Psychometric Field Study of Hereditary Angioedema Quality of Life Questionnaire for Adults: HAE-QoL

Prior, Nieves; Remor, Eduardo; Pérez‐Fernández, Elia; Caminoa, Magdalena; Gómez‐Traseira, Carmen; Gayá, Francisco; Aabom, Anne; Aberer, Werner; Betschel, Stephen; Boccon‐Gibod, Isabelle; Bouillet, Laurence; Bygum, Anette; Csuka, Dorottya; Farkas, Henriette; Gomide, Maria; Grumach, Anete Sevciovic; Leibovich, Iris; Malbrán, Alejandro; Moldovan, Dumitru; Mihály, Enikő; Obtułowicz, Krystyna; Perpén, Cecilia; Peveling‐Oberhag, Adriane; Porębski, Grzegorz; Chavannes, Celine Rayonne; Reshef, Avner; Staubach, Petra; Wiednig, Michaela; Caballero, Teresa

doi:10.1016/j.jaip.2015.12.010

Cited by 58 publications

(79 citation statements)

References 45 publications

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“…The AE-QoL has a 4-dimensional structure (functioning, fatigue/mood, fears/shame, and food) and a total score. The HAE-QoL is a disease-specific QoL questionnaire recently introduced to specifically assess HR-QoL in HAE patients [150, 151]. …”

Section: Therapiesmentioning

confidence: 99%

Hereditary Angio-Oedema for Dermatologists

Bygum¹

2019

Dermatology

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Among angio-oedema patients, hereditary angio-oedema (HAE) should not be overlooked. Besides skin swellings, these patients might have very painful abdominal attacks and potentially life-threatening angio-oedema of the upper airway. They will not respond to traditional anti-allergic therapy with antihistamines, corticosteroids, and adrenaline, and instead need specific drugs targeting the kallikrein-kinin pathway. Classically, patients with HAE have a quantitative or qualitative deficiency of the C1 inhibitor (C1INH) due to different mutations in SERPING1, although a new subtype with normal C1INH has been recognised more recently. This latter variant is diagnosed based on clinical features, family history, or molecular genetic testing for mutations in F12, ANGPT1,or PLG.The diagnosis of HAE is often delayed due to a general unfamiliarity with this orphan disease. However, undiagnosed patients are at an increased risk of unnecessary surgical interventions or life-threatening laryngeal swellings. Within the last decade, new and effective therapies have been developed and launched for acute and prophylactic therapy. Even more drugs are under evaluation in clinical trials. It is therefore of utmost importance that patients with HAE are diagnosed as soon as possible and offered relevant therapy with orphan drugs to reduce morbidity, prevent mortality, and improve quality of life.

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Section: Therapiesmentioning

confidence: 99%

Hereditary Angio-Oedema for Dermatologists

Bygum¹

2019

Dermatology

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“…Preparaty zawierające estrogeny (doustne leki antykoncepcyjne, hormonalna terapia zastępcza) oraz inhibitory konwertazy angiotensyny zwiększają częstość występowania obrzęków [44][45][46]. Przewlekły charakter choroby istotnie obniża jakość życia pacjentów [47].…”

Section: Obraz Kliniczny Wrodzonego Obrzęku Naczynioruchowego Z Niedounclassified

Management of hereditary angioedema with C1 inhibitor-deficiency – consensus statement of the HAE Section of the Polish Society of Allergology. Part I: classification, pathophysiology, clinical symptoms, and diagnosis

Porębski¹,

Gocki²,

Juchacz³

et al. 2018

pja

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“…Monitorowanie aktywności choroby, jakości życia chorych, działań niepożądanych leków powinno się odbywać podczas regularnych wizyt w ośrodku specjalizującym się w leczeniu HAE-C1-INH co najmniej raz w roku. Pacjenci z niestabilnym przebiegiem choroby, nowo zdiagnozowani, przewlekle leczeni androgenami wymagają częstszych wizyt kontrolnych [28,43].…”

Section: Leczenie Domoweunclassified