How familiar are internists with a potentially deadly orphan disease?: Hereditary angioedema [Ölümcül olabilen yetim bir hastalığa ıç hastalıkları uzmanları ne kadar Aşina?: Herediter anjiyoödem]

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dc.contributor.authorMete Gökmen N.
dc.contributor.authorGülbahar O.
dc.contributor.authorPeker Koç Z.
dc.contributor.authorBüyüköztürk S.
dc.contributor.authorSin A.Z.
dc.contributor.authorGelincik A.
dc.contributor.authorGürlek F.
dc.contributor.authorArdeniz Ö.
dc.contributor.authorKokuludağ A.
dc.date.accessioned2019-10-26T21:25:42Z
dc.date.available2019-10-26T21:25:42Z
dc.date.issued2015
dc.departmentEge Üniversitesien_US
dc.description.abstractObjective: Hereditary angioedema (HAE) is a rare genetic disorder characterized by episodes of swelling in the skin, gastrointestinal tract and larynx. Laryngeal attacks can be fatal, especially in mis-/undiagnosed cases. In Turkey, the mean diagnostic delay of HAE is as long as 26 years. This study was conducted to assess Turkish doctors’ awareness of HAE. Material and Methods: A 20- question questionnaire was completed by 155 internal medicine specialists from among the attendants of 14th National Congress of Internal Medicine in Turkey. The questionnaire included HAE-related questions as well as demographic items. Results: Most doctors (93.5%) reported that they had heard of HAE, and 41.9% had followed at least one patient with HAE, however, 22% of them understood the role of C1 inhibitor in HAE, but 38.7% had no idea about HAE pathogenesis. The only fatal symptom, laryngeal edema, was named by 18% of respondents. Five percent of the respondents knew C4 level was the screening test; 6% knew that C1-INH level/function analysis is necessary for diagnosis. Approximately 10.3% of respondents knew an effective treatment for acute attacks; 18.7% knew a long-term prophylactic therapy. Conclusion: We concluded that although most internists are aware of HAE, they are not knowledgeable enough to diagnose and manage the disease. © 2015 by Türkiye Klinikleri.en_US
dc.identifier.doi10.5336/medsci.2014-42236
dc.identifier.endpage72en_US
dc.identifier.issn1300-0292
dc.identifier.issn1300-0292en_US
dc.identifier.issue2en_US
dc.identifier.scopusqualityQ4en_US
dc.identifier.startpage67en_US
dc.identifier.urihttps://doi.org/10.5336/medsci.2014-42236
dc.identifier.urihttps://hdl.handle.net/11454/17283
dc.identifier.volume35en_US
dc.indekslendigikaynakScopusen_US
dc.language.isoenen_US
dc.publisherTurkiye Kliniklerien_US
dc.relation.ispartofTurkiye Klinikleri Journal of Medical Sciencesen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/openAccessen_US
dc.subjectAngioedemasen_US
dc.subjectAwarenessen_US
dc.subjectComplement c1 inhibitor proteinen_US
dc.subjectHereditaryen_US
dc.subjectInternal medicineen_US
dc.subjectKnowledgeen_US
dc.subjectPhysiciansen_US
dc.titleHow familiar are internists with a potentially deadly orphan disease?: Hereditary angioedema [Ölümcül olabilen yetim bir hastalığa ıç hastalıkları uzmanları ne kadar Aşina?: Herediter anjiyoödem]en_US
dc.typeArticleen_US

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