title: Pulmonary hypertension with coexisting portal hypertension.
abstract: A 10-year-old boy who had portal hypertension secondary to portal fibrosis/intrahepatic biliary atresia developed syncopal episodes related to strenuous activity. A work-up excluded a metabolic or neurologic etiology and cardiac catheterization demonstrated significant pulmonary hypertension. Six months later he died and an autopsy revealed pulmonary plexogenic arteriopathy without microemboli. Previous reported cases of this symptom complex are reviewed. In addition, he had a history of nonspecific colitis, ulcerative stomatitis, and conjunctivitis responsive to steroid therapy. The possible relationship of these manifestations to the portal pulmonary pathology is discussed.
authors.affiliation: Department of Pathology and Laboratory Medicine, University of Wisconsin School of Medicine, Madison 53792.
authors.forename: S O
authors.initials: SO
authors.lastname: Rossi
authors.forename: E
authors.initials: E
authors.lastname: Gilbert-Barness
authors.forename: T
authors.initials: T
authors.lastname: Saari
authors.forename: R
authors.initials: R
authors.lastname: Corliss
mesh_terms: D001656:Biliary Atresia / Q000150:complications; D002648:Child; D006801:Humans; D006975:Hypertension, Portal / Q000150:complications* / Q000209:etiology; D006976:Hypertension, Pulmonary / Q000150:complications* / Q000473:pathology; D008103:Liver Cirrhosis / Q000150:complications; D008297:Male