[Banti's syndrome complicating angiomatosis].
A boy, aged aged 11, developed sucessively splenomegaly, portal hypertension with hepatic fibrosis and finally pulmonary arteriovenous shunts which were responsible for chronic hypoxia. The authors discuss the classification of syndromes with hepatosplenomegaly that cause Banti's syndrome, and the pathogenesis of multiple pulmonary arterio-venous fistulae in certain liver diseases. The existence of multifocal abdominal, pulmonary and cutaneous vascular malformations suggests that there may be a diffuse vascular abnormality.
D000798:Angiomatosis / Q000150:complications*; D001165:Arteriovenous Malformations / Q000150:complications; D002648:Child; D005402:Fistula; D006801:Humans; D006971:Hypersplenism / Q000150:complications* / Q000473:pathology; D006975:Hypertension, Portal / Q000209:etiology; D008107:Liver Diseases / Q000150:complications; D008297:Male; D011651:Pulmonary Artery; D014652:Vascular Diseases / Q000235:genetics
Syndrome de banti au cours d'une maladie angiomateuse.