division of Rheumatology, Allergy and Immunology, US HAEA Angioedema Center, University of CaliforniaSan Diego School of Medicine, La Jolla, California, USA.
    Background: The currently approved method of C1 inhibitor (C1 INH) administration for patients with hereditary angioedema with C1 INH deficiency (HAE) is by intravenous injection. A C1 INH subcutaneous formulation may provide an attractive mode of administration for some patients.

    Objective: To evaluate efficacy and safety of two doses of subcutaneous, plasma-derived C1 INH with the dispersing agent, recombinant human hyaluronidase (rHuPH20) to prevent angioedema attacks in patients with HAE. Read More

    Effect of bradykinin receptor antagonism on ACE inhibitor-associated angioedema.
    J Allergy Clin Immunol 2016 Nov 29. Epub 2016 Nov 29.
    Department of Medicine, Vanderbilt University Medical Center, Nashville, Tenn. Electronic address:
    Background: The B2 receptor antagonist icatibant is approved for treatment of attacks of hereditary angioedema. Icatibant has been reported to decrease time-to-resolution of angiotensin-converting enzyme (ACE) inhibitor-associated angioedema in 1 study of European patients.

    Objective: We sought to test the hypothesis that a bradykinin B2 receptor antagonist would shorten time-to-resolution from ACE inhibitor-associated angioedema. Read More

    Treatment for hereditary angioedema with normal C1-INH and specific mutations in the F12 gene (HAE-FXII).
    Allergy 2017 Feb 1;72(2):320-324. Epub 2016 Dec 1.
    Department of Medical Psychology and Medical Sociology, Johannes Gutenberg University, Mainz, Germany.
    Hereditary angioedema with normal C1 esterase inhibitor and mutations in the F12 gene (HAE-FXII) is associated with skin swellings, abdominal pain attacks, and the risk of asphyxiation due to upper airway obstruction. It occurs nearly exclusively in women. We report our experience treating HAE-FXII with discontinuation of potential trigger factors and drug therapies. Read More

    Hereditary angioedema (HAE): a cause for recurrent abdominal pain.
    BMJ Case Rep 2016 Nov 14;2016. Epub 2016 Nov 14.
    Department of Cardiology, Maimonides Medical Center, Brooklyn, New York, USA.
    A 44-year-old Hispanic woman presented to the emergency room with a 2-day history of sudden onset of severe cramping left lower quadrant abdominal pain associated with ∼20 episodes diarrhoea. Abdominal CT scan exhibited bowel wall oedema and acute extensive colitis. On the basis of the preliminary diagnosis of acute abdomen, the patient was admitted under the surgical team and treated for acute colitis. Read More

    C1-esterase inhibitor for short-term prophylaxis in a patient with hereditary angioedema with normal C1 inhibitor function.
    J Clin Anesth 2016 Dec 18;35:488-491. Epub 2016 Oct 18.
    Department of Anesthesia, Trauma, Emergency and Critical Care Program, Sunnybrook Health Sciences Centre, University of Toronto, Toronto, ON M4N 3M5, Canada.
    Hereditary angioedema with normal C1-esterase inhibitor (HAE-nC1INH) perioperative is a rare condition which could have potential disastrous ramifications for the anesthesiologist in the perioperative period. However, there is limited evidence and/or guidelines on the optimal way to manage these patients. We present the case of a patient with HAE-nC1INH who was successfully managed in the perioperative period with plasma derived C1-esterase inhibitor (pdC1INH). Read More


    Hereditary haemorrhagic telangiectasia: to transplant or not to transplant - is there a right time for liver transplantation?
    Liver Int 2016 Dec;36(12):1735-1740
    Division of Clinical Immunology and Allergology, Department of Medical Specialties, University Hospitals and Medical Faculty, Geneva, Switzerland.
    Background & Aims: Hereditary haemorrhagic telangiectasia is characterized by arterio-venous malformations (AVM). It frequently involves the liver without clinical symptoms, but may lead to biliary ischaemia, portal hypertension, or fatal high-output heart failure. The indication of liver transplantation is controversial. Read More

    Hereditary haemorrhagic telangiectasia: to transplant or not to transplant?
    Liver Int 2016 Dec;36(12):1745-1747
    Interdisciplinary Department of Medicine, University of Bari School of Medicine, Bari, Italy.
    The Association of hereditary hemorrhagic telangiectasia (HHT) and type I hereditary angioedema is a very rare condition in medicine. The case reported by Muller et al., describes the coexistence of the two diseases and emphasizes the need for listing HHT patients for liver transplantation in case indications occurs, such as the presence of HCC, abnormally increased cardiac output, and gastrointestinal bleeding as reported for this case. Read More

    C1 Esterase Inhibitor (Berinert) for ACE Inhibitor-Induced Angioedema: Two Case Reports.
    J Pharm Pract 2016 Nov 11. Epub 2016 Nov 11.
    Pharmacy Department, The Valley Hospital, Ridgewood, NJ, USA.
    Objective: To describe 2 cases of angiotensin-converting enzyme inhibitor (ACEI)-induced angioedema treated with C1 esterase inhibitor (human) [Berinert].

    Summary: Case 1 is a 60-year-old Caucasian male with angioedema from lisinopril. He was initially treated with a conventional regimen of an antihistamine, methylprednisolone, epinephrine, and fresh frozen plasma. Read More

    A Case of Type 2 Hereditary Angioedema With SERPING1 Mutation.
    Allergy Asthma Immunol Res 2017 Jan;9(1):96-98
    Division of Allergy and Immunology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea.
    Hereditary angioedema is a disease of congenital deficiency or functional defect in the C1 esterase inhibitor (C1-INH) consequent to mutation in the SERPING1 gene, which encodes C1-INH. This disease manifests as recurrent, non-pitting, non-pruritic subcutaneous, or submucosal edema as well as an erythematous rash in some cases. These symptoms result from the uncontrolled localized production of bradykinin. Read More

    Cytokine and estrogen stimulation of endothelial cells augments activation of the prekallikrein-high molecular weight kininogen complex: Implications for hereditary angioedema.
    J Allergy Clin Immunol 2016 Nov 5. Epub 2016 Nov 5.
    Department of Medicine, Medical University of South Carolina, Charleston, SC.
    Background: When the prekallikrein-high molecular weight kininogen complex is bound to endothelial cells, prekallikrein is stoichiometrically converted to kallikrein because of release of heat shock protein-90 (Hsp90). Although bradykinin formation is typically initiated by factor XII autoactivation, it is also possible to activate factor XII either by kallikrein, thus formed, or by plasmin.

    Objective: Because attacks of hereditary angioedema can be related to infection and/or exposure to estrogen, we questioned whether estrogen or cytokine stimulation of endothelial cells could augment release of Hsp90 and prekallikrein activation. Read More

    Subcutaneous Icatibant for the Treatment of Hereditary Angioedema Attacks: Comparison of Home Self-Administration with Administration at a Medical Facility.
    J Allergy Clin Immunol Pract 2016 Nov 3. Epub 2016 Nov 3.
    Division of Rheumatology, Allergy, and Immunology, Department of Medicine, Massachusetts General Hospital, Harvard Medical School, Boston, Mass.
    Background: Hereditary angioedema (HAE) is a life-threatening disorder characterized by recurrent angioedema. Icatibant, a subcutaneous bradykinin-B2-receptor antagonist, is an effective on-demand therapy. Data outside the United States suggest that self-administration is tolerated and patient-preferred compared with administration by health care professionals at medical facilities (HCP-administration). Read More

    How Dextran Sulfate Affects C1-inhibitor Activity: A Model for Polysaccharide Potentiation.
    Structure 2016 Dec 3;24(12):2182-2189. Epub 2016 Nov 3.
    Department of Biophysical Structural Chemistry, Gorlaeus Laboratories, Leiden University, Einsteinweg 55, 2333 CC Leiden, the Netherlands. Electronic address:
    C1-inhibitor is a key inhibitor of the complement and contact activation systems, and mutations in the protein can cause hereditary angioedema. Through an unknown mechanism, polysaccharides can increase C1-inhibitor activity against some of its target proteases. Here we present the crystal structures of the serine protease inhibitor (serpin) domain of active C1-inhibitor by itself and in complex with dextran sulfate. Read More

    [Angioedema].
    Rev Alerg Mex 2016 Oct-Dec;63(4):373-384
    Universidad de Antioquia. Medellín, Antioquia, Colombia.
    Angioedema is defined as edema of the skin or mucosa, including the respiratory and the gastrointestinal mucosa, which is self-limiting, and in most cases is completely resolved in less than 72 hours. It occurs due to increased permeability of the mucosal and submucosal capillaries and postcapillary venules, with resulting plasma extravasation. There are different types of angioedema: histaminergic (which may be mediated by immunoglobulin E), hereditary, from acquired C1 inhibitor deficiency, from angiotensin converting enzyme inhibitor, bradykinin-mediated, and non-histaminergic idiopathic angioedema. Read More

    Hereditary angioedema with F12 mutation: Clinical features and enzyme polymorphisms in 9 Southwestern Spanish families.
    Ann Allergy Asthma Immunol 2016 Nov 24;117(5):520-526. Epub 2016 Oct 24.
    Immunology Department, Virgen del Rocío University Hospital, Seville, Spain.
    Background: Information on F12 mutation hereditary angioedema (HAE) is still limited, but Spain is now recognized as having one of the highest concentrations of cases in Western Europe.

    Objective: To describe unique features of HAE in Spanish carriers of the F12 mutation and investigate a potential role for angiotensin-converting enzyme (ACE) and aminopeptidase-P polymorphisms in disease expression.

    Methods: This was a prospective observational cohort study of 35 individuals (80% females) from 9 unrelated families carrying the p. Read More

    Assessment of inhibitory antibodies in patients with hereditary angioedema treated with plasma-derived C1 inhibitor.
    Ann Allergy Asthma Immunol 2016 Nov 24;117(5):508-513. Epub 2016 Oct 24.
    CSL Behring, King of Prussia, Pennsylvania. Electronic address:
    Background: Limited data are available regarding C1 inhibitor (C1-INH) administration and anti-C1-INH antibodies.

    Objective: To assess the incidence of antibody formation during treatment with pasteurized, nanofiltered plasma-derived C1-INH (pnfC1-INH) in patients with hereditary angioedema with C1-INH deficiency (C1-INH-HAE) and the comparative efficacy of pnfC1-INH in patients with and without antibodies.

    Methods: In this multicenter, open-label study, patients with C1-INH-HAE (≥12 years of age) were given 20 IU/kg of pnfC1-INH per HAE attack that required treatment and followed up for 9 months. Read More

    Regulation of Complement and Contact System Activation via C1 Inhibitor Potentiation and Factor XIIa Activity Modulation by Sulfated Glycans - Structure-Activity Relationships.
    PLoS One 2016 26;11(10):e0165493. Epub 2016 Oct 26.
    Department of Pharmaceutical Biology, Pharmaceutical Institute, Christian-Albrechts-University of Kiel, Kiel, Schleswig-Holstein, Germany.
    The serpin C1 inhibitor (C1-INH) is the only regulator of classical complement activation as well as the major regulator of the contact system. Its importance is demonstrated by hereditary angioedema (HAE), a severe disease with potentially life-threatening attacks due to deficiency or dysfunction of C1-INH. C1-INH replacement is the therapy of choice in HAE. Read More

    Misdiagnosis trends in patients with hereditary angioedema from the real-world clinical setting.
    Ann Allergy Asthma Immunol 2016 Oct;117(4):394-398
    Allergy Department, Hospital La Paz Institute for Health Research, Biomedical Research Network on Rare Diseases (CIBERER, U754), Madrid, Spain.
    Background: Hereditary angioedema due to C1 inhibitor deficiency (C1-INH-HAE) causes swelling in the skin and upper airways and pain in the abdomen because of mucosal swelling. C1-INH-HAE is frequently misdiagnosed, leading to delays in diagnosis, inadequate treatment, and unnecessary procedures.

    Objective: To evaluate the history of misdiagnosis in patients participating in the Icatibant Outcome Survey (IOS). Read More

    Hereditary Angioedema with Recurrent Abdominal Pain in a Patient with a Novel Mutation.
    Intern Med 2016;55(19):2885-2887. Epub 2016 Oct 1.
    Emergency and Critical Care Center, Kishiwada Tokushukai Hospital, Japan.
    We describe a patient with hereditary angioedema type I. The patient had experienced recurrent abdominal pain around the time of her menstrual period for 13 years. A laboratory examination showed reduced functional and antigenic levels of C4 and C1 inhibitor (C1-INH). Read More

    Overview of Laboratory Testing and Clinical Presentations of Complement Deficiencies and Dysregulation.
    Adv Clin Chem 2016;77:1-75. Epub 2016 Jul 5.
    Mayo Clinic, Rochester, MN, United States. Electronic address:
    Historically, complement disorders have been attributed to immunodeficiency associated with severe or frequent infection. More recently, however, complement has been recognized for its role in inflammation, autoimmune disorders, and vision loss. This paradigm shift requires a fundamental change in how complement testing is performed and interpreted. Read More

    Use of a C1 Inhibitor Concentrate in Adults ≥65 Years of Age with Hereditary Angioedema: Findings from the International Berinert(®) (C1-INH) Registry.
    Drugs Aging 2016 Nov;33(11):819-827
    CSL Behring, 1020 First Ave, King of Prussia, PA, 19406, USA.
    Background: Treatment of hereditary angioedema (HAE) in 'older adults' (those aged ≥65 years) has not been well studied. The international Berinert Patient Registry collected data on the use of intravenous plasma-derived, pasteurized, nanofiltered C1-inhibitor concentrate (pnfC1-INH; Berinert(®)/CSL Behring) in patients of any age, including many older adults.

    Methods: This observational registry, conducted from 2010 to 2014 at 30 US and seven European sites, gathered prospective (post-enrollment) and retrospective (pre-enrollment) usage and adverse event (AE) data on subjects treated with pnfC1-INH. Read More

    Perioperative course in patients with hereditary or acquired angioedema.
    J Clin Anesth 2016 Nov 5;34:385-91. Epub 2016 Jun 5.
    Department of Anesthesiology, Mayo Clinic, Rochester, MN. Electronic address:
    Purpose: Two types of bradykinin-mediated angioedema, hereditary angioedema (HAE) and acquired angioedema (AAE), result from deficiency or dysfunction of C1 esterase inhibitor, leading to an overproduction of bradykinin, which can lead to vascular permeability and life-threatening angioedema of the airway. The objective of this study was to review perioperative outcomes in a series of patients with HAE and AAE and to review current knowledge about anesthetic complications in patients with HAE or AAE.

    Methods: Medical records were retrospectively reviewed for perioperative complications in patients with HAE or AAE who underwent general anesthesia from January 1, 2000, to December 31, 2014, at our institution. Read More

    High attack frequency in patients with angioedema due to C1-inhibitor deficiency is a major determinant in switching to home therapy: a real-life observational study.
    Orphanet J Rare Dis 2016 Sep 29;11(1):133. Epub 2016 Sep 29.
    Dipartimento di Science Mediche Traslazionali, Università degli Studi di Napoli Federico II, Via S. Pansini 5, 80131, Naples, Italy.
    Background: Hereditary angioedema with C1-inhibitor deficiency (C1-INH-HAE) is characterized by recurrent attacks of swelling that affect various body sites. Such attacks are a frequent cause of visits to the emergency department and are often treated in the hospital. In recent years, self-administration of C1-inhibitor (C1-INH) concentrates at home has become an increasingly used option, with a positive impact on patient outcomes and quality of life. Read More

    Kininogen Cleavage Assay: Diagnostic Assistance for Kinin-Mediated Angioedema Conditions.
    PLoS One 2016;11(9):e0163958. Epub 2016 Sep 29.
    GREPI EA7408, Université Grenoble Alpes, Grenoble, France.
    Background: Angioedema without wheals (AE) is a symptom characterised by localised episodes of oedema presumably caused by kinin release from kininogen cleavage. It can result from a hereditary deficiency in C1 Inhibitor (C1Inh), but it can present with normal level of C1Inh. These forms are typically difficult to diagnose although enhanced kinin production is suspected or demonstrated in some cases. Read More

    Efficacy of Treatment of Non-hereditary Angioedema.
    Clin Rev Allergy Immunol 2016 Sep 27. Epub 2016 Sep 27.
    Laboratory of Translational Immunology, University Medical Center Utrecht, Utrecht, The Netherlands.
    Non-hereditary angioedema (AE) with normal C1 esterase inhibitor (C1INH) can be presumably bradykinin- or mast cell-mediated, or of unknown cause. In this systematic review, we searched PubMed, EMBASE, and Scopus to provide an overview of the efficacy of different treatment options for the abovementioned subtypes of refractory non-hereditary AE with or without wheals and with normal C1INH. After study selection and risk of bias assessment, 61 articles were included for data extraction and analysis. Read More

    Emergency Department Management of Hereditary Angioedema Attacks: Patient Perspectives.
    J Allergy Clin Immunol Pract 2017 Jan - Feb;5(1):128-134.e4. Epub 2016 Sep 21.
    Division of Rheumatology, Allergy, and Immunology, Department of Medicine, Massachusetts General Hospital, Harvard Medical School, Boston, Mass.
    Background: Emergency department (ED) management of hereditary angioedema (HAE) has been hindered by misdiagnosis and limited treatment options. Food and Drug Administration approval of 4 on-demand HAE therapies starting in 2009 and the publication of ED guidelines for angioedema management in 2014 should facilitate improvement of HAE management in the ED.

    Objective: The objective of this study was to identify patient-reported areas for improvement in ED management of HAE attacks. Read More

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