CASE OF RENDU-OSLER DISEASE WITH THE LESIONS OF THE NASAL CAVITY AND BRONCHI

Abstract

A brief review of the literature devoted to the etiology, pathogenesis, clinical features and diagnosis of congenital hemorrhagic telangiectasia (Rendu-Osler disease) was done. There is also demonstrated a rare case of the personal experience of the authors, namely the observation of the patient with Rendu-Osler disease with local telangiectasia on the lower lip and the mucosa of the nasal cavity and in the bronchi; and in their absence, on the exposed parts of the body, which in its turn created significant difficulties in diagnosis. There were defined the following clinical features of this case: the debut of the disease at the age of 15 years old in the form of recurrent nasal bleeding; despite repeated surveys the causes of bleeding were not identified and there was only the treatment of anemia; the deteriorating course of the disease was associated with stressful situations in the family, a viral infection, exposure to mercury as a result of the person’s occupation, which led to pulmonary hemoptysis and bleeding of up to 200 ml per day; difficulties in diagnosis were due to the lack of typical telangiectasia on the open for examination parts of the body; the diagnosis was suspected clinically by careful examination of the lower lip mucosa where multiple telangiectasia were found. The diagnose was subsequently re-confirmed by bronchoscopy. So, if a patient has nasal or bronchopulmonary unspecified bleeding, hemoptysis, we recommend to have a targeted research on congenital hemorrhagic telangiectasia on the available to visual examination parts of the body to eliminate the local forms of the Rendu-Osler disease.