Evidence map›Paper›PMID 40551525›Full record

ArticleClinical and translational allergy2025

C1 inhibitor deficient hereditary angioedema is related to endothelial dysfunction in young adult and middle-aged patients.

Gokce Gul Atay Sensoy, Derya Baykız, İlkim Deniz Toprak, Deniz Eyice Karabacak, Erdem Bektas, Derya Unal, Aslı Gelincik, Semra Demir

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Article in Clinical and translational allergy, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 4 papers.

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4citing papers in PubMed
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1 · What the graph read from it

What it found

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3 · Its place in the literature

Who cites it

4 citing papers in PubMed.

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4 · The record

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5 · Who and what money

Authors and funding

8 authors.

Gokce Gul Atay SensoyDepartment of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey.ORCID https://orcid.org/0000-0002-0371-9972
Derya BaykızDepartment of Cardiology, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey.ORCID https://orcid.org/0000-0003-0666-6631
İlkim Deniz ToprakDivision of Immunology and Allergic Diseases, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey.ORCID https://orcid.org/0000-0002-9320-1252
Deniz Eyice KarabacakDivision of Immunology and Allergic Diseases, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey.ORCID https://orcid.org/0000-0001-7627-8621
Erdem BektasDepartment of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey.ORCID https://orcid.org/0000-0002-1348-4118
Derya UnalDivision of Immunology and Allergic Diseases, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey.ORCID https://orcid.org/0000-0001-9741-5939
Aslı GelincikDivision of Immunology and Allergic Diseases, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey.ORCID https://orcid.org/0000-0002-3524-9952
Semra DemirDivision of Immunology and Allergic Diseases, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey.ORCID https://orcid.org/0000-0003-3449-5868

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundHereditary angioedema (HAE) is a rare, autosomal dominantly inherited disease characterised by mucocutaneous oedema attacks. Vasoactive mediators and the endothelium play an important role in the pathogenesis of HAE. We aimed to evaluate the endothelial dysfunction in HAE.

methodsThe study included 35 C1 inhibitor deficient (C1-INH) HAE patients aged 18-50 years old without any risk factor that could cause endothelial dysfunction, and 25 sex- and age-matched healthy controls (HCs). Bilateral carotid intima-media thickness (CIMT), flow-mediated dilation (FMD) measurements, and transthoracic echocardiography (ECHO) imaging were performed. Demographic and clinical features of the patients were evaluated.

resultsThe percentage of FMD (FMD [%]) in C1-INH HAE patients was significantly lower than in the HCs (p < 0.001). There was no significant difference in the CIMT between C1-INH HAE patients and HCs. In addition, the findings of the ECHO were similar between the groups. C4 and C1 INH levels at diagnosis, gender, age, disease severity, presence of long-term prophylaxis treatment and attack frequency were not associated with FMD (%), whereas disease duration was correlated with lower FMD (%) (r = -0.480, p = 0.003).

conclusionThe present study indicated the presence of structural endothelial dysfunction in C1-INH HAE in the absence of atherosclerosis. Moreover, the study revealed that endothelial dysfunction was associated with disease duration, irrespective of disease severity. Further studies are required in order to assess mortality and morbidity due to endothelial dysfunction in C1-INH HAE and to determine the molecular mechanisms underlying endothelial dysfunction in C1-INH HAE.

Indexed as

carotid intima‐media thicknessendothelial dysfunctionflow‐mediated dilationhereditary angioedema

Identifiers

PMID40551525
PMCPMC12185901

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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.