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Öğe 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](Turkiye Klinikleri, 2015) Mete Gökmen N.; Gülbahar O.; Peker Koç Z.; Büyüköztürk S.; Sin A.Z.; Gelincik A.; Gürlek F.; Ardeniz Ö.; Kokuludağ A.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.Öğe Quality-of-life in insect venom allergy: validation of the Turkish version of the “Vespid Allergy Quality of Life Questionnaire” (VQLQ-T)(SpringerOpen, 2016) Sin B.A.; Öztuna D.; Gelincik A.; Gürlek F.; Baysan A.; Sin A.Z.; Aydın Ö.; Mısırlıgil Z.Purpose: “Vespid Allergy Quality of Life Questionnaire (VQLQ)” has been used to assess psychological burden of disease. The aim of this study was to evaluate validity, reliability and responsiveness to interventions of the Turkish version. Methods: The Turkish language Questionnaire (VQLQ-T) was administered to 81 patients with bee allergy and 65 patients with vespid allergy from different groups to achieve cross-sectional validation. To establish longitudinal validity, the questionnaire was administered to 36 patients treated with venom immunotherapy. Results: The cross-sectional validation in patients with vespid venom allergy showed a correlation coefficient of 0.97 (Cronbach ?). Spearman’s correlation coefficient of the pretreatment VQLQ-T score with Expectation of Outcome (EoO) questionnaire score was 0.55 (p < 0.001). After treatment, correlation between VQLQ-T score and EoO score was 0.64 (p = 0.003) in these patients. The cross-sectional instrument validation for non-beekeepers with bee venom allergy yielded a correlation coefficient of 0.96 (Cronbach ?). Spearman’s correlation coefficient between pretreatment VQLQ-T score and EoO score was 0.47 (p < 0.001) and after treatment, correlation between VQLQ-T score and EoO score was 0.78 (p = 0.008) in these patients. These findings indicate cross-sectional validity of VQLQ-T. In the longitudinal validation, there was a positive correlation between EoO and VQLQ-T with a correlation coefficient of 0.562 (p < 0.001). While mean (±SD) VQLQ-T score was 5.27 (±1.29) in pretreatment, it was 2.78 (±1.01) after treatment (p < 0.001). The correlation between the mean change in VQLQ-T score and the mean change in EoO score was 0.42 (p = 0.011). Conclusions: The Turkish version of VQLQ-T enables measurement of Quality of Life (QoL) in patients with either vespid or bee venom allergy. Furthermore, responsiveness of this instrument demonstrates the questionnaire’s ability to detect changes over time. © 2016, The Author(s).