Osler-Weber-Rendu sendromu: Bir olgu sunumu
Öz
Özet
Osler-Weber-Rendu sendromu [Herediter Hemorajik Telenjiektazi] deri ve mukozalarda telenjiektaziler ve iç organlarda arteriyovenöz malformasyonlar ile karakterize otozomal dominant geçiş gösteren bir hastalıktır. Dudak, dil, damak, kulak, nazal mukoza, palmoplantar bölge ve tırnak yataklarında yerleşim gösterebilen telenjiektaziler hastalığın tipik bulgusudur. İç organ tutulumu olan hastalıkta pulmoner arteriovenöz malformasyon sıktır. Olgumuz dudağında ve yüzün sağ yarısında hemanjiom, oral mukozada telenjiektaziler ve sol akciğer alt lobunda arteriyovenöz malformasyonu olan 34 yaşında kadın hastaydı. Osler-Weber-Rendu sendromu tanısı konuldu. Hastalığının nadir görülmesi ve akciğerinde eşlik eden arteriyovenöz malformasyonu bulunması nedeniyle olgumuzu sunmayı uygun gördük.
Anahtar sözcükler: Herediter hemorajik telenjiektazi, Osler-Weber-Rendu sendromu, pulmoner arteriyovenöz malformasyon
Abstract
Osler-Weber-Rendu (Hereditary Hemorrhagic Telangiectasia) is an autosomal dominant disease characterized by telangiectasia on mucosa and skin and arteriovenous malformations in visceral organs. Telangiectasia on lips, tongue, ear, nasal mucosa, palmoplantar region and nail beds are typical findings in the disease. Visceral arteriovenous malformations are common in patients who have visceral involvements. Our case was 34 years old woman presented with hemangioma on her right side of face and lips, telangiectasia on oral mucosa and arteriovenous malformation in her lower lobe of left lung. Diagnosis was Osler-Weber-Rendu disease. We decided to report this case because it is rarely seen and arteriovenous malformation was present in the lung of the patient.
Keywords: Osler-Weber-Rendu syndrome, hereditary hemorrhagic Telangiectasia, pulmonary arteriovenous malformationAnahtar Kelimeler
References
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Details
Primary Language
Turkish
Subjects
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Journal Section
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Publication Date
March 28, 2014
Submission Date
October 22, 2013
Acceptance Date
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Published in Issue
Year 2014 Volume: 36 Number: 1