ReviewAdvances in therapy2023
Hereditary Angioedema: Diagnosis, Clinical Implications, and Pathophysiology.
Review in Advances in therapy, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 79 papers, 3 of them syntheses that pooled it.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
79 citing papers in PubMed, 3 syntheses or guidelines pooled it, 105 citations in OpenAlex.
- International Guideline on the Diagnosis and Management of Pediatric Patients With Hereditary Angioedema.Allergy · 2026Guideline
- Prevalence and Incidence of Hereditary Angioedema: A Systematic Literature Review.International archives of allergy and immunology · 2026Pooled it
- Epidemiology, economic, and humanistic burden of hereditary angioedema: a systematic review.Orphanet journal of rare diseases · 2024Pooled it
- Donidalorsen for Long-Term Prophylaxis of Hereditary Angioedema Attacks: Results from the OASISplus Open-Label Extension Cohort at Year 1.Journal of asthma and allergy · 2026Trial
- Exposure-Response Analysis of Donidalorsen for the Treatment of Hereditary Angioedema.Clinical and translational science · 2025Trial
- Beyond the Index Case: A Practical Framework for Cascade Family Screening in Hereditary Angioedema.Clinical reviews in allergy & immunology · 2026Review
- Prevalence and impact of sleep disorders in patients with hereditary angioedema: A multicenter cross-sectional study in Latin America.Sleep & breathing = Schlaf & Atmung · 2026Article
- The swell within: a rare case of type 1 hereditary angioedema.Proceedings (Baylor University. Medical Center) · 2026Article
- Beyond Swelling: Clinical Insights into the Diagnosis and Management of Hereditary Angioedema.International journal of molecular sciences · 2026Review
- Transient C1-Inhibitor Functional Abnormalities During Acute Idiopathic Pancreatitis: A Diagnostic Challenge in Hereditary Angioedema Evaluation.Digestive diseases and sciences · 2026Article
- Deep Dive into Evolution: How Cetaceans Adapt Their Anticoagulant Genes for Underwater Survival.Journal of molecular evolution · 2026Article
- Long-term prophylaxis in hereditary angioedema: Real-world treatment patterns and healthcare resource utilization.The World Allergy Organization journal · 2026Article
- The peri-operative management of patients with hereditary angioedema requiring dental treatment: a case series.British dental journal · 2026Article
- Limited access to on-demand treatment in hereditary angioedema is linked to delayed treatment and impaired patient-reported outcomes: A multinational real-world study.The journal of allergy and clinical immunology. Global · 2026Article
- A novel SERPING1 splice-site variant (c.1029 + 2T > A) causing hereditary angioedema type I: functional characterization and clinical analysis.Orphanet journal of rare diseases · 2026Article
- Real-world treatment patterns and clinical burden of patients with hereditary angioedema treated with long-term prophylaxis.Orphanet journal of rare diseases · 2026Article
- A year in pharmacology: new drugs approved by the US food and drug administration in 2025.Naunyn-Schmiedeberg's archives of pharmacology · 2026Review
- The 2025 WAO Guidelines for the classification, diagnosis, and treatment of hereditary angioedema, with consideration of worldwide disparities.The World Allergy Organization journal · 2026Article
- Patient Preferences for Long-Term Prophylactic Treatment in Hereditary Angioedema: A Discrete-Choice Experiment.The patient · 2026Article
- Complement factor H supplementation rather than complete C3 knockout provides therapeutic benefits in IgA nephropathy.Molecular biomedicine · 2026Article
19 more citing papers are in PubMed but not listed here.
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
10 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Hereditary angioedema (HAE) is an autosomal dominant disorder caused by a mutation in the C1 esterase inhibitor gene. HAE affects 1/50,000 people worldwide. Three main types of HAE exist: type I, type II, and type III. Type I is characterized by a deficiency in C1-INH. C1-INH is important in the coagulation complement, contact systems, and fibrinolysis. Most HAE cases are type I. Type I and II HAE result from a mutation in the SERPING1 gene, which encodes C1-INH. Formally known as type III HAE is typically an estrogen-dependent or hereditary angioedema with normal C1-INH activity. Current guidelines now recommend subdividing hereditary angioedema with normal C1 esterase inhibitor gene (HAE-nl-C1-INH formerly known as HAE type III) based on underlying mutations such as in kininogen-1 (HAE-KNG1), plasminogen gene (PLG-HAE), myoferlin gene mutation (MYOF-HAE), heparan sulfate-glucosamine 3-sulfotransferase 6 (HS3ST6), mutation in Hageman factor (factor XII), and in angiopoietin-1 (HAE-ANGPT-1). The clinical presentation of HAE varies between patients, but it usually presents with nonpitting angioedema and occasionally abdominal pain. Young children are typically asymptomatic. Those affected by HAE usually present with symptoms in their early 20s. Symptoms can arise as a result of stress, infection, or trauma. Laboratory testing shows abnormal levels of C1-INH and high levels of bradykinin. C4 and D-dimer levels can also be monitored if an acute HAE attack is suspected. Acute treatment of HAE can include IV infusions of C1-INH, receptor antagonists, and kallikrein inhibitors. Short- and long-term prophylaxis can also be administered to patients with HAE. First-line therapies for long-term prophylaxis also include IV infusion of C1-INH. This review aims to thoroughly understand HAE, its clinical presentation, and how to treat it.
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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.