ReviewTherapeutics and clinical risk management2016
Diagnosis and screening of patients with hereditary angioedema in primary care.
Review in Therapeutics and clinical risk management, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 31 papers, 1 of them a synthesis that pooled it.
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The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
31 citing papers in PubMed, 1 synthesis or guideline pooled it, 82 citations in OpenAlex.
- Interventions for the long-term prevention of hereditary angioedema attacks.The Cochrane database of systematic reviews · 2022Pooled it
- Effective long-term prophylaxis with lanadelumab in adolescents with hereditary angioedema: EMPOWER/ENABLE.Pediatric allergy and immunology : official publication of the European Society of Pediatric Allergy and Immunology · 2025Trial
- Beyond the Index Case: A Practical Framework for Cascade Family Screening in Hereditary Angioedema.Clinical reviews in allergy & immunology · 2026Review
- Family Tree Mapping of Genetic and Phenotypic Codes of Hereditary Angioedema.Medicina (Kaunas, Lithuania) · 2026Article
- Patient Preferences for Long-Term Prophylactic Treatment in Hereditary Angioedema: A Discrete-Choice Experiment.The patient · 2026Article
- Urgent diagnosis of hereditary angioedema in the ICU and ED: the critical role of rapid complement testing - a case report.International journal of emergency medicine · 2025Article
- Therapeutic Advances in Hereditary Angioedema: A Focus on Present and Future Options.Advances in therapy · 2025Review
- Proactive identification of rare diseases: lessons from hereditary angioedema diagnosis using electronic medical records.Orphanet journal of rare diseases · 2025Article
- Reductions in medical visits and hospitalizations following berotralstat initiation in patients with hereditary angioedema in the United States.Journal of managed care & specialty pharmacy · 2025Article
- Clinical validity of dried blood spot assay for the measurement of functional C1 inhibitor in angioedema due to C1 inhibitor deficiency.The journal of allergy and clinical immunology. Global · 2025Article
- Attack frequency and associated factors in hereditary angioedema patients: a single-centre experience.Postepy dermatologii i alergologii · 2025Article
- Analysis of disease burden in patients with hereditary angioedema from Japan by patient-reported outcomes.The Journal of dermatology · 2025Article
- Unveiling the Complexities of Hereditary Angioedema.Biomolecules · 2024Review
- Clinical Experience with Berotralstat in Patients with Hereditary Angioedema with Normal C1-Esterase Inhibitor: A Commented Case Series.Journal of asthma and allergy · 2024Article
- Could it be hereditary angioedema?-Perspectives from different medical specialties.Clinical and translational allergy · 2023Review
- The multifactorial impact of receiving a hereditary angioedema diagnosis.The World Allergy Organization journal · 2023Review
- 3D facial analysis for rare disease diagnosis and treatment monitoring: Proof-Of-Concept plan for hereditary angioedema.PLOS digital health · 2023Article
- Hereditary Angioedema: Diagnosis, Clinical Implications, and Pathophysiology.Advances in therapy · 2023Review
- Managing Diagnosis, Treatment, and Burden of Disease in Hereditary Angioedema Patients with Normal C1-Esterase Inhibitor.Journal of asthma and allergy · 2023Review
- Abdominal and pelvic imaging in the diagnosis of acute abdominal attacks in patients with hereditary angioedema due to C1-inhibitor deficiency.Postepy dermatologii i alergologii · 2022Article
Corrections and comments
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Authors and funding
4 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Hereditary angioedema (HAE) is a rare autosomal dominant disease that commonly manifests with episodes of cutaneous or submucosal angioedema and intense abdominal pain. The condition usually presents due to a deficiency of C1 esterase inhibitor (C1-INH) that leads to the overproduction of bradykinin, causing an abrupt increase in vascular permeability. A less-understood and less-common form of the disease presents with normal C1-INH levels. Symptoms of angioedema may be confused initially with mast cell-mediated angioedema, such as allergic reactions, and may perplex physicians when epinephrine, antihistamine, or glucocorticoid therapies do not provide relief. Similarly, abdominal attacks may lead to unnecessary surgeries or opiate dependence. All affected individuals are at risk for a life-threatening episode of laryngeal angioedema, which continues to be a source of fatalities due to asphyxiation. Unfortunately, the diagnosis is delayed on average by almost a decade due to a misunderstanding of symptoms and general lack of awareness of the disease. Once physicians suspect HAE, however, diagnostic methods are reliable and available at most laboratories, and include testing for C4, C1-INH protein, and C1-INH functional levels. In patients with HAE, management consists of acute treatment of an attack as well as possible short- or long-term prophylaxis. Plasma-derived C1-INH, ecallantide, icatibant, and recombinant human C1-INH are new treatments that have been shown to be safe and effective in the treatment of HAE attacks. The current understanding of HAE has greatly improved in recent decades, leading to growing awareness, new treatments, improved management strategies, and better outcomes for patients.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.