Non-cirrhotic portal fibrosis associated with pulmonary arteriovenous communication and pulmonary arterial hypertension.
Southeast Asian J Trop Med Public Health
;
1998 Mar; 29(1): 76-9
Article
in English
| IMSEAR
| ID: sea-35135
ABSTRACT
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.
Full text:
Available
Index:
IMSEAR (South-East Asia)
Main subject:
Pedigree
/
Humans
/
Male
/
Family
/
Child
/
Hematemesis
/
Hypertension, Portal
/
Hypertension, Pulmonary
/
Liver
/
Liver Cirrhosis
Language:
English
Journal:
Southeast Asian J Trop Med Public Health
Year:
1998
Type:
Article
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