Hereditary hemorrhagic telangiectasia associated to chronic active hepatitis.
Hereditary hemorrhagic telangiectasia or Rendu-Osler-Weber syndrome (ROWS) is an angiodisplasia characterized clinically by the presence of telangiectasias in the skin and mucosas, particularly on the face, conjunctivas, nose, pharynx and rarely on the thorax. Visceral involvement specially hepatic cirrhosis associated with pulmonary arteriovenous fistulae have been described. We describe a 7 10/12 years old girl with the diagnosis of ROWS associated with chronic active hepatitis with positive HBAg that evolved to hepatic cirrhosis. This is the second case of ROWS associated with chronic active hepatitis reported in the literature, being however, the first one described in the childhood.
D001707:Biopsy, Needle; D002648:Child; D002908:Chronic Disease; D005260:Female; D006509:Hepatitis B / Q000150:complications*; D006801:Humans; D008099:Liver / Q000473:pathology; D008105:Liver Cirrhosis, Biliary / Q000150:complications*; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000150:complications*