Cystic fibrosis-cirrhosis, portal hypertension, and liver biopsy: Reply.

摘要

We welcome the letter by Witters et al. highlighting the important issue of noncirrhotic portal hypertension (NCPH) in cystic fibrosis (CF). This group's data are complementary to ours, focusing on liver biopsy findings in 12 patients (ages not supplied), all with established portal hypertension yet only five with established cirrhosis. Of seven cases without cirrhosis, four were from explanted livers permitting direct inspection and avoidance of sampling error. The presinusoidal origin of the portal hypertension was confirmed in two patients who had hepatic venous pressure gradients measured at only 5 and 9 mm Hg. Witters et al. also report portal venopathy in liver biopsies from all patients classified as NCPH (whose fibrosis on biopsy did not reach criteria for established cirrhosis), a finding more prevalent than in biopsies from an uncharacterized cohort of 20 children with CF-associated liver disease (CFLD) without portal hypertension.