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]
Küçük Resim Yok
Tarih
2015
Dergi Başlığı
Dergi ISSN
Cilt Başlığı
Yayıncı
Turkiye Klinikleri
Erişim Hakkı
info:eu-repo/semantics/openAccess
Özet
Objective: 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.
Açıklama
Anahtar Kelimeler
Angioedemas, Awareness, Complement c1 inhibitor protein, Hereditary, Internal medicine, Knowledge, Physicians
Kaynak
Turkiye Klinikleri Journal of Medical Sciences
WoS Q Değeri
Scopus Q Değeri
Q4
Cilt
35
Sayı
2
