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.

Hematologic traits and primary biliary cholangitis: a Mendelian randomization study

“…Additionally, hypoxia has been suggested to be involved in the pathogenesis of chronic liver diseases [16,17], including PBC [18]. Meanwhile, cases of PBC with pernicious anemia or hypoxia have been frequently reported [19,20], suggesting the possible functional links between PBC and anemia. Similarly, hematocrit reflects the percentage of blood volume that is composed of red blood cells, and provides similar clinical information as hemoglobin.…”