{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,5]],"date-time":"2026-09-05T15:57:32Z","timestamp":1788623852343,"version":"build-2803163510"},"reference-count":42,"publisher":"Oceanside Publications Inc.","issue":"2","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["allergy asthma proc"],"published-print":{"date-parts":[[2026,3,1]]},"abstract":"<jats:p>\n                    <jats:bold>Background:<\/jats:bold>\n                    Hereditary angioedema with C1INH deficiency (HAE-C1INH) is a rare, debilitating genetic disorder characterized by recurrent, unpredictable attacks. Although treatments exist, patients with HAE still alter their lives to avoid triggers and experience substantial\n physical, psychosocial, and financial burdens.\n                    <jats:bold>Objective:<\/jats:bold>\n                    To estimate the burden that HAE-C1INH patients experience despite currently approved therapies, aiming to identify unmet needs related to HAE, its therapies and the ability to achieve normalization of life.\n                    <jats:bold>Methods:<\/jats:bold>\n                    A web-based survey was conducted from March to April 2025 among 100 US adults with HAE-C1INH currently receiving long-term prophylaxis and\/or on-demand therapies. Responses captured attack frequency, the impact of living with HAE, avoidance of attack triggers, and the patients\u2019\n unmet needs. Descriptive statistical analysis was conducted.\n                    <jats:bold>Results:<\/jats:bold>\n                    Even with treatment, 80% of respondents reported \u22651 HAE attack in the past year and 61% thought about HAE at least weekly. Mental health was the aspect that respondents felt was most impacted by HAE\n (54% of respondents), and 73% reported taking \u22652 measures to avoid attack triggers. Several concerns impacted the ability to reach normalization; lifetime use of medication was the most commonly reported concern (68% of respondents). The greatest unmet needs associated with long-term prophylaxis\n were cost- and access-related.\n                    <jats:bold>Conclusion:<\/jats:bold>\n                    Substantial unmet needs related to disease control and achieving normalization remain for patients with HAE-C1INH, despite existing treatments. This study re-enforces the need to not only assess the frequency and severity of attacks,\n but also the psychosocial, mental, logistical, and financial burden of lifelong management of HAE-C1INH in clinical practice.\n                  <\/jats:p>","DOI":"10.2500\/aap.2026.47.260004","type":"journal-article","created":{"date-parts":[[2026,2,17]],"date-time":"2026-02-17T01:05:16Z","timestamp":1771290316000},"page":"92-101","source":"Crossref","is-referenced-by-count":4,"title":["Rethinking the management of hereditary angioedema"],"prefix":"10.2500","volume":"47","author":[{"given":"Paula","family":"Busse","sequence":"first","affiliation":[{"name":"From the Division of Clinical Immunology, Department of Medicine, Icahn School of Medicine at Mount Sinai, New York, New York;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kim","family":"Wilson","sequence":"additional","affiliation":[{"name":"Patient Partner, Expert by Experience;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Henriette","family":"Farkas","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine and Haematology, Hungarian Angioedema Center of Reference and Excellence Semmelweis University, Budapest, Hungary;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shanna","family":"Fishel","sequence":"additional","affiliation":[{"name":"Patient Partner, Expert by Experience;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amod","family":"Athavale","sequence":"additional","affiliation":[{"name":"Trinity Life Sciences, Waltham, Massachusetts; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Abigail","family":"Silber","sequence":"additional","affiliation":[{"name":"Trinity Life Sciences, Waltham, Massachusetts; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ellie","family":"Goldman","sequence":"additional","affiliation":[{"name":"Trinity Life Sciences, Waltham, Massachusetts; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Catherine","family":"Miller","sequence":"additional","affiliation":[{"name":"Intellia Therapeutics, Cambridge, Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shruti","family":"Nambiar","sequence":"additional","affiliation":[{"name":"Intellia Therapeutics, Cambridge, Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1355","reference":[{"key":"B1","article-title":"Hereditary Angioedema","author":"Abdulkarim","year":"2023"},{"key":"B2","doi-asserted-by":"crossref","first-page":"398","DOI":"10.1016\/j.jaci.2024.03.024","article-title":"Definition, acronyms, nomenclature, and classification of angioedema (DANCE): AAAAI, ACAAI, ACARE, and APAAACI DANCE consensus","volume":"154","author":"Reshef","year":"2024","journal-title":"J Allergy Clin Immunol"},{"key":"B3","doi-asserted-by":"crossref","first-page":"1961","DOI":"10.1111\/all.15214","article-title":"The international WAO\/EAACI guideline for the management of hereditary angioedema-the 2021 revision and update","volume":"77","author":"Maurer","year":"2022","journal-title":"Allergy"},{"key":"B4","doi-asserted-by":"crossref","first-page":"835503","DOI":"10.3389\/falgy.2022.835503","article-title":"SERPING1 variants and C1-INH biological function: a close relationship with C1-INH-HAE","volume":"3","author":"Drouet","year":"2022","journal-title":"Front Allergy"},{"key":"B5","doi-asserted-by":"crossref","first-page":"1136","DOI":"10.1056\/NEJMra1808012","article-title":"Hereditary angioedema.","volume":"382","author":"Busse","year":"2020","journal-title":"N Engl J Med"},{"key":"B6","doi-asserted-by":"crossref","first-page":"1027","DOI":"10.1056\/NEJMcp0803977","article-title":"Clinical practice. 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