Research & Literature

Safety and efficacy of C1 esterase inhibitor for acute attacks in children with hereditary angioedema.

Lumry W, Soteres D, Gower R, et al.

Pediatric allergy and immunology : official publication of the European Society of Pediatric Allergy and Immunology 2015; (26(7)):674-80 doi:10.1111/pai.12444.

Hereditary Angioedema and Gastrointestinal Complications: An Extensive Review of the Literature.

Patel N, Suarez LD, Kapur S, Bielory L

Case reports in immunology 2015; (2015()):925861 doi:10.1155/2015/925861.

Bradykinin: Inflammatory Product of the Coagulation System.

Hofman Z, de Maat S, Hack CE, Maas C

Clinical reviews in allergy & immunology 2016; (51(2)):152-61 doi:10.1007/s12016-016-8540-0.

Diagnosis and screening of patients with hereditary angioedema in primary care.

Henao MP, Kraschnewski JL, Kelbel T, Craig TJ

Therapeutics and clinical risk management 2016; (12()):701-11 doi:10.2147/TCRM.S86293.

HAE Pathophysiology and Underlying Mechanisms.

Zuraw BL, Christiansen SC

Clinical reviews in allergy & immunology 2016; (51(2)):216-29 doi:10.1007/s12016-016-8561-8.

Emergency Department Management of Hereditary Angioedema Attacks: Patient Perspectives.

Otani IM, Christiansen SC, Busse P, et al.

The journal of allergy and clinical immunology. In practice 2017; (5(1)):128-134.e4 doi:10.1016/j.jaip.2016.06.029.

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.

Squeglia V, Barbarino A, Bova M, et al.

Orphanet journal of rare diseases 2016; (11(1)):133 doi:10.1186/s13023-016-0518-8.

Misdiagnosis trends in patients with hereditary angioedema from the real-world clinical setting.

Zanichelli A, Longhurst HJ, Maurer M, et al.

Annals of allergy, asthma & immunology : official publication of the American College of Allergy, Asthma, & Immunology 2016; (117(4)):394-398 doi:10.1016/j.anai.2016.08.014.

Comparing acquired angioedema with hereditary angioedema (types I/II): findings from the Icatibant Outcome Survey.

Longhurst HJ, Zanichelli A, Caballero T, et al.

Clinical and experimental immunology 2017; (188(1)):148-153 doi:10.1111/cei.12910.

Breakthrough attacks in patients with hereditary angioedema receiving long-term prophylaxis are responsive to icatibant: findings from the Icatibant Outcome Survey.

Aberer W, Maurer M, Bouillet L, et al.

Allergy, asthma, and clinical immunology : official journal of the Canadian Society of Allergy and Clinical Immunology 2017; (13()):31 doi:10.1186/s13223-017-0203-z.

The relationship between anxiety and quality of life in children with hereditary angioedema.

Kessel A, Farkas H, Kivity S, et al.

Pediatric allergy and immunology : official publication of the European Society of Pediatric Allergy and Immunology 2017; (28(7)):692-698 doi:10.1111/pai.12758.

Acute Genital Swelling Heralding C1 Esterase Inhibitor Deficiency in a Child.

Murad A, Leahy TR, Lenane P

Pediatric dermatology 2017; (34(5)):e277-e278 doi:10.1111/pde.13205.

Angioedema.

Hahn J, Hoffmann TK, Bock B, et al.

Deutsches Arzteblatt international 2017; (114(29-30)):489-496.

Health-Related Quality of Life with Subcutaneous C1-Inhibitor for Prevention of Attacks of Hereditary Angioedema.

Lumry WR, Craig T, Zuraw B, et al.

The journal of allergy and clinical immunology. In practice 2018; (6(5)):1733-1741.e3 doi:10.1016/j.jaip.2017.12.039.

Refractory Abdominal Pain in a Patient with Chronic Lymphocytic Leukemia: Be Wary of Acquired Angioedema due to C1 Esterase Inhibitor Deficiency.

Abdulkareem A, D'Souza RS, Mundorff J, et al.

Case reports in hematology 2018; (2018()):7809535 doi:10.1155/2018/7809535.

Management of patients with hereditary angioedema in Germany: comparison with other countries in the Icatibant Outcome Survey.

Maurer M, Bork K, Martinez-Saguer I, et al.

Journal of the European Academy of Dermatology and Venereology : JEADV 2019; (33(1)):163-169 doi:10.1111/jdv.15232.

Improvement in diagnostic delays over time in patients with hereditary angioedema: findings from the Icatibant Outcome Survey.

Zanichelli A, Magerl M, Longhurst HJ, et al.

Clinical and translational allergy 2018; (8()):42 doi:10.1186/s13601-018-0229-4.

A review of kallikrein inhibitor lanadelumab in hereditary angioedema.

Hwang G, Johri A, Ng S, Craig T

Immunotherapy 2019; (11(11)):937-944 doi:10.2217/imt-2018-0197.

Hereditary angioedema: an update on causes, manifestations and treatment.

Longhurst HJ, Bork K

British journal of hospital medicine (London, England : 2005) 2019; (80(7)):391-398 doi:10.12968/hmed.2019.80.7.391.

Lanadelumab to treat hereditary angioedema.

Wedi B

Drugs of today (Barcelona, Spain : 1998) 2019; (55(7)):439-448 doi:10.1358/dot.2019.55.7.2985293.

Hereditary and acquired angioedema.

Patel G, Pongracic JA

Allergy and asthma proceedings 2019; (40(6)):441-445 doi:10.2500/aap.2019.40.4267.

Hereditary angioedema in a single family with specific mutations in both plasminogen and SERPING1 genes.

Bork K, Zibat A, Ferrari DM, et al.

Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG 2020; (18(3)):215-223 doi:10.1111/ddg.14036.

[Hereditary angioedema due to C1-esterase inhibitor deficiency : novel approaches].

Stehlin F, Ribi C

Revue medicale suisse 2020; (16(689)):675-678.

Hereditary angioedema: Pathophysiology (HAE type I, HAE type II, and HAE nC1-INH).

Wedner HJ

Allergy and asthma proceedings 2020; (41(Suppl 1)):S14-S17 doi:10.2500/aap.2020.41.200081.

Current medical management of hereditary angioedema: Follow-up survey of US physicians.

Riedl MA, Banerji A, Gower R

Annals of allergy, asthma & immunology : official publication of the American College of Allergy, Asthma, & Immunology 2021; (126(3)):264-272 doi:10.1016/j.anai.2020.10.009.

Oral berotralstat for the prophylaxis of hereditary angioedema attacks in patients in Japan: A phase 3 randomized trial.

Ohsawa I, Honda D, Suzuki Y, et al.

Allergy 2021; (76(6)):1789-1799 doi:10.1111/all.14670.

Impact of lanadelumab on health-related quality of life in patients with hereditary angioedema in the HELP study.

Lumry WR, Weller K, Magerl M, et al.

Allergy 2021; (76(4)):1188-1198 doi:10.1111/all.14680.

Discovery of a Bradykinin B2 Partial Agonist Profile of Raloxifene in a Drug Repurposing Campaign.

Gomez-Gutierrez P, Perez JJ

International journal of molecular sciences 2020; (22(1)) doi:10.3390/ijms22010257.

Psychology and hereditary angioedema: A systematic review.

Savarese L, Mormile I, Bova M, et al.

Allergy and asthma proceedings 2021; (42(1)):e1-e7 doi:10.2500/aap.2021.42.200073.

A new oral kallikrein inhibitor for long-term prophylaxis of hereditary angioedema.

Doña I, Torres MJ

Allergy 2021; (76(6)):1619-1620 doi:10.1111/all.14798.

Therapeutic management of hereditary angioedema: past, present, and future.

Valerieva A, Nedeva D, Yordanova V, et al.

Balkan medical journal 2021; (38(2)):89-103 doi:10.5152/balkanmedj.2021.21094.

Hereditary angioedema: how to approach it at the emergency department?

Serpa FS, Mansour E, Aun MV, et al.

Einstein (Sao Paulo, Brazil) 2021; (19()):eRW5498 doi:10.31744/einstein_journal/2021RW5498.

Transition to lanadelumab-flyo from three medications for a hereditary angioedema patient with a variant in the SYTL2 gene: A case report.

Burns S, Lewis E

Clinical case reports 2021; (9(4)):2438-2441 doi:10.1002/ccr3.4060.

The importance of recognizing and managing a rare form of angioedema: hereditary angioedema due to C1-inhibitor deficiency.

Jacobs J, Neeno T

Postgraduate medicine 2021; (133(6)):639-650 doi:10.1080/00325481.2021.1905364.

Consensus on treatment goals in hereditary angioedema: A global Delphi initiative.

Maurer M, Aygören-Pürsün E, Banerji A, et al.

The Journal of allergy and clinical immunology 2021; (148(6)):1526-1532 doi:10.1016/j.jaci.2021.05.016.

Intestinal angioedema caused by an acquired C1 esterase inhibitor deficiency associated with underlying splenic marginal zone lymphoma.

Thongtan T, Deb A, Bedanie G, et al.

Proceedings (Baylor University. Medical Center) 2021; (34(4)):519-520 doi:10.1080/08998280.2021.1885277.

Berotralstat (BCX7353): Structure-Guided Design of a Potent, Selective, and Oral Plasma Kallikrein Inhibitor to Prevent Attacks of Hereditary Angioedema (HAE).

Kotian PL, Wu M, Vadlakonda S, et al.

Journal of medicinal chemistry 2021; (64(17)):12453-12468 doi:10.1021/acs.jmedchem.1c00511.

Recognition and Management of Hereditary Angioedema: Best Practices for Dermatologists.

Manning ME

Dermatology and therapy 2021; (11(5)):1829-1838 doi:10.1007/s13555-021-00593-x.

Variability of disease activity in patients with hereditary angioedema type 1/2: longitudinal data from the Icatibant Outcome Survey.

Maurer M, Caballero T, Aberer W, et al.

Journal of the European Academy of Dermatology and Venereology : JEADV 2021; (35(12)):2421-2430 doi:10.1111/jdv.17654.

Medical algorithm: Management of C1 inhibitor hereditary angioedema.

Caballero T, Cabañas R, Pedrosa M

Allergy 2022; (77(3)):1060-1063 doi:10.1111/all.15115.

The role of C1 inhibitor and complement as acute phase reactants: are we missing the diagnosis of hereditary angioedema?

Stepaniuk P, Bosonea AM, Pourshahnazari P, et al.

Allergy, asthma, and clinical immunology : official journal of the Canadian Society of Allergy and Clinical Immunology 2021; (17(1)):103 doi:10.1186/s13223-021-00607-5.

Malignancy and immune disorders in patients with hereditary angioedema.

Stepaniuk P, Kanani A

Allergy, asthma, and clinical immunology : official journal of the Canadian Society of Allergy and Clinical Immunology 2021; (17(1)):134 doi:10.1186/s13223-021-00621-7.

Emerging drugs for the treatment of hereditary angioedema due to C1-inhibitor deficiency.

Zanichelli A, Montinaro V, Triggiani M, et al.

Expert opinion on emerging drugs 2022; (27(2)):103-110 doi:10.1080/14728214.2022.2105834.

SERPING1 Variants and C1-INH Biological Function: A Close Relationship With C1-INH-HAE.

Drouet C, López-Lera A, Ghannam A, et al.

Frontiers in allergy 2022; (3()):835503 doi:10.3389/falgy.2022.835503.

Systemic lupus erythematosus with various clinical manifestations in a patient with hereditary angioedema: a case report.

Ushio Y, Wakiya R, Kameda T, et al.

Allergy, asthma, and clinical immunology : official journal of the Canadian Society of Allergy and Clinical Immunology 2022; (18(1)):84 doi:10.1186/s13223-022-00725-8.

Long-term Prophylaxis with Androgens in the management of Hereditary Angioedema (HAE) in emerging countries.

Guo Y, Zhang H, Lai H, et al.

Orphanet journal of rare diseases 2022; (17(1)):399 doi:10.1186/s13023-022-02536-x.

Hereditary Angioedema: The Clinical Picture of Excessive Contact Activation.

Petersen RS, Fijen LM, Levi M, Cohn DM

Seminars in thrombosis and hemostasis 2024; (50(7)):978-988 doi:10.1055/s-0042-1758820.

Hereditary angioedema: 24 years of experience in a Portuguese reference center.

Varandas C, Esteves Caldeira L, Silva SL, et al.

European annals of allergy and clinical immunology 2024; (56(5)):210-218 doi:10.23822/EurAnnACI.1764-1489.278.

National survey on clinical and genetic characteristics of patients with hereditary angioedema in Latvia.

Kanepa A, Nartisa I, Rots D, et al.

Allergy, asthma, and clinical immunology : official journal of the Canadian Society of Allergy and Clinical Immunology 2023; (19(1)):28 doi:10.1186/s13223-023-00783-6.

Bradykinin Metabolism and Drug-Induced Angioedema.

Smolinska S, Antolín-Amérigo D, Popescu FD

International journal of molecular sciences 2023; (24(14)) doi:10.3390/ijms241411649.

Pathogenic variant in SERPING1 gene causing autosomal dominant hereditary angioedema in early childhood.

Kron K, Joshi S

BMJ case reports 2023; (16(11)) doi:10.1136/bcr-2023-257212.

Screening for type II hereditary angioedema-the "poor man's c1-inhibitor function".

Jindal AK, Chiang V, Barman P, et al.

The journal of allergy and clinical immunology. Global 2024; (3(1)):100179 doi:10.1016/j.jacig.2023.100179.

Recessive SERPING1 Variant Leads to Kinin-Kallikrein System Control Failure in a Consanguineous Brazilian Family with Hereditary Angioedema.

Maia LSM, Burger B, Ghannam A, et al.

Journal of clinical medicine 2023; (12(23)) doi:10.3390/jcm12237299.

The current situation of hereditary angioedema patients in Germany: results of an online survey.

Magerl M, Martinez-Saguer I, Schauf L, et al.

Frontiers in medicine 2023; (10()):1274397 doi:10.3389/fmed.2023.1274397.

The future of therapeutic options for hereditary angioedema.

Smith TD, Riedl MA

Annals of allergy, asthma & immunology : official publication of the American College of Allergy, Asthma, & Immunology 2024; (133(4)):380-390 doi:10.1016/j.anai.2024.04.029.

Garadacimab for hereditary angioedema attack prevention: long-term efficacy, quality of life, and safety data from a phase 2, randomised, open-label extension study.

Craig TJ, Levy DS, Reshef A, et al.

The Lancet. Haematology 2024; (11(6)):e436-e447 doi:10.1016/S2352-3026(24)00081-4.

Current and Emerging Therapeutics in Hereditary Angioedema.

Do T, Riedl MA

Immunology and allergy clinics of North America 2024; (44(3)):561-576 doi:10.1016/j.iac.2024.03.009.

Case Report: Early presentation of hereditary angioedema symptoms in a 2-year-old boy.

Staikuniene-Kozonis J, Staikunaite J, Gasiuniene E, Sematonyte J

Frontiers in pediatrics 2024; (12()):1408110 doi:10.3389/fped.2024.1408110.

Long-term prevention of hereditary angioedema attacks with lanadelumab in adolescents.

Craig T, Tachdjian R, Bernstein JA, et al.

Annals of allergy, asthma & immunology : official publication of the American College of Allergy, Asthma, & Immunology 2024; (133(6)):712-719.e1 doi:10.1016/j.anai.2024.08.001.

Hereditary Angioedema in Pregnancy: A Case Report and Review of Obstetric Anesthesia Management.

de Valle MK, Stevenson C, Adkison M, et al.

A&A practice 2024; (18(9)):e01833 doi:10.1213/XAA.0000000000001833.

Hereditary angioedema (HAE) in children and adolescents: New treatment options.

Fasshauer M, Wedi B

Allergologie select 2024; (8()):336-345 doi:10.5414/ALX02532E.

Analysis of prodromal symptoms and need for short-term prophylaxis in angioedema patients under long-term prophylaxis.

Lochbaum R, Hoffmann TK, Greve J, Hahn J

Orphanet journal of rare diseases 2025; (20(1)):47 doi:10.1186/s13023-025-03562-1.

Impact of Dental Procedures on Hereditary Angioedema Attacks: An Exploratory Observational Study.

Nadasan V, Kiss KO, Borka-Balás R, Bara NA

Oral health & preventive dentistry 2025; (23()):173-182 doi:10.3290/j.ohpd.c_1907.

Expert Consensus on the Diagnosis and Treatment of Hereditary Angioedema in China (2024 Edition).

Xu Y, Liu S, Wang X, et al.

International archives of allergy and immunology 2026; (187(1)):61-73 doi:10.1159/000545808.

Assessment of potential drug-drug interactions in patients with hereditary angioedema from the ITACA cohort: simulations from a real-life dataset considering danazol versus berotralstat.

Zanichelli A, Cattaneo D, Gidaro A, et al.

Frontiers in pharmacology 2025; (16()):1550133 doi:10.3389/fphar.2025.1550133.

Can depression and anxiety be predicted in hereditary angioedema? A comprehensive assessment.

Akten HS, Dilek E, Orman M, Mete Gokmen EN

Allergy and asthma proceedings 2025; (46(3)):e71-e81 doi:10.2500/aap.2025.46.250017.

A Two-Decade-Delayed Diagnosis of Hereditary Angioedema: A Case Report.

Shrestha S, Yadav AK, Khadka D, et al.

Cureus 2025; (17(5)):e85019 doi:10.7759/cureus.85019.

Treatment Patterns and Characteristics of Patients with Hereditary Angioedema Treated with Lanadelumab: A US Retrospective Chart Review.

Watt M, Chang R, Yu LH, et al.

Drugs - real world outcomes 2025; (12(3)):351-365 doi:10.1007/s40801-025-00505-x.

Impact of providing education on the recognition and differential diagnosis of angioedema among emergency department physicians.

Unal D, Demir S, Işik SR, et al.

The World Allergy Organization journal 2025; (18(8)):101101 doi:10.1016/j.waojou.2025.101101.

Quality of life and burden of disease in patients with hereditary angioedema and their caregivers.

Levy DS, Nagase FI, Cheung A, et al.

Allergy and asthma proceedings 2025; (46(5)):398-405 doi:10.2500/aap.2025.46.250048.

Real-World Evidence on the Management of Hereditary Angioedema With Normal C1 Inhibitor.

Lobão NTM, Bardou MLD, Vila SYC, et al.

The journal of allergy and clinical immunology. In practice 2026; (14(1)):233-242.e2 doi:10.1016/j.jaip.2025.10.001.

C1 Esterase Inhibitor Deficiency in an Indian Female: A Rare Case Report and a Review of Literature with Treatment Update.

Singh J, Kumar N, Kumar K, et al.

Current drug targets 2026; (27(3)):211-216 doi:10.2174/0113894501411809251001113142.

Delphi Consensus on Attenuated Androgen Use for Long-Term Prophylaxis in Hereditary Angioedema: AURA Project.

Dias de Castro E, Cardoso LM, Jácome de Castro J, Ferreira MB

Clinical and translational allergy 2025; (15(11)):e70116 doi:10.1002/clt2.70116.

Hereditary Angioedema Prophylaxis Therapy: Berotralstat and Lanadelumab Safety Profile.

Donadoni M, La Cava L, Bizzi E, et al.

Medicina (Kaunas, Lithuania) 2025; (61(11)) doi:10.3390/medicina61111897.

Patient Preferences for Long-Term Prophylactic Treatment in Hereditary Angioedema: A Discrete-Choice Experiment.

Villa KF, Mansfield C, Yarlas A, et al.

The patient 2026; (19(3)):481-495 doi:10.1007/s40271-025-00798-8.

A Treat-to-Target approach in hereditary angioedema: expert consensus from a European committee.

Cancian M, Caballero T, Boccon-Gibod I, et al.

Frontiers in immunology 2026; (17()):1773279 doi:10.3389/fimmu.2026.1773279.