Portal Hypertension and Primary Biliary Cirrhosis

Abstract

Portal hypertension has been regarded as an uncommon and late complication of primary biliary cirrhosis (PBC). 24 patients with PBC were investigated for portal hypertension. Esophageal varices were present in 20, 50, and 90 % of the patients 1, 3, and 9 years, respectively, after the onset of pruritus and/or jaundice. Portal hypertension was responsible for gastrointestinal bleedings in 11 patients; bleeding was the first clinical manifestation of PBC in two of them. Wedged hepatic venous pressure was increased in all the patients with portal hypertension whether regenerative nodules were present or absent. Portacaval shunt was performed in five patients and was well tolerated in three of them. It is concluded that (a) portal hypertension is common in PBC; (b) the intrahepatic block is of the so-called postsinusoidal type, even in patients without regenerative nodules; (c) gastrointestinal bleeding due to portal hypertension occurs in about half of the patients and may be the first manifestation of PBC; (d) portacaval shunt seems to be indicated when gastrointestinal bleeding occurs in earlier stage of the disease.

Variceal bleeding in primary biliary cirrhosis patients: a subgroup with improved prognosis and a model to predict survival after first bleeding

“…Only a few publications in the last 37 years have reported on the prevalence of portal hypertension in PBC and a very few on bleeding [14,[23][24][25][26]. In our original cohort of 370 patients, 102 (27.5%) bled, a similar proportion as in some previous publications [23,24], but lower than others with a shorter follow-up [25].…”

“…Although large controlled trials have shown no improvement in survival of cirrhotic patients after shunt surgery [32], including those with PBC [33], it has been suggested that portocaval shunt is indicated in PBC when bleeding occurs in earlier stages of the disease [26]. In our cohort 23 patients underwent shunt surgery.…”

Somatomedin Production in Normal Adults and Cirrhotic Patients

“…Portal blood samples were not collected in this study but we have previously reported no difference between portal and peripheral SM serum activities in normal dogs (Schimpff el al. In a first study no correlation was found between the hepatic blood flow and the degree of liver failure (Lebrec et al 1973). The average of SM activity production is 398 ± 334 U/min in normal subjects and significantly lower (P < 0.01) in cirrhotic patients.…”

Portal hypertension in primary biliary cirrhosis (PBC): A reversible condition? Yes, but not in all UDCA treated patients

“…Although well recognized, the exact physiopathology of this phenomenon is still not completely elucidated. Nodular regenerative hyperplasia 24 a rare condition, as well as lymphoid aggregates in the portal tracts (such as granulomas) can be contributing factors 3,22–24 . Thrombosis of terminal portal venules and contracture of the sinusoidal endothelial cells and/or stellate cells can also be incriminated but are found in other types of cirrhosis independently of the site of portal hypertension.…”