title: Primary biliary cirrhosis complicated by severe hypoxemia.
abstract: The case reported here is of a 61-year-old woman diagnosed with hepatopulmonary syndrome. She had had severe hypoxemia for 5 years. In room air, her arterial oxygen tension was low, and although we anticipated that treatment with oxygen would increase it, the treatment failed to do so. Pulmonary perfusion imaging with Tc-99m-labelled macroaggregated albumin showed an arterio-venous shunt in the lungs. Blood tests and liver scanning with Tc-99m-labelled galactosyl human serum albumin showed abnormalities of liver function, and per-rectal portal scintigraphy with Tc-99m pertechnetate showed severe portal hypertension. Inspection of a biopsy specimen taken under ultrasonographic guidance showed primary biliary cirrhosis of Scheuer stage I. The causes of hepatopulmonary syndrome are unknown, but seem to include a decrease in hepatic functional reserve, portal hypertension, or other factors. The development of hepatopulmonary syndrome due to portal hypertension in this patient is of interest.
authors.affiliation: Third Department of Internal Medicine, Osaka City University Medical School, Japan.
authors.forename: S
authors.initials: S
authors.lastname: Shiomi
authors.forename: T
authors.initials: T
authors.lastname: Kuroki
authors.forename: S
authors.initials: S
authors.lastname: Fujimoto
authors.forename: M
authors.initials: M
authors.lastname: Yamaoka
authors.forename: H
authors.initials: H
authors.lastname: Sakaguchi
authors.forename: T
authors.initials: T
authors.lastname: Takeda
authors.forename: S
authors.initials: S
authors.lastname: Nishiguchi
authors.forename: S
authors.initials: S
authors.lastname: Nakajima
authors.forename: K
authors.initials: K
authors.lastname: Kobayashi
authors.forename: H
authors.initials: H
authors.lastname: Ochi
mesh_terms: D001706:Biopsy; D005260:Female; D006801:Humans; D006975:Hypertension, Portal / Q000150:complications* / Q000175:diagnosis; D000860:Hypoxia / Q000175:diagnosis / Q000209:etiology*; D008099:Liver / Q000000981:diagnostic imaging / Q000473:pathology; D008105:Liver Cirrhosis, Biliary / Q000150:complications* / Q000175:diagnosis; D008168:Lung / Q000000981:diagnostic imaging; D008875:Middle Aged; D011877:Radionuclide Imaging; D013577:Syndrome