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ACS Med Chem Lett 2022 Aug 29;13(8):1217-1218. Epub 2022 Jul 29.

Smith, Gambrell & Russell LLP, 1105 West Peachtree Street NE, Suite 1000, Atlanta, Georgia 30309, United States.

Thromb Haemost 2022 Aug 17. Epub 2022 Aug 17.

University of Amsterdam, Department of Vascular Medicine, Amsterdam Cardiovascular Sciences, Amsterdam UMC Location AMC, Amsterdam, Netherlands.

Background Congenital prekallikrein deficiency has been associated with increased risk of thrombosis in a few uncontrolled studies. Prekallikrein levels were reduced by 36-94% (median 75%) in a phase 2 study with the prekallikrein specific antisense oligonucleotide, donidalorsen, in patients with hereditary angioedema (HAE). Objectives To estimate the effects of plasma prekallikrein reduction on coagulation and fibrinolysis. Read More

Ann Allergy Asthma Immunol 2022 Aug 14. Epub 2022 Aug 14.

University Hospitals, Cleveland Medical Center, Cleveland, Ohio; Allergy/Immunology Associates Inc., Mayfield Heights, Ohio.

J Allergy Clin Immunol Pract 2022 Aug 11. Epub 2022 Aug 11.

Background: Recommendations regarding family screening for hereditary angioedema (HAE) remain variable and mostly based on expert opinion; and studies evaluating its implementation and efficacy are lacking.

Objectives: A novel HAE screening programme was established to evaluate the efficacy and impact of cascade family screening (CFS) for at-risk relatives.

Methods: Potential HAE relatives were screened through the CFS approach. Read More

J Clin Gastroenterol 2022 Aug 15. Epub 2022 Aug 15.

US HAEA Angioedema Center, University of California San Diego, San Diego, CA.

Health care providers are likely to encounter patients with recurrent unexplained abdominal pain. Because hereditary angioedema (HAE) is a rare disease, it may not be part of the differential diagnosis, especially for patients who do not have concurrent skin swelling in addition to abdominal symptoms. Abdominal pain is very common in patients with HAE, occurring in up to 93% of patients, with recurrent abdominal pain reported in up to 80% of patients. Read More

Front Allergy 2022 31;3:835503. Epub 2022 Mar 31.

CeMIA SA, Larissa, Greece.

Hereditary angioedema with C1 Inhibitor deficiency (C1-INH-HAE) is caused by a constellation of variants of the gene ( = 809; 1,494 pedigrees), accounting for 86.8% of HAE families, showing a pronounced mutagenic liability of and pertaining to 5.6% variants. Read More

Front Allergy 2022 11;3:818741. Epub 2022 Apr 11.

UOC Medicina Generale, ASST Fatebenefratelli Sacco, Ospedale Luigi Sacco-Università degli Studi di Milano, Milan, Italy.

Hereditary angioedema due to C1-inhibitor deficiency (C1-INH-HAE) is characterized by swelling attacks that may be even life-threatening. To reduce the frequency of attacks, some patients need a long-term prophylaxis (LTP). In addition to the intravenous administration, plasma-derived C1-inhibitor (pdC1-INH) has been proved effective also if administered subcutaneously at the dose of 120 IU/kg/week. Read More

Front Genet 2022 18;13:914376. Epub 2022 Jul 18.

Institute of Human Genetics, University of Bonn, School of Medicine and University Hospital Bonn, Bonn, Germany.

Angioedema is a relatively rare but potentially life-threatening adverse reaction to angiotensin-converting enzyme inhibitors (ACEi) and angiotensin receptor blockers (ARBs). As with hereditary forms of angioedema (HAE), this adverse reaction is mediated by bradykinin. Research suggests that ACEi/ARB-induced angioedema has a multifactorial etiology. Read More

J Dtsch Dermatol Ges 2022 08 1;20(8):1127-1129. Epub 2022 Aug 1.

Institute of Allergology, Charité - Universitätsmedizin Berlin, Berlin, Germany.

Qatar Med J 2022 30;2022(2). Epub 2022 Mar 30.

Allergy and Immunology Division, Department of Medicine, Hamad Medical Corporation, Doha, Qatar E-mail:

Allergic diseases are common medical conditions that now show an increasing trend globally and contributes to poor quality of life of the affected individual. Allergies can be fatal in a few instances such as anaphylaxis, severe asthma, and hereditary angioedema if the symptoms are not recognized and treated correctly. (1, 2) The first Allergy Conference in Qatar aimed to highlight the burden of allergic diseases in Qatar and globally with a special focus on educating both the physicians and the community. Read More

World Allergy Organ J 2022 Jul 12;15(7):100664. Epub 2022 Jul 12.

Fraunhofer Institute for Translational Medicine and Pharmacology ITMP, Allergology and Immunology, Berlin, Germany.

Aim: To explore and compare the efficacy of standard (300 mg every 2 weeks) and extended (300 mg every 4 weeks) dosing regimens of lanadelumab for long-term prophylaxis of hereditary angioedema (HAE).

Methods: We conducted a retrospective chart review of all patients with HAE on lanadelumab, which identified a total of 9 patients: 5 females and 4 males. The median age of patients was 31 years (IQR 20. Read More

Expert Opin Emerg Drugs 2022 Jun 26;27(2):103-110. Epub 2022 Jul 26.

Department of Systems Medicine, University Hospital of Padua, Padua, Italy.

Introduction: Hereditary angioedema due to C1-inhibitor (C1-INH-HAE) is a rare disease characterized by unpredictable swelling attacks that may be life-threatening when affecting the upper airways. Understanding the pathophysiology of HAE and the mechanism of bradykinin-mediated angioedema allowed the development of new therapies for the treatment of HAE: clinical trials are ongoing to expand the number of drugs available for on-demand treatment and prophylaxis.

Areas Covered: Authors discuss the products that have been used to treat this disease for many years and present the most recently marketed products and those which are under development. Read More

Front Allergy 2022 7;3:868185. Epub 2022 Jul 7.

Department of Immunology and Histocompatibility, Faculty of Medicine, School of Health Sciences, University of Thessaly, Larissa, Greece.

Existing evidence indicates that modifier genes could change the phenotypic outcome of the causal variant and thus explain the expression variability of hereditary angioedema due to C1-inhibitor deficiency (C1-INH-HAE). To further examine this hypothesis, we investigated the presence or absence of 18 functional variants of genes encoding proteins involved in the metabolism and function of bradykinin, the main mediator of C1-INH-HAE attacks, in relation to three distinct phenotypic traits of patients with C1-INH-HAE, i.e. Read More

Pediatr Allergy Immunol 2022 07;33(7):e13825

Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, India.

J Clin Pharmacol 2022 Jul 24. Epub 2022 Jul 24.

Department of Pharmacy, University Hospital of Angers, Angers, France.

C1-inhibitor (C1INH) concentrates and the selective bradykinin B2 receptor antagonist icatibant are approved only for treating hereditary angioedema with C1INH deficiency. Yet, they are regularly prescribed off label in other types of bradykinin-mediated angioedema including angiotensin-converting enzyme inhibitor (ACEi)-related and undetermined angioedema. We conducted a retrospective chart review of inpatient prescriptions of C1INH concentrates and icatibant between 2016 and 2020 in the University Hospital of Angers. Read More

Allergy Asthma Proc 2022 Jul 22. Epub 2022 Jul 22.

Department of Internal Medicine and Clinical Immunology, Lille University, Inserm, Lille University Hospital, CREAK, U1286.

Background: Hereditary angioedema (HAE) is characterized by unpredictable and potentially life-threatening attacks ofcutaneous and submucosal swelling. Over the past decade, new agents, based on a better understanding of the underlying biologic mechanisms of HAE, have changed the face of long-term prophylaxis (LTP).

Objective: The objective was to describe current practices and unmet needs with regard to LTP for HAE in expert centers in France. Read More

Clin Exp Allergy 2022 Jul 21. Epub 2022 Jul 21.

Addenbrooke's Hospital, Cambridge University Hospitals NHS Foundation Trust, Cambridge, and University College London Hospitals, London, UK.

In patients with hereditary angioedema (HAE), bradykinin causes swelling episodes by activating bradykinin B receptors. Icatibant, a selective bradykinin B receptor antagonist, is approved for on-demand treatment of HAE attacks. The Icatibant Outcome Survey (IOS; NCT01034969) is an ongoing observational registry initiated in 2009 to monitor the effectiveness/safety of icatibant in routine clinical practice. Read More

Allergy Asthma Clin Immunol 2022 Jul 13;18(1):64. Epub 2022 Jul 13.

Yale University School of Medicine, New Haven, CT, USA.

Hereditary angioedema (HAE) is a rare, autosomal disorder that manifests with unpredictable episodes of severe swelling of the skin and mucous membranes. These attacks can be highly disfiguring and range in severity from mild to-in cases of airway swelling-life-threatening. Fluctuations in female sex hormones-such as the changes that occur during puberty, menses, contraceptive use, pregnancy, and menopause-can all affect the frequency and severity of HAE attacks. Read More

Arerugi 2022 ;71(5):402-403

Arerugi 2022 ;71(5):374-378

Department of Dermatology, Showa University School of Medicine.

Hereditas 2022 Jul 11;159(1):28. Epub 2022 Jul 11.

Department of Allergy & Clinical Immunology, National Clinical Research Center for Immunologic Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, #1 Shuaifuyuan, Wangfujing, Beijing, 100730, P.R. China.

Background: Hereditary angioedema (HAE) is a rare disease characterized by recurrent attacks of severe swellings of the skin and submucosa. More than 900 variants of the SERPING1 gene associated with HAE have been identified. However, only approximately 50 variants have been identified in the Chinese population. Read More

Allergy Asthma Proc 2022 Jul 12. Epub 2022 Jul 12.

Background: Hereditary angioedema (HAE) is a rare genetic disorder characterized by recurrent, localized episodes of edema. Current treatment guidelines highlight the importance of shared decision-making (SDM) during implementation of HAE management plans.

Objective: To determine what constitutes a successful SDM approach in HAE management. Read More

J Trop Pediatr 2022 06;68(4)

Department of Pediatric Allergy and Immunology, University of Health Sciences, Ankara Children's Hospital, Ankara City Hospital, Ankara 06800, Turkey.

Objective: Hereditary angioedema (HAE) is clinically characterized by recurrent attacks of angioedema. This study evaluated the clinical findings and examination results of patients admitted due to angioedema who then underwent a diagnostic test for HAE. The study aimed to assess the contribution of laboratory findings to the diagnostic process and to determine clinicians' level of awareness regarding the differential diagnosis of angioedema and the appropriate laboratory tests. Read More

Allergy Asthma Clin Immunol 2022 Jul 4;18(1):60. Epub 2022 Jul 4.

Department of Internal Medicine and Hematology, Semmelweis University, Szentkirályi u. 46, Budapest, 1088, Hungary.

Background: Hereditary angioedema (HAE) is a rare, life-threatening disease. The knowledge about the molecular pathogenesis of HAE has derived mainly from investigating blood samples. However, limited data are available on the role of the molecular mechanisms in the affected tissues during HAE attack. Read More

Allergol Int 2022 Jul 1. Epub 2022 Jul 1.

Department of Biomedical and Clinical Sciences, Luigi Sacco, University of Milan, IRCCS ICS Maugeri, Milano, Italy.

Monoclonal antibodies (mAbs) have been shown to be effective and generally safe across a continually expanding list of therapeutic areas. We describe the advantages and limitations of mAbs as a therapeutic option compared with small molecules. Specifically, we discuss a novel mAb in the treatment of hereditary angioedema (HAE), a rare and potentially life-threatening condition characterized by recurrent unpredictable swelling attacks. Read More

Front Allergy 2022 4;3:860355. Epub 2022 May 4.

Department of Internal Medicine and Haematology, Hungarian Angioedema Center of Reference and Excellence, Semmelweis University, Budapest, Hungary.

Background: Hereditary Angioedema with C1-inhibitor deficiency (C1-INH-HAE) is a rare disease characterized by recurrent subcutaneous and/or submucosal edematous (HAE) episodes, which may occur at any age. The mean age of the symptom onset is 10-12 years. Diagnostic protocols differ by age group and family history. Read More

Allergol Immunopathol (Madr) 2022 7;50(S Pt 1):1-6. Epub 2022 Apr 7.

Clinical Immunology, Centro Universitario FMABC, Santo André, Brazil;

Hereditary angioedema is a genetic disease with autosomal dominant inheritance and, in most cases, caused by C1 inhibitor deficiency. Patients present with recurrent edema affecting sub-cutaneous and mucus membranes with variable onset and severity. More than 50% of patients may become symptomatic before 10 years of age. Read More

Orphanet J Rare Dis 2022 06 16;17(1):232. Epub 2022 Jun 16.

Evidera, 615 2nd Ave Ste 500, Seattle, WA, 98104, USA.

Background: Hereditary angioedema (HAE) is a genetic disorder characterized by re-occurring swelling episodes called "attacks," usually in the limbs, face, airways, and intestinal tract. New prophylactic therapies have reduced the frequency of these attacks. This study describes results from a literature review and clinician interviews assessing patient HAE symptom experiences and timing, and then evaluates whether existing patient-reported outcome (PRO) tools adequately reflect this experience. Read More

ACS Med Chem Lett 2022 Jun 13;13(6):883-884. Epub 2022 May 13.

Smith, Gambrell & Russell LLP, 1105 West Peachtree Street NE, Suite 1000, Atlanta, Georgia 30309, United States.

J Allergy Clin Immunol Pract 2022 Jun 9. Epub 2022 Jun 9.

Department of Vascular Medicine, Amsterdam Cardiovascular Sciences, Amsterdam UMC, University of Amsterdam, Amsterdam, The Netherlands.