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Pediatr Int 2019 Jan;61(1):101-103

Department of Pediatrics, Tokyo Women's Medical University Medical Center East, Tokyo, Japan.

J Allergy Clin Immunol Pract 2019 Feb;7(2):754

Allergy/Immunology Associates Inc, Mayfield Heights, Ohio.

Ann Allergy Asthma Immunol 2019 Feb;122(2):228-229

Department of Medical Sciences and Public Health, Unit of Internal Medicine, Allergy and Clinical Immunology, University of Cagliari, Italy.

J Nephrol 2019 Jan 30. Epub 2019 Jan 30.

Department of Emergency and Organ Transplantation, Nephrology, Dialysis and Transplantation Unit, University of Bari, Bari, Italy.

Urinary tract infections (UTIs) after kidney transplantation are associated with significant morbidity. However, data on the impact of UTI on graft survival are controversial. We conducted a retrospective cohort study of 380 kidney transplant patients. Read More

J Manag Care Spec Pharm 2019 Feb;25(2):148-151

1 Division of Rheumatology, Allergy and Immunology, Department of Medicine, University of California San Diego School of Medicine and Medicine Service, San Diego VA Healthcare.

Disclosures: No funding supported the writing of this commentary. The author reports personal fees from BioCryst, CSL Behring, Shire, and Pharming and grants from Ionis. He is chair of the US HAEA Medical Advisory Board and scientific advisor for HAE International. Read More

J Manag Care Spec Pharm 2019 Feb;25(2):143-148

1 Institute for Clinical and Economic Review, Boston, Massachusetts.

Disclosures: Funding for this summary was contributed by the Laura and John Arnold Foundation, Blue Shield of California, and California Health Care Foundation to the Institute for Clinical and Economic Review (ICER), an independent organization that evaluates the evidence on the value of health care interventions. ICER's annual policy summit is supported by dues from Aetna, AHIP Anthem, Blue Shield of California, CVS Caremark, Express Scripts, Harvard Pilgrim Health Care, Cambia Health Solutions, United Healthcare, Kaiser Permanente, Premera Blue Cross, AstraZeneca, Genentech, GlaxoSmithKline, Johnson & Johnson, Merck, National Pharmaceutical Council, Prime Therapeutics, Sanofi, Spark Therapeutics, Health Care Service Corporation, Editas, Alnylam, Regeneron, Mallinkrodt, Biogen, HealthPartners, and Novartis. Agboola, Dreitlein, and Pearson are ICER employees. Read More

Arch Med Sci 2019 01 31;15(1):92-98. Epub 2017 Jul 31.

School and Operative Unit of Allergy and Clinical Immunology, Department of Clinical and Experimental Medicine, University of Messina, Messina, Italy.

Introduction: Hereditary angioedema due to C1-INH deficiency (C1-INH-HAE) or with normal C1-INH is characterized by recurrent swellings due to uncontrolled production of vasoactive mediators, among which bradykinin (BK) is crucial. Through the binding and activation of the two human BK-receptors, kinins may have dual beneficial and deleterious effects in vascular and inflammation physiopathology by inducing oxidative stress. We aimed to assess the serum concentrations of advanced glycation end products (AGEs) and advanced oxidation protein products (AOPPs) in patients affected by HAE. Read More

Pediatr Allergy Immunol 2019 Jan 28. Epub 2019 Jan 28.

Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences, Tehran, Iran.

Hereditary angioedema is an autosomal dominant deficiency of C1-inhibitor (C1-INH-HAE). This rare but potentially fatal disease gives rise to episodes of bradykinin-mediated edema in face, trunk, extremities, genitalia, gastrointestinal tract and upper airway. This leads to severe outcomes including asphyxiation, unnecessary emergency abdominal surgeries, and a considerably diminished quality of life in addition to high economic burden for the health care system. Read More

Clin Exp Allergy 2019 Jan 28. Epub 2019 Jan 28.

Medical Genetics, Department of Clinical and Experimental Medicine, University of Foggia, Foggia, Italy.

Backgound: Different mutations of the angiopoietin-1 gene (ANGPT1) have been associated with the occurrence of Hereditary Angioedema (HAE).

Objective: The purpose of the study is to clarify whether the ANGPT1 A119S variant plays its role via haploinsufficiency or a dominant negative effect.

Methods: The ability of ANGPT1 A119S variant to affect the endothelial barrier function was assessed by immunocytochemistry. Read More

Mol Immunol 2019 Mar 24;107:91-96. Epub 2019 Jan 24.

Centre for Cardiovascular Surgery and Transplantation, Brno, Czech Republic; Department of Clinical Immunology and Allergology, Faculty of Medicine, Masaryk University, Brno, Czech Republic.

Mutations in the C1 inhibitor (C1INH) encoding gene, SERPING1, are associated with hereditary angioedema (HAE) which manifests as recurrent submucosal and subcutaneous edema episodes. The major C1INH function is the complement system inhibition, preventing its spontaneous activation. The presented study is focused on SERPING1 exon 3, an alternative and extraordinarily long exon (499 bp). Read More

J Allergy Clin Immunol Pract 2019 Jan 22. Epub 2019 Jan 22.

Hungarian Angioedema Reference Center, Semmelweis University, Budapest, Hungary.

Background: Hereditary angioedema (HAE) with C1 inhibitor deficiency (C1-INH) is characterized by swelling of subcutaneous and/or submucosal tissues.

Objective: To evaluate efficacy/ safety of fixed-dose subcutaneous plasma-derived C1-INH (pdC1-INH) liquid for HAE attack prevention (NCT02584959).

Methods: Eligible patients were ≥12 years with ≥2 monthly attacks prescreening or pre-long-term prophylaxis. Read More

Exp Ther Med 2019 Feb 16;17(2):1068-1072. Epub 2018 Nov 16.

Allergology and Immunology Discipline, 'Iuliu Hațieganu' University of Medicine and Pharmacy, Cluj-Napoca 400012, Romania.

Angioedema can occur in isolation, accompanied by urticaria, or as a feature of anaphylaxis in mast cell-mediated disorders, bradykinin-mediated disorders, as well as in others with unknown mechanisms, such as infections, rare disorders, or idiopathic angioedema. In mast cell-mediated angioedema, other signs and symptoms of mast cell-mediator release are frequently seen. However, clear evidence of mast cell degranulation may be absent in histaminergic angioedema. Read More

J Allergy Clin Immunol Pract 2019 Jan 17. Epub 2019 Jan 17.

Department of Immunology, Addenbrooke's Hospital, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK CB1 0QQ.

Hautarzt 2019 Feb;70(2):107-115

Klinik für Dermatologie, Venerologie und Allergologie, Allergie-Centrum-Charité/ECARF, Charité - Universitätsmedizin Berlin, Charitéplatz 1, 10117, Berlin, Deutschland.

Angioedema is a spontaneous, edematous swelling of the deep layers of the skin or mucous membrane. Angioedema in the respiratory tract is potentially life-threatening. The classification of angioedema into mast-cell-mediated (e. Read More

Hautarzt 2019 Feb;70(2):101-106

Spezialsprechstunde Angioödeme, Hautklinik und Poliklinik, Universitätsmedizin Mainz, Langenbeckstr. 1, 55131, Mainz, Deutschland.

Manifestation of angioedema can occur at any age. We distinguish between two main subtypes: mast cell mediator-associated angioedema (often with hives) and the non-mast cell mediator-associated angioedema. The patient's history is very important due to the fact that one subtype can be hereditary, but we also have to consider new mutations and even not yet diagnosed patients. Read More

Res Pract Thromb Haemost 2019 Jan 16;3(1):38-43. Epub 2018 Dec 16.

Department of Molecular Cell Biology Sanquin Research & Landsteiner Laboratory Amsterdam The Netherlands.

Congenital deficiency of C1 inhibitor, the main inhibitor of the classic complement system pathway, leads to paroxysmal angioedema (hereditary angioedema) that can be debilitating or life-threatening for affected patients. In the past few years many new insights on the pathogenesis of angioedema formation in the presence of low levels of C1 inhibitor has been accumulated. There is a central role for bradykinin that is released upon activation of the kallikrein-kinin system that is insufficiently controlled by adequate levels of C1 inhibitor. Read More

Hautarzt 2019 Feb;70(2):92-100

Klinik für Dermatologie, Allergologie und Venerologie, Comprehensive Allergy Center (CAC), Medizinische Hochschule Hannover, Carl-Neuberg-Str. 1, 30625, Hannover, Deutschland.

Background: Angioedema can be triggered by mediators bradykinin or histamine. Gender-specific differences and potential biomarkers for follow-up/therapy monitoring are mostly unknown.

Objectives: To what extent are gender-related defects, prodromes, trigger factors, clinical parameters such as number of attacks, frequency, localization, laboratory values, hormones and response to therapy different for the variant types of angioedema. Read More

Allergy Asthma Clin Immunol 2019 5;15. Epub 2019 Jan 5.

2Department of Neuroscience, Division of Dentistry, University of Padua, Via Giustiniani 2, 35128 Padua, Italy.

Background: Melkersson-Rosenthal syndrome (MRS) is a rare, neuro-mucocutaneous disease which presents as orofacial swelling, facial palsy and fissured tongue. These symptoms may occur simultaneously or, more frequently, with a oligosymptomatic or monosymptomatic pattern. Swelling, that is the most common initial finding, may mimic hereditary or acquired angioedema, a disorder caused by histamine or bradykinin-mediated plasma-leakage affecting subcutaneous and/or submucosal tissue. Read More

Ann Allergy Asthma Immunol 2018 Dec 31. Epub 2018 Dec 31.

George Washington School of Medicine and Health Sciences, Washington, DC.

Blood 2018 Dec 27. Epub 2018 Dec 27.

Department of Pathology, Microbiology and Immunology, Vanderbilt University Medical Center, Nashville, TN, United States;

The plasma proteins factor XII (FXII) and prekallikrein undergo reciprocal activation to the proteases FXIIa and kallikrein by a process that is enhanced by surfaces (contact activation), and regulated by the serpin C1-inhibitor. Kallikrein cleaves high-molecular-weight kininogen (HK), releasing the vasoactive peptide bradykinin. Patients with (HAE) experience episodes of soft tissue swelling as a consequence of unregulated kallikrein activity or increased prekallikrein activation. Read More

Vnitr Lek 2018 ;64(10):928-933

Angiotensin-converting enzyme-induced angioedema occurs in 0.1-0.7 % of recipients. Read More

Allergy Asthma Proc 2019 Jan;40(1):7-13

Hereditary angioedema (HAE) is a rare, autosomal dominant, genetic disorder associated with a deficiency in C1 inhibitor protein. HAE is characterized by recurrent and unpredictable episodes of swelling of the extremities, abdomen, face, and upper airway. There are several newly approved drugs as well as investigational products that are currently under study for the management of patients with HAE, with the potential to optimize care and improve quality of life for patients with HAE. Read More

BMJ Case Rep 2018 Dec 13;11(1). Epub 2018 Dec 13.

Internal Medicine, Abington Hospital - Jefferson Health, Abington, Pennsylvania, USA.

Hereditary angioedema (HAE) is a relatively rare clinical entity that can potentially cause life-threatening airway or intestinal oedema, patients with the latter usually presents with symptoms of gastroenteritis like vomiting, diarrhoea and abdominal pain. Here, we present a unique case of a less recognised type of HAE that is type III in a patient who presented with signs and symptoms consistent with infectious colitis. She previously had similar episodes and was managed multiple times with antibiotics, with no satisfactory response. Read More

MBio 2018 12 18;9(6). Epub 2018 Dec 18.

Department of Microbiology and Immunology, University of Michigan Medical School, Ann Arbor, Michigan, USA

Antibiotic-resistant is increasingly recognized as a cause of difficult-to-treat nosocomial infections, including pneumonia, wound infections, and bacteremia. Previous studies have demonstrated that the metalloprotease CpaA contributes to virulence and prolongs clotting time when added to human plasma as measured by the activated partial thromboplastin time (aPTT) assay. Here, we show that CpaA interferes with the intrinsic coagulation pathway, also called the contact activation system, in human as well as murine plasma, but has no discernible effect on the extrinsic pathway. Read More

P T 2018 Dec;43(12):734-735

Mulpleta (lusutrombopag) for thrombocytopenia; ajovy (fremenezumab-vfrm) for migraine; takhzyro (lanadelumab-flyo) for hereditary angioedema attacks. Read More

Allergy 2018 Dec 17. Epub 2018 Dec 17.

Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, SP, Brazil.

J Allergy Clin Immunol Pract 2018 Dec 12. Epub 2018 Dec 12.

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

BioDrugs 2019 Feb;33(1):33-43

Division of Rheumatology, Allergy and Immunology, University of California, San Diego, 8899 University Center Lane, Suite 230, San Diego, CA, 92122, USA.

Hereditary angioedema (HAE) with C1 esterase inhibitor (C1-INH) deficiency (C1-INH-HAE) is a rare disease characterized by diminished levels or dysfunctional activity of C1-INH, leading to dysregulated plasma kallikrein activity within the kallikrein-kinin pathway. Symptoms manifest as painful, potentially life-threatening swelling of subcutaneous tissues throughout the body and/or submucosal edema in the upper airway or gastrointestinal tract. Attacks recur with unpredictable frequency, intensity, and duration, placing a heavy burden on patients' daily lives. Read More

Nurs Open 2019 Jan 28;6(1):126-135. Epub 2018 Aug 28.

AARA Research Center Dallas Texas.

Aims: The aim of this study was to provide recommendations for training patients with hereditary angioedema, based on nursing clinical trial experience, to self-administer subcutaneous C1-INH (C1-INH[SC]) used as routine prophylaxis.

Background: A volume-reduced, subcutaneous C1-INH concentrate (C1-INH(SC); HAEGARDA®; CSL Behring) was recently FDA-approved for the routine prevention of hereditary angioedema attacks. Nurses will play an important role in patient training. Read More

J Clin Invest 2019 Jan 10;129(1):66-68. Epub 2018 Dec 10.

Hereditary angioedema (HAE) is a rare genetic disorder primarily caused by mutations in the SERPING1 gene encoding the C1 inhibitor (C1INH) that leads to plasma deficiency, resulting in recurrent attacks of severe swelling. In the current issue of the JCI, Haslund et al. show that in a subset of patients with type I HAE, mutated C1INH encoded by HAE-causing SERPING1 acts upon wildtype (WT) C1INH in a dominant-negative manner and forms intracellular C1INH aggregates. Read More

Ann Allergy Asthma Immunol 2018 Nov 30. Epub 2018 Nov 30.

Department of Biophysics, Universidade Federal de São Paulo, São Paulo, SP, Brazil. Electronic address:

Hautarzt 2019 Feb;70(2):84-91

Klinik für Dermatologie, Venerologie und Allergologie, Charité - Universitätsmedizin Berlin, Charitéplatz 1, 10117, Berlin, Deutschland.

Background: The classification of angioedema in daily clinical practice is often challenging.

Objectives: The goal is to review the most recent classification of angioedema and to discuss the underlying pathology.

Materials And Methods: Current guidelines and research on the pathophysiology and classification of angioedema were evaluated. Read More

Anticancer Res 2018 Dec;38(12):6801-6807

Internal Medicine Department, Tor Vergata University Hospital, Medical Oncology Unit, Rome, Italy.

Background: Hereditary angioedema (HAE) is an autosomal dominant hereditary disorder characterized by episodic swelling of many body regions (especially throat and abdomen), potentially triggered by medication. No data are available for HAE in patients with cancer assigned to standard chemotherapy. The aim of our study was to identify circulating mediators potentially predictive of acute HAE attacks during chemotherapy. Read More

Case Reports Immunol 2018 29;2018:7435870. Epub 2018 Oct 29.

Cleveland Clinic Abu Dhabi, UAE.

A 27-year-old Emirate man presented to Cleveland Clinic Abu Dhabi emergency department with a 4 year history of recurrent episodes of severe swellings affecting different parts of his body. He used to get 2 swelling episodes every week affecting either his face, hands, feet or scrotum and severe abdominal pain twice a week. Abdominal CT scan and a colonoscopy showed bowel wall oedema. Read More

JAMA 2018 11;320(20):2108-2121

Department of Dermatology and Allergy, Dermatological Allergology, Charité-Universitätsmedizin Berlin, Berlin, Germany.

Importance: Current treatments for long-term prophylaxis in hereditary angioedema have limitations.

Objective: To assess the efficacy of lanadelumab, a fully human monoclonal antibody that selectively inhibits active plasma kallikrein, in preventing hereditary angioedema attacks.

Design, Setting, And Participants: Phase 3, randomized, double-blind, parallel-group, placebo-controlled trial conducted at 41 sites in Canada, Europe, Jordan, and the United States. Read More

Allergy Asthma Clin Immunol 2018 21;14:83. Epub 2018 Nov 21.

10GREPI EA7408, University Grenoble Alpes, Grenoble, France.

Background: Hereditary angioedema due to C1 inhibitor deficiency (C1-INH-HAE) is an autosomal dominant disease resulting in random and unpredictable attacks of swelling. The swelling in C1-INH-HAE is a result of impaired regulation of bradykinin production. The fact that the array of tests needed to diagnose HAE is not always available to the treating physicians is challenging for them and their patients. Read More

RNA 2019 Feb 21;25(2):255-263. Epub 2018 Nov 21.

Alnylam Pharmaceuticals, Cambridge, Massachusetts 02142, USA.

Hereditary angioedema (HAE) is a genetic disorder mostly caused by mutations in the C1 esterase inhibitor gene (C1INH) that results in poor control of contact pathway activation and excess bradykinin generation. Bradykinin increases vascular permeability and is ultimately responsible for the episodes of swelling characteristic of HAE. We hypothesized that the use of RNA interference (RNAi) to reduce plasma Factor XII (FXII), which initiates the contact pathway signaling cascade, would reduce contact pathway activation and prevent excessive bradykinin generation. Read More

J Dtsch Dermatol Ges 2018 Dec 21;16(12):1443-1449. Epub 2018 Nov 21.

Department of Oto-Rhino-Laryngology Head and Neck Surgery, Ulm University Medical Center, Germany.

Background: Hereditary angioedema (HAE) is a rare genetic disorder that leads to recurrent development of mucosal and dermal swelling. It is mediated by bradykinin, and can affect virtually any part of the human body, including the gastrointestinal tract, the extremities, and the laryngeal and pharyngeal regions. Safe and potent drugs are available for acute and prophylactic treatment of HAE. Read More

Braz J Med Biol Res 2018 Nov 14;51(12):e7813. Epub 2018 Nov 14.

Disciplina de Imunologia Clínica, Faculdade de Medicina do ABC, Santo André, SP, Brasil.

Hereditary angioedema (HAE) is a rare autosomal dominant disease due to C1 esterase inhibitor deficiency (C1-INH). The disease is characterized by subcutaneous and submucosal edema in the absence of urticaria due to the accumulation of bradykinin. This descriptive study aimed to evaluate the clinical characteristics of patients with a confirmed diagnosis of HAE referred to our Outpatient Clinic between December 2009 and November 2017. Read More

Case Rep Emerg Med 2018 22;2018:6363787. Epub 2018 Oct 22.

Romanian Network for Hereditary Angioedema, 11a Sântana St, 540256 Tîrgu-Mureş, Romania.

Emergency department (ED) physicians frequently encounter patients presenting with angioedema. Most of these involve histamine-mediated angioedema; however, less common forms of angioedema (bradykinin-mediated) also occur. It is vital physicians correctly recognize and treat this; particularly since bradykinin-mediated angioedema does not respond to antihistamines, corticosteroids or epinephrine and hereditary angioedema (HAE) laryngeal attacks can be fatal. Read More

Presse Med 2018 Nov 8. Epub 2018 Nov 8.

Centre de référence national des angioedèmes (CREAK), 38043 Grenoble, France; Service d'immunologie, CHUGA, 38043 Grenoble, France.

Bradykinin mediated angioedema (BK-AE) can be associated either with C1Inhibitor deficiency (hereditary and acquired forms), either with normal C1Inh (hereditary form and drug induced AE as angiotensin converting enzyme inhibitors…). In case of high clinical suspicion of BK-AE, C1Inh exploration must be done at first: C1Inh function and antigenemy as well as C4 concentration. C1Inh deficiency is significant if the tests are below 50 % of the normal values and controlled a second time. Read More

Nat Biotechnol 2018 Nov;36(11):1027

J Clin Invest 2019 Jan 10;129(1):388-405. Epub 2018 Dec 10.

Department of Biomedicine, Aarhus University, Aarhus, Denmark.

Hereditary angioedema (HAE) is an autosomal dominant disease characterized by recurrent edema attacks associated with morbidity and mortality. HAE results from variations in the SERPING1 gene that encodes the C1 inhibitor (C1INH), a serine protease inhibitor (serpin). Reduced plasma levels of C1INH lead to enhanced activation of the contact system, triggering high levels of bradykinin and increased vascular permeability, but the cellular mechanisms leading to low C1INH levels (20%-30% of normal) in heterozygous HAE type I patients remain obscure. Read More

J Thromb Haemost 2019 Jan 10;17(1):183-194. Epub 2018 Dec 10.

Department of Clinical Chemistry and Haematology, University Medical Center Utrecht, Utrecht University, Utrecht, the Netherlands.

Essentials During contact system activation, factor XII is progressively cleaved by plasma kallikrein. We investigated the role of factor XII truncation in biochemical studies. Factor XII contains naturally occurring truncating cleavage sites for a variety of enzymes. Read More

Forensic Sci Med Pathol 2018 Nov 3. Epub 2018 Nov 3.

Forensic Science SA, 21 Divett Place, Adelaide, 5000, Australia.

An 86-year-old woman with a history of angioedema was found dead at her home address. She had recently complained of a swollen tongue. At autopsy the tongue was grossly edematous, protruding from the mouth. Read More

Gene 2019 Feb 30;685:179-185. Epub 2018 Oct 30.

Center for Research and Molecular Diagnosis of Genetic Diseases, Department of Biophysics, Federal University of São Paulo, Rua Pedro de Toledo, 669, 9° andar, Vila Clementino, 04039-032 São Paulo, SP, Brazil. Electronic address:

Background: Hereditary angioedema (HAE) is a rare genetic disorder mainly caused by mutations in the SERPING1 gene, determining a deficit of C1 inhibitor (C1-INH). In approximately 10% of the cases, HAE with C1-INH deficiency (C1-INH-HAE) is caused by large gene rearrangements, which are not detected by Sanger sequencing. Here we present the exon quantification technique (EQT), a molecular diagnostic test for the detection of large genetic rearrangements in SERPING1, mapping the exact size and location of the deletion caused by the recombination of Alu elements. Read More

Allergy Asthma Clin Immunol 2018 25;14:75. Epub 2018 Oct 25.

1Clinical Center of Allergology, Clinic of Allergy and Asthma, University Hospital "Alexandrovska", Medical University of Sofia, 1, Georgi Sofiiski Str., 1431 Sofia, Bulgaria.

Background: Hereditary angioedema due to C1 inhibitor deficiency (C1-INH-HAE) is a rare autosomal dominant disease characterized by episodes of acute subcutaneous swelling, and/or recurrent severe abdominal pain. The disease is potentially fatal if the upper-airway is involved. Iatrogenic harm can occur if HAE is not considered in the differential diagnosis, the specialists are not aware of the natural history, diagnosis and treatment of HAE, or as a result of unnecessary surgical and other iatrogenic interventions. Read More

Allergy Asthma Clin Immunol 2018 25;14:69. Epub 2018 Oct 25.

1Carol Davila University of Medicine and Pharmacy, Bulevardul Eroii Sanitari, no. 8, District 5, 050474 Bucharest, Romania.

Background: Angioedema due to acquired deficiency of C1-inhibitor (C1-INH-AAE) is a rare disease sharing some clinical and laboratory similarities with hereditary angioedema, but with late onset and no positive family history. The underlining cause may be malignant or due to autoimmune diseases, but some cases remain idiopathic.

Case Presentation: We report a case of a 75 year old woman suffering from recurrent episodes of angioedema since the age of 66, considered first induced by treatment with angiotensin-converting-enzyme inhibitors (ACEI). Read More

Allergy Asthma Clin Immunol 2018 25;14:44. Epub 2018 Oct 25.

Barts Health, Grahame Hayton Unit, Ambrose King Centre, The Royal London Hospital, Whitechapel, London, E1 1BB UK.

Background: Hereditary angioedema (HAE) due to C1 esterase inhibitor (C1-INH) deficiency is characterized by recurrent swelling attacks that can be life-threatening if left untreated. Prompt treatment is vital during acute attacks; plasma-derived C1-INH (Berinert) is one treatment currently licensed for the intravenous treatment of acute HAE attacks in adults, adolescents and children. A new, volume-reduced formulation, of C1-INH is currently available which aims to reduce the time to treatment, and provide greater convenience to patients and healthcare professionals. Read More

Clin Transl Allergy 2018 12;8:42. Epub 2018 Oct 12.

2Department of Dermatology and Allergy, Allergie-Centrum-Charité, Charité-Universitätsmedizin Berlin, Berlin, Germany.

The objective of this analysis was to evaluate the change over time in age at first symptoms, age at diagnosis, and delay in diagnosis using data from the Icatibant Outcome Survey (IOS). Patients with a diagnosis of C1-INH-HAE who were born before the year 1990 and who were diagnosed before they reached 25 years of age were included in the analysis. Both age at diagnosis and delay in diagnosis of C1-INH-HAE appear to decline with later decade of birth, despite wide variation across the countries assessed, suggesting that improved disease awareness causes increased rates of earlier diagnosis over time. Read More