ArticleThe World Allergy Organization journal2022
The prevalence of hereditary angioedema in a Chinese cohort with decreased complement 4 levels.
Article in The World Allergy Organization journal, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 7 papers, 1 of them a synthesis that pooled it.
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Who cites it
7 citing papers in PubMed, 1 synthesis or guideline pooled it, 11 citations in OpenAlex.
- Epidemiology, economic, and humanistic burden of hereditary angioedema: a systematic review.Orphanet journal of rare diseases · 2024Pooled it
- Lanadelumab safety, efficacy, and pharmacokinetics in patients aged ≥12 with hereditary angioedema in China: an open-label, multicenter study.Frontiers in immunology · 2026Trial
- Advances in hereditary angioedema in the modern treatment era in China: a focus on diagnosis, treatment, and prognosis.Orphanet journal of rare diseases · 2026Review
- Hereditary Angioedema (HAE) in China: Advancing Awareness, Access, Advocacy and Alliances From the Greater Bay Area to the Global HAE Community.Clinical and experimental allergy : journal of the British Society for Allergy and Clinical Immunology · 2025Review
- Proactive identification of rare diseases: lessons from hereditary angioedema diagnosis using electronic medical records.Orphanet journal of rare diseases · 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
- Case Report: Two cases of hereditary angioedema in a Chinese family.Frontiers in allergy · 2025Article
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Authors and funding
10 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
objectiveHereditary angioedema (HAE) is a rare, life-threatening autosomal dominant disorder. We aimed to investigate the prevalence of HAE in a Chinese population with a decreased Complement 4 (C4) level.
methodsAll the patients present in Tongji Hospital with C4 below lower normal range were included from January 2019 to June 2020. The individual data were extracted from the database and categorized by diagnosis. Patients suspected of HAE were further evaluated by C1 inhibitor level and function test to confirm the HAE diagnosis.
resultsA total of 8226 patients were enrolled in our study, among whom 18 had symptoms similar to HAE and received C1 inhibitor level and function tests. Two (1 male and 1 female) of the 18 patients were identified as HAE patients. This means the prevalence of HAE was 2.43/10 000 among the C4-decreased population and 10.1/10 000 in the C4-decreased population with etiology undetermined. The 2 HAE patients had experienced skin and oropharynx edema attack and received tracheotomy. The female patient had a family history. Laboratory tests showed significant decrease of C4 and C1 inhibitor levels in the 2 patients, both of whom were diagnosed as type 1 HAE.
conclusionThe prevalence of HAE is low in C4-decreased patients. In a large cohort, C4 level can serve as a practical indicator to screen the HAE patients, but further testing of C1 inhibitor activity and levels is needed to confirm the diagnosis of HAE.
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