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Journals / Turkish Journal of Hematology / 2019 / Cilt: 36 - Sayı: 1

Thalidomide for the Management of Bleeding Episodes in Patients with Hereditary Hemorrhagic Telangiectasia: Effects on Epistaxis Severity Score and Quality of Life

Mehmet BAYSAL
Elif ÜMİT
Hakkı KIRKIZLAR
Ali ÖZDÖVER
Ahmet DEMİR
Pages
43–47
DOI
—
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AI Summary & Key Takeaways

AI Insight
⚡ TL;DR(Too Long; Didn't Read)

Thalidomidemay be effective to control bleeding episodes with a reasonabletolerance profile in patients with HHT.

📌 3 Key Takeaways

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Objective & Hypothesis:Hereditary hemorrhagic telangiectasia (HHT) is a rare autosomaldominantly inherited disorder characterized by bleeding episodes.These episodes tend to happen spontaneously and reduce the qualityof life.
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Key Findings & Data:In ourstudy, we evaluated 6 patients with HHT, calculating their EpistaxisSeverity Score (ESS) and performing a quality of life assessmentwith the 36-Item Short Form Health Survey Questionnaire (SF-36),and we studied the alterations of these evaluations with thalidomidetreatment.
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Conclusion & Impact:Thalidomidemay be effective to control bleeding episodes with a reasonabletolerance profile in patients with HHT.
🔬Design:Survey & Empirical Study
📍Scope:Comprehensive Sample
⚙️Variables:Domain Parameters

Özet

Hereditary hemorrhagic telangiectasia (HHT) is a rare autosomaldominantly inherited disorder characterized by bleeding episodes.These episodes tend to happen spontaneously and reduce the qualityof life. Patients are often unresponsive to local measures. With thepathophysiological role of angiogenesis in HHT, antiangiogenic drugsincluding thalidomide are used to control bleeding episodes. In ourstudy, we evaluated 6 patients with HHT, calculating their EpistaxisSeverity Score (ESS) and performing a quality of life assessmentwith the 36-Item Short Form Health Survey Questionnaire (SF-36),and we studied the alterations of these evaluations with thalidomidetreatment. Three patients were male and three were female. Mean agewas 60.50 years. No side effects were observed during the treatmentperiod. Improvements of certain SF-36 dimensions including physicalfunctioning, physical component summary, and mental componentsummary and of the ESS were observed after treatment. Thalidomidemay be effective to control bleeding episodes with a reasonabletolerance profile in patients with HHT.

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Cite This Article

BAYSAL, M., ÜMİT, E., KIRKIZLAR, H., ÖZDÖVER, A., DEMİR, A. (2019). Thalidomide for the Management of Bleeding Episodes in Patients with Hereditary Hemorrhagic Telangiectasia: Effects on Epistaxis Severity Score and Quality of Life. Turkish Journal of Hematology, Cilt: 36 - Sayı: 1, 43–47. https://ajindex.com/articles/412380

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