SAGE Open Med Case Rep 2017 8;5:2050313X17713912. Epub 2017 Jun 8.

Department of Anesthesiology and Critical Care Medicine, Vanderbilt University Medical Center, Nashville, TN, USA.

Objective: To describe the perioperative management of a patient with acquired angioedema (AAE).

Methods: A 66-year-old Caucasian male presented from an outside hospital with a history of acquired angioedema and gastrointestinal stromal tumor-related intractable urticaria and mastocytosis. He was admitted for urgent laparoscopic partial gastrectomy, secondary to gastric outlet obstruction symptomatology. Read More

Allergy Asthma Clin Immunol 2017 13;13:28. Epub 2017 Jun 13.

Division of Allergy and Clinical Immunology, Department of Medicine, St. Michael's Hospital, Toronto, Canada.

Rationale: Angioedema without co-existent urticaria is due to a limited number of causes, including hereditary and acquired C1 esterase inhibitor deficiency, drug-induced angioedema or idiopathic histaminergic or non-histaminergic angioedema. We describe a cohort of patients with recurrent angioedema whose clinical features and response to medications are distinct from the causes above.

Methods: Patients were accrued retrospectively from an academic allergy practice between 2007 and 2014. Read More

J Allergy Clin Immunol 2017 Jun 7. Epub 2017 Jun 7.

Medical Genetics, Dept. of Clinical and Experimental Medicine, University of Foggia,. Electronic address:

Background: Hereditary angioedema (HAE) is a rare genetic disease usually due to mutation within the C1 inhibitor or the coagulation Factor XII gene. However, in a series of patients with HAE no causative variants have been described and the pathophysiology of the disease remains unknown (U-HAE). Identification of causative genes in U-HAE is valuable for understanding the cause of the disease. Read More

J Allergy Clin Immunol Pract 2017 Jun 7. Epub 2017 Jun 7.

Institute for Asthma and Allergy, Chevy Chase, Md.

Background: Clinical manifestations of hereditary angioedema with C1 inhibitor deficiency (C1-INH-HAE) usually begin in childhood, often intensifying during puberty. Currently there are insufficient efficacy/safety data for HAE therapies in children and adolescents due to the small number of pediatric patients enrolled in studies.

Objective: The objective of this phase 3 study was to evaluate the efficacy/safety of a single subcutaneous dose of icatibant (0. Read More

Mol Immunol 2017 Jun 5. Epub 2017 Jun 5.

3rd Department of Internal Medicine, Semmelweis University, Budapest, Hungary.

Hereditary angioedema (HAE) is a rare, but potentially life-threatening disorder, characterized by acute, recurring, and self-limiting edematous episodes of the face, extremities, trunk, genitals, upper airways, or the gastrointestinal tract. HAE may be caused by the deficiency of C1-inhibitor (C1-INH-HAE) but another type of the disease, hereditary angioedema with normal C1-INH function (nC1-INH-HAE) was also described. The patient population is quite heterogeneous as regards the location, frequency, and severity of edematous attacks, presenting large intra- and inter-individual variation. Read More

Immunol Lett 2017 May 31. Epub 2017 May 31.

Hungarian Angioedema Center, 3(rd) Department of Internal Medicine, Semmelweis University, Kútvölgyi street 4, Budapest, H-1125, Hungary. Electronic address:

Background: Hereditary angioedema with C1-inhibitor deficiency (C1-INH-HAE) is a rare, autosomal dominant disorder. The characteristic episodes of subcutaneous/submucosal edema formation may be preceded by erythema marginatum (EM) - the occurrence of a 'map-like' pattern on the skin. EM can occur as an isolated finding or accompanying a hereditary angiooedema (HAE) attack as well. Read More

J Formos Med Assoc 2017 May 25. Epub 2017 May 25.

Department of Allergy, Immunology and Rheumatology, Mackay Children's Hospital, Taipei, Taiwan.

Ann Allergy Asthma Immunol 2017 Jun 2;118(6):736-737. Epub 2017 May 2.

Allergy Department, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Instituto de Investigación Sanitaria Gregorio Marañón, Madrid, Spain; Biomedical Research Network on Rare Diseases (CIBERER) U761, Madrid, Spain.

Ann Allergy Asthma Immunol 2017 Jun 29;118(6):734-735. Epub 2017 Apr 29.

Haemophilia Centre Rhine Main GmbH, Frankfurt-Mörfelden, Germany.

Hand (N Y) 2017 May 7;12(3):NP46-NP50. Epub 2016 Oct 7.

1 Medical College of Wisconsin, Milwaukee, USA.

Background: Compartment syndrome of the upper extremity is a surgical emergency that, when left untreated, can have dire consequences. Its causes are numerous, one of which is the uncommon entity hereditary angioedema, an autosomal dominant disease resulting in edema in a variety of potential locations, including the extremities. This is only the second time hereditary angioedema has been mentioned in the literature as a cause of compartment syndrome. Read More

Allergy Asthma Proc 2017 May;38(3):216-221

Background: Increased estrogen levels during pregnancy can exacerbate hereditary angioedema (HAE), yet disease and treatment ramifications remain poorly studied in pregnant women.

Objective: Data from the international Berinert Patient Registry were used to evaluate outcomes of pregnancies exposed to plasma-derived, pasteurized, nanofiltered C1-inhibitor concentrate (pnfC1-INH) during routine HAE management.

Methods: This observational registry, conducted between 2010 and 2014 at 30 U. Read More

Allergol Int 2017 Apr 19. Epub 2017 Apr 19.

Division of Nephrology, Department of Internal Medicine, Juntendo University Faculty of Medicine, Tokyo, Japan.

Background: Hereditary angioedema (HAE) is an autosomal dominant disease caused by deficiency of C1 esterase inhibitor. Symptoms of HAE include edema, which can potentially cause suffocation. Some patients with HAE exhibit immunological abnormalities, which could prevent an accurate diagnosis. Read More

Clin Biochem 2017 Apr 12. Epub 2017 Apr 12.

Department of Cancer & Inflammation Research, Institute of Molecular Medicine, University of Southern Denmark, Odense, Denmark.

Objectives: Low complement factor C4 is usually considered a valuable screening tool for patients with the potentially life-threatening hereditary angioedema with C1-inhibitor (C1-INH) deficiency (C1-INH-HAE). However, there are patients with C1-INH-HAE presenting with normal C4 levels. This means, that C1-INH-HAE may potentially be overlooked, if screening is performed only by measurement of C4. Read More

Int J Emerg Med 2017 Dec 13;10(1):15. Epub 2017 Apr 13.

Department of Emergency Medicine, University Hospital, Aintree, Liverpool, UK.

Background: Angioedema is a common presentation in the emergency department (ED). Airway angioedema can be fatal; therefore, prompt diagnosis and correct treatment are vital.

Objective Of The Review: Based on the findings of two expert panels attended by international experts in angioedema and emergency medicine, this review aims to provide practical guidance on the diagnosis, differentiation, and management of histamine- and bradykinin-mediated angioedema in the ED. Read More

Allergy Rhinol (Providence) 2017 Mar;8(1):13-19

Background: The plasma-derived, pasteurized, nanofiltered C1-inhibitor concentrate (pnfC1-INH) is approved in the United States as an intravenous (IV) on-demand treatment for hereditary angioedema (HAE) attacks, and, in Europe, as on demand and short-term prophylaxis.

Objective: This analysis evaluated Berinert Patient Registry data regarding IV pnfC1-INH used as long-term prophylaxis (LTP).

Methods: The international registry (2010-2014) collected prospective and retrospective usage, dosing, and safety data on individuals who used pnfC1-INH for any reason. Read More

Ann Allergy Asthma Immunol 2017 May 1;118(5):631-632. Epub 2017 Apr 1.

University of California-San Francisco, San Francisco, California.

J Eur Acad Dermatol Venereol 2017 Apr 1. Epub 2017 Apr 1.

Shire, Zug, Switzerland.

Background: Hereditary angioedema (HAE) due to C1-inhibitor deficiency (C1-INH-HAE) is a rare, potentially fatal, bradykinin-mediated disease. Icatibant is a bradykinin B2 receptor antagonist originally approved in 2008 in the European Union and 2011 in the United States as an acute therapy option for HAE attacks in adults.

Objective: To compare demographics, disease characteristics and treatment outcomes of icatibant-treated HAE attacks in patients with C1-INH-HAE enrolled in the Icatibant Outcome Survey across six European countries: Austria, France, Germany, Italy, Spain and the UK. Read More

Semin Thromb Hemost 2017 Mar 27. Epub 2017 Mar 27.

Clinical Chemistry, Department of Molecular Medicine and Surgery, Karolinska Institutet and University Hospital, Stockholm, Sweden.

Contact activation is the surface-induced conversion of factor XII (FXII) zymogen to the serine protease FXIIa. Blood-circulating FXII binds to negatively charged surfaces and this contact to surfaces triggers a conformational change in the zymogen inducing autoactivation. Several surfaces that have the capacity for initiating FXII contact activation have been identified, including misfolded protein aggregates, collagen, nucleic acids, and platelet and microbial polyphosphate. Read More

J Stomatol Oral Maxillofac Surg 2017 Apr 22;118(2):109-114. Epub 2017 Feb 22.

Service d'odontologie, hôpital Saint-Julien, CHU de Rouen, 76031 Rouen, France.

Bradykinin-mediated angioedema (AE) is a rare disease characterized by recurrent cutaneous or mucosal angioedema. This hereditary or acquired disease is of rapid installation, non-pruritic, usually painless and can affect the face, lips, larynx, gastrointestinal tract or extremities. When the affected area involves the upper respiratory tract, laryngeal angioedema can lead to imminent death by asphyxia. Read More

N Engl J Med 2017 03;376(12):1131-1140

From Barts Health NHS Trust (H.L.) and St. John's Institute of Dermatology, Guy's Hospital (C.G.), London, and the Clinical Investigation and Research Unit, Royal Sussex County Hospital, Brighton (M.T.) - all in the United Kingdom; Ospedale Luigi Sacco-U.O. Medicina Generale, Milan (M.C.), and the Department of Internal Medicine, University of Catania, Catania (S.N.) - both in Italy; Department of Medicine and Pediatrics, Penn State Hershey Allergy, Asthma, and Immunology, Hershey (T. Craig), and CSL Behring, King of Prussia (D.B.-K., J.E., D.P.) - both in Pennsylvania; the Department of Dermatology, Johannes Gutenberg University Mainz, Mainz (K.B.), and CSL Behring, Marburg (H. Feuersenger, J.-P.L., T.M., I.P.) - both in Germany; Baker Allergy, Asthma and Dermatology Research Center, Portland, OR (J. Baker); Institute for Asthma and Allergy, Chevy Chase, MD (H.H.L.); Allergy and Immunology Unit, Chaim Sheba Medical Center, Tel Hashomer (A.R.), and Allergy and Immunology Unit, Tel Aviv Sourasky Medical Center, Tel Aviv (S.K.) - both in Israel; Clinical Research Center of Alabama, Birmingham (J. Bonner, J.A.); Department of Internal Medicine, Allergy Section Cincinnati, University of Cincinnati College of Medicine, Cincinnati (J.A.B.), and Toledo Institute of Clinical Research, Toledo (S.M.R.) - both in Ohio; Allergy Asthma Research Associates Research Center, Dallas (W.R.L.); Hungarian Angioedema Center, Third Department of Internal Medicine, Semmelweis University, Budapest (H. Farkas); the Department of Medicine, Immunology, and Allergy, Campbelltown Hospital, Campbelltown, NSW, Australia (C.H.K.); the Department of Clinical Immunology and Allergy, St. Michael's Hospital, Toronto (G.L.S.), Centre de Recherche Appliqué en Allergie de Québec, Quebec, QC (J.H.), McMaster University, Hamilton, ON (P.K.K.), Ottawa Allergy Research and University of Ottawa Medical School, Ottawa (W.Y.), and University of Alberta Hospital, Edmonton (B.R.) - all in Canada; Allergy and Asthma Clinical Research, Walnut Creek (J.J.), University of California, San Diego School of Medicine, La Jolla (M.R., B.L.Z.), and 705 W. La Veta Ave., Suite 101, Orange (D.S.L.) - all in California; Medical Research of Arizona, Scottsdale (M.E.M.); Hospital General Universitario Gregorio Marañón and Biomedical Research Network on Rare Diseases-U761, Institute for Health Research, Gregorio Marañón (M.L.B.), and the Allergy Department, Hospital La Paz Institute for Health Research, Biomedical Research Network on Rare Diseases (T. Caballero), Madrid, the Allergy Department, IIS Hospital Universitario La Fe, Valencia (M.D.H.), and Hospital Universitario Vall d'Hebron, Barcelona (M.G.) - all in Spain; Asthma and Allergy Association, Colorado Springs, CO (R.N.); Department of Internal Medicine, Virginia Commonwealth University, Richmond (L.B.S.); Spitalul Clinic Municipal, Cluj-Napoca, Romania (I.C.); Vital Prospects Clinical Research Institute, Tulsa, OK (I.H.); Institute of Clinical Immunology and Allergology, University Hospital, Hradec Kralove, Czech Republic (P.K.); Division of Rheumatology, Allergy, and Immunology, Massachusetts General Hospital, Boston (A.B.); and Marycliff Allergy Specialists, Spokane, WA (R.G.G.).

Background: Hereditary angioedema is a disabling, potentially fatal condition caused by deficiency (type I) or dysfunction (type II) of the C1 inhibitor protein. In a phase 2 trial, the use of CSL830, a nanofiltered C1 inhibitor preparation that is suitable for subcutaneous injection, resulted in functional levels of C1 inhibitor activity that would be expected to provide effective prophylaxis of attacks.

Methods: We conducted an international, prospective, multicenter, randomized, double-blind, placebo-controlled, dose-ranging, phase 3 trial to evaluate the efficacy and safety of self-administered subcutaneous CSL830 in patients with type I or type II hereditary angioedema who had had four or more attacks in a consecutive 2-month period within 3 months before screening. Read More

Orphanet J Rare Dis 2017 Mar 16;12(1):55. Epub 2017 Mar 16.

Department of Dermatology and Allergy Centre, Odense University Hospital, Odense, Denmark.

Background: With a potentially early onset, hereditary angioedema (HAE) requires special knowledge also in infancy and early childhood. In children from families with HAE, the diagnosis should be confirmed or refuted early, which can be difficult. Studies of childhood HAE and the diagnostic approaches are limited. Read More

Clin Imaging 2017 May - Jun;43:122-126. Epub 2017 Mar 8.

Department of Radiology, Northwestern Memorial Hospital, Northwestern University Feinberg School of Medicine, 676 N. Saint Clair St., Suite 800, Chicago, IL 60611, United States. Electronic address:

Angioedema is a condition in which an increase in vascular permeability leads to the swelling of body tissues. There are both hereditary and acquired forms of the disease, with the latter often associated with the administration of angiotensin-converting enzyme inhibitor medication. Involvement of the intestinal tract is a rare manifestation of angioedema, and can present with abdominal pain, nausea, and vomiting. Read More

Crit Care Med 2017 Apr;45(4):725-735

1Division of Pulmonary, Critical Care, Allergy and Immunology, Department of Medicine, Wake Forest Baptist Medical Center, Winston-Salem, NC.2Division of Allergy and Clinical Immunology, Department of Medicine, W.G. (Bill) Hefner VA Medical Center, Salisbury, NC.

Objectives: Angioedema is a potentially life-threatening occurrence that is encountered by critical care providers. The mechanistic understanding of angioedema syndromes has improved in recent years, and novel medications are available that improve outcomes from these syndromes. This clinically focused review will describe the underlying genetics, pathophysiology, classification and treatment of angioedema syndromes, with an emphasis on the novel pharmacologic agents that have recently become available for acute treatment. Read More

Int J Otolaryngol 2017 14;2017:1476402. Epub 2017 Feb 14.

Department of Dermatology and Allergy Centre, Odense University Hospital, Odense, Denmark.

Objective. To asses a cohort of 105 consecutive patients with angiotensin converting enzyme-inhibitor induced angioedema with regard to demographics, risk factors, family history of angioedema, hospitalization, airway management, outcome, and use of diagnostic codes used for the condition. Study Design. Read More

J Allergy Clin Immunol Pract 2017 Mar 9. Epub 2017 Mar 9.

CSL Behring, King of Prussia, Pa.

Ann Allergy Asthma Immunol 2017 Apr 9;118(4):452-455. Epub 2017 Mar 9.

Division of Rheumatology, Allergy and Immunology, Department of Medicine, University of California-San Diego, La Jolla, California.

Background: Symptoms of hereditary angioedema (HAE) attacks can recur soon after initial treatment; the durability of response for recombinant human C1 esterase inhibitor (rhC1INH) treatment is unknown.

Objective: To examine the efficacy and durability of rhC1INH for acute HAE attacks.

Methods: In this pooled post hoc analysis of 2 trials, patients with type I or II HAE (functional C1INH levels <50% of normal) and a baseline visual analog scale score of at least 50 mm were included if they had received at least 1 intravenous dose of 50 IU/kg of rhC1INH. Read More

J Allergy Clin Immunol Pract 2017 Mar 8. Epub 2017 Mar 8.

Department of Biomedical and Clinical Sciences, Luigi Sacco Hospital, University of Milan, Milan, Italy; ASST Fatebenefratelli Sacco, Milan, Italy.

Background: Acquired angioedema due to C1-inhibitor deficiency (C1-INH-AAE) is a rare disease with no prevalence data or approved therapies.

Objective: To report data on patients with C1-INH-AAE followed at Angioedema Center, Milan (from 1976 to 2015).

Methods: Diagnostic criteria included history of recurrent angioedema without wheals; decreased C1-INH antigen levels and/or functional activity of C1-INH and C4 antigen less than 50% of normal; late symptom onset (>40 years); no family history of angioedema and C1-INH deficiency. Read More

Ann Allergy Asthma Immunol 2017 Apr 7;118(4):456-460.e1. Epub 2017 Mar 7.

Division of Rheumatology, Allergy, and Immunology, Department of Medicine, University of California at San Diego, La Jolla, California.

Background: Hereditary angioedema due to C1 inhibitor deficiency (HAE) is a rare, life-threatening disease that imposes a significant burden on affected patients. 17α-alkylated androgens (anabolic androgens) decrease attack frequency and severity but carry the risk of potentially serious dose-related adverse effects. Despite the emergence of targeted therapies for HAE, continued anabolic androgen use has been driven in part by their low cost. Read More

Ann Allergy Asthma Immunol 2017 Apr 6;118(4):520-521. Epub 2017 Mar 6.

Department of Medical Psychology and Medical Sociology, Johannes Gutenberg University, Mainz, Germany.

Pediatr Allergy Immunol 2017 Jun 4;28(4):370-376. Epub 2017 Apr 4.

Division of Allergy and Clinical Immunology, Technion Faculty of Medicine, Bnai Zion Medical Center, Haifa, Israel.

Background: The clinical expressions of hereditary angioedema with C1-inhibitor deficiency (C1-INH-HAE) and its related burden may negatively affect patient quality of life. This study aimed to assess health-related quality of life (HRQoL) in children with C1-INH-HAE.

Methods: Children (N = 98: 34 C1-INH-HAE patients, 64 healthy controls) aged 3-18 years were recruited in Israel and Hungary. Read More

Eur J Dermatol 2017 Apr;27(2):155-159

National Reference Centre for Angioedema, CREAK, France, Department of Internal Medicine, Saint Antoine University Hospital, AP-HP, Paris, France.

Hereditary angioedema (HAE) is a rare genetic disorder characterised by episodes of swelling without urticaria. Berinert® (CSL Behring) is a plasma-derived human C1 inhibitor (C1-INH) concentrate, approved for the treatment of HAE with C1-INH deficiency (C1-INH-HAE), however, it is often used off-label in Europe to treat HAE with normal C1-INH. To report the clinical characteristics of patients with HAE with normal C1-INH (with F12 gene mutation; FXII-HAE) or of unknown origin (U-HAE), and their response to Berinert®. Read More

Immun Inflamm Dis 2017 Mar 11;5(1):29-36. Epub 2017 Jan 11.

Department of Internal Medicine Saint-Antoine Hospital (AP-HP) Paris 6 University, DHUi2B Paris France.

Introduction: The clinical characteristics and icatibant-treatment outcomes of patients with hereditary angioedema with normal C1 inhibitor (HAE-nC1 INH) are limited.

Methods: We retrospectively analyzed data from French HAE patients enrolled in the Icatibant Outcome Survey registry (from July 2009 to September 2013) to compare disease characteristics and the effectiveness and safety of acute icatibant-treated angioedema attacks in patients with HAE-nC1 INH, HAE with C1 INH deficiency (type I), or dysfunction (type II).

Results: One center in Grenoble contributed 22 patients with HAE-nC1 INH and a family history of HAE while 15 centers across France contributed 153 patients with HAE type I and seven patients with HAE type II. Read More

N Engl J Med 2017 02;376(8):788-789

From the Barts Health NHS Trust, London.

N Engl J Med 2017 02;376(8):717-728

From the Division of Rheumatology, Allergy, and Immunology, Department of Medicine, Massachusetts General Hospital, Harvard Medical School, Boston (A.B.), Dyax, Burlington (C. Soo, R.I., D.J.S., C.T., J.A.K., R.F., H.K., R.M., C. Stevens, J.C.B., Y.C., B.A.), and ICON Clinical Research, Marlborough (J.G.S.) - all in Massachusetts; the Division of Clinical Immunology and Allergy, Department of Medicine, Icahn School of Medicine at Mount Sinai, New York (P.B.), and Winthrop University Hospital, Mineola (M.D.-L.) - both in New York; Triumpharma, Amman, Jordan (M.S., A.A.-G.); Asthma and Allergy Research Associates, Dallas (W.L.); the Division of Allergy and Immunology, Washington University School of Medicine, St. Louis (H.J.W.); Allergy and Asthma Medical Group, Walnut Creek (J.J.), and the Department of Rheumatology, Allergy, and Immunology, University of California, San Diego, San Diego (M.R.) - both in California; Baker Allergy, Asthma, and Dermatology, Lake Oswego, OR (J.B.); the Department of Internal Medicine-Allergy Section Cincinnati, University of Cincinnati College of Medicine, Cincinnati (J.A.B.); the Division of Allergy and Immunology, Department of Internal Medicine, Morsani College of Medicine, University of South Florida, Tampa (R.L.); the Institute for Asthma and Allergy, Chevy Chase, MD (H.H.L.); the Department of Medicine and Pediatrics, Penn State Hershey Allergy, Asthma, and Immunology, Hershey, PA (T.C.); and the Department of Biomedical and Clinical Sciences, Luigi Sacco, University of Milan, and Luigi Sacco Hospital Milan, Milan (M.C.).

Background: Hereditary angioedema with C1 inhibitor deficiency is characterized by recurrent, unpredictable swelling episodes caused by uncontrolled plasma kallikrein generation and excessive bradykinin release resulting from cleavage of high-molecular-weight kininogen. Lanadelumab (DX-2930) is a new kallikrein inhibitor with the potential for prophylactic treatment of hereditary angioedema with C1 inhibitor deficiency.

Methods: We conducted a phase 1b, multicenter, double-blind, placebo-controlled, multiple-ascending-dose trial. Read More

Int Arch Allergy Immunol 2017 22;172(1):55-63. Epub 2017 Feb 22.

Rheumatology, Allergology and Clinical Immunology, Department of "Medicina dei Sistemi", University of Rome Tor Vergata, Rome, Italy.

Background: Angioedema (AE) is a potentially life-threatening condition with hereditary (HAE), acquired (AAE), or iatrogenic causes. A careful workup allows for the identification of the etiology of attacks and the appropriate management. In this cohort study, based on a clinical practice setting, we aimed at investigating clinical and laboratory findings concerning different features of patients with recurrent AE who were referred to a single, tertiary-level center for HAE. Read More

Mol Immunol 2017 May 20;85:120-122. Epub 2017 Feb 20.

GREPI, UE7408 Université Grenoble Alpes, Grenoble, France; Centre de Référence des Angioedèmes (CREAK), CHU Grenoble Alpes, Grenoble, France. Electronic address:

Background: Angioedema diagnosis classically targets the complement system (via C1 inhibitor (C1Inh) function and antigenic C4 level) and contact phase activation (via amidase activity). Bradykinin is responsible for angioedema attacks and is produced from contact phase activation secondary to failed C1Inh control.

Objective: We aimed to compare the diagnostic performances of spontaneous amidase activity and antigenic C4 level in C1Inh hereditary angioedema (C1Inh-HAE) patients. Read More

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

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

Clin Exp Immunol 2017 Jun 19;188(3):430-436. Epub 2017 Mar 19.

Division of Hematology and Central Hematology Laboratory, Department of Internal Medicine, Kantonsspital Lucerne, Lucerneand University of Berne, 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 extremely heterogeneous, varying even within the same family. Compared to HAE cohorts in other countries, the genetic background of the Swiss HAE patients has not yet been elucidated. Read More

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

Am J Emerg Med 2017 Jun 24;35(6):942.e5-942.e6. Epub 2017 Jan 24.

Kanuni Sultan Suleyman Research and Education Hospital, Department of Emergency Medicine, Istanbul, Turkey.

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

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

Allergy Asthma Clin Immunol 2017 19;13. Epub 2017 Jan 19.

Division 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

Allergy Asthma Proc 2017 Mar 16;38(2):143-151. 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

Expert Rev Neurother 2017 Jun 6;17(6):579-591. Epub 2017 Mar 6.

d Department of Neurology , Gloucestershire Hospitals NHS Foundation Trust , 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

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

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

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

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