Primary biliary cirrhosis complicated by severe hypoxemia.
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.
Shiomi
S
S
Third Department of Internal Medicine, Osaka City University Medical School, Japan.
Kuroki
T
T
Fujimoto
S
S
Yamaoka
M
M
Sakaguchi
H
H
Takeda
T
T
Nishiguchi
S
S
Nakajima
S
S
Kobayashi
K
K
Ochi
H
H
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