Aralik 2015
Kalp yetmezligi ve tipik karaciger tutulumu bulgulariyla kendini gösteren Osler-Weber- Rendu olgusu
A case of Osler-Weber-Rendu syndrome with typical liver involvement and hearth failure
- Ana Sayfa
- Sayılar
- Aralik 2015
- Kalp yetmezligi ve tipik karaciger tutulumu bulgulariyla kendini gösteren Osler-Weber- Rendu olgusu...
Özet
Osler-Weber-Rendu sendromu deri ve mukozalarda telenjiektaziler ve iç organlarda arteriovenöz malformasyonlar ile karakterize, nadir görülen, otozomal dominant geçis gösteren bir hastaliktir. Altmis yasindaki kadin hasta tekrarlayan üst gastrointestinal sistem ve burun kanamasi nedeniyle klinigimize yatirildi. Hastanin fizik muayenesinde agiz içi, dudak ve burun mukozasinda yaygin telenjiektaziler, anemi, kalp yetmezligi ve hepatomegali bulgulari vardi. Yapilan tetkikler sonucu karacigerde yaygin arteriovenöz santlar, pulmoner hipertansiyon ve kalp yetmezligi saptandi.
Abstract
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
