ArticleFrontiers in medicine2026
Frailty in elderly with a rare genetic disease: a geriatric score analysis in C1 inhibitor Hereditary Angioedema.
Article in Frontiers in medicine, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
Introduction: Patients with Hereditary Angioedema related to deficiency in C1-esterase inhibitor (HAE-C1INH) experience a relevant burden of both lifelong disease and treatments. The coexistence of additional comorbidities concurs with the reduction of the quality of life (QoL) often resulting in social-psychological isolation and potential multidimensional frailty. We aimed at assessing for the first time frailty in elderly with HAE-C1INH by using a comprehensive geriatric assessment (CGA). Patients and methods: A single-center cross-sectional comparative study with a control group included elderly patients with a defined diagnosis of HAE-C1INH recruited from the HAE Reference Center (Tor Vergata University Hospital, Rome, Italy). Healthy controls (HC) were enrolled from subjects referring to the Geriatric Unit, at the same Hospital. HAE-C1INH burden was assessed by disease activity (HAE-AS) and quality of life (HAE-QoL). All subjects in the study underwent CGA including Mini Mental State Examination (MMSE), Geriatric Depression Scale (GDS), Activities of Daily Living (ADLs) and Instrumental ADL (IADLs), and the Tinetti test. The comorbidity burden was measured by Charlson Comorbidity Index (CCI) and frailty status by using the Clinical Frailty Scale (CFS). Results: Thirty HAE-C1INH patients from 20 unrelated families were included and compared with age/sex matched HC ( Conclusion: Geriatric score analysis shows that older adults with HAE-C1INH exhibit age-related frailty comparable to general population controls. As the proportion of HAE-C1INH patients achieving the geriatric age threshold is growing, defining disease-specific frailty remains crucial for enhancing care in older adults.
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