Elif Tuğba TUNCEL, Hüseyin KAÇMAZ, Muhsin KAYA

Kalp yetmezliği ve tipik karaciğer tutulumu bulgularıyla kendini gösteren Osler-Weber- Rendu olgusu

Osler-Weber-Rendu sendromu deri ve mukozalarda telenjiektaziler ve iç organlarda arteriovenöz malformasyonlar ile karakterize, nadir görülen, otozomal dominant geçiş gösteren bir hastalıktır. Altmış yaşındaki kadın hasta tekrarlayan üst gastrointestinal sistem ve burun kanaması nedeniyle kliniğimize yatırıldı. Hastanın fizik muayenesinde ağız içi, dudak ve burun mukozasında yaygın telenjiektaziler, anemi, kalp yetmezliği ve hepatomegali bulguları vardı. Yapılan tetkikler sonucu karaciğerde yaygın arteriovenöz şantlar, pulmoner hipertansiyon ve kalp yetmezliği saptandı.

A case of Osler-Weber-Rendu syndrome with typical liver involvement and hearth failure

Osler-Weber-rendu syndrome is characterized by telengectasia, which is mostly situated in the skin and mucous membrane, and multiple arteriovenous malformations localized to the internal organs. It is a rarely seen, inherited disease with autosomal dominant transmission. A 60-year-old female was admitted to our clinic for recurrent upper gastrointestinal tract and nose bleeding. Physical examination showed multiple telengectasia localized to the mouth, lips and nose, as well as anemia, heart failure and hepatomegaly. Radiological investigations revealed multiple arteriovenous shunts localized in the liver, pulmonary hypertension and heart failure.

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