Non-cirrhotic portal fibrosis associated with pulmonary arteriovenous communication and pulmonary arterial hypertension.
A case of non-cirrhotic portal fibrosis associated with pulmonary arteriovenous communication and pulmonary arterial hypertension is reported. The patient was a 7-year old boy who presented with hematemesis, cyanosis, hypoxemia and orthodeoxia. His liver pathology was compatible with non-cirrhotic portal fibrosis. His pulmonary angiography showed arteriovenous shunting and pulmonary arterial hypertension (mean pulmonary artery pressure 34 mmHg). His sister also had non-cirrhotic portal fibrosis with neither hypoxemia nor orthodeoxia. This report raises the possibility of non-cirrhotic portal fibrosis having a genetic etiology.
Chongsrisawat
V
V
Department of Pediatrics, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand.
Vivatvakin
B
B
Suwangool
P
P
Vajragupta
L
L
Poovorawan
Y
Y
D002648:Child; D005190:Family; D006396:Hematemesis / Q000150:complications / Q000473:pathology; D006801:Humans; D006975:Hypertension, Portal / Q000150:complications* / Q000473:pathology; D006976:Hypertension, Pulmonary / Q000150:complications* / Q000473:pathology; D000860:Hypoxia / Q000150:complications / Q000473:pathology; D008099:Liver / Q000473:pathology; D008103:Liver Cirrhosis / Q000473:pathology*; D008297:Male; D010375:Pedigree