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
Artículo
en Inglés
| 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.
Texto completo:
Disponible
Índice:
IMSEAR (Asia Sudoriental)
Asunto principal:
Linaje
/
Humanos
/
Masculino
/
Familia
/
Niño
/
Hematemesis
/
Hipertensión Portal
/
Hipertensión Pulmonar
/
Hígado
/
Cirrosis Hepática
Idioma:
Inglés
Revista:
Southeast Asian J Trop Med Public Health
Año:
1998
Tipo del documento:
Artículo
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