title: Bile-duct destruction and collagen deposition: a prominent ultrastructural feature of the liver in cystic fibrosis.
abstract: To study the liver disease of patients with cystic fibrosis, percutaneous liver biopsies were performed in 10 patients with cystic fibrosis aged 6 to 22 yr. Nine of 10 patients had high Shwachman scores, eight had normal serum levels of transaminases. Light-microscopical examination showed steatosis in seven cases and in five slight or moderate inflammatory infiltration. Eight patients showed varying degrees of fibrosis and even cirrhosis. Six patients had bile-duct proliferation and, in one case a bile plug was found. Other signs of cholestasis were not seen. Electron-microscopical investigation showed no specific signs of cholestasis such as ductal plugs or intracellular bile pigments. The canaliculi were not dilated, except in one case. Most patients had bile-duct cells with irregular shapes, protruding into the lumen, and some cases even had necrotic cells. Around the bile ducts and ductules, collagen was deposited and fat-storing cells were a common finding. Our findings do not support the view that cholestasis is the pathogenetic factor in liver disease in cystic fibrosis. A cytotoxic influence on the biliary cells, stimulating collagen deposition, seems more likely.
authors.affiliation: Department of Pediatrics, Karolinska Institutet, Huddinge Hospital, Sweden.
authors.forename: A
authors.initials: A
authors.lastname: Lindblad
authors.forename: R
authors.initials: R
authors.lastname: Hultcrantz
authors.forename: B
authors.initials: B
authors.lastname: Strandvik
mesh_terms: D000293:Adolescent; D000328:Adult; D001652:Bile Ducts / Q000648:ultrastructure*; D002648:Child; D002779:Cholestasis / Q000473:pathology; D003094:Collagen / Q000378:metabolism*; D003550:Cystic Fibrosis / Q000378:metabolism / Q000473:pathology*; D005260:Female; D006801:Humans; D008099:Liver / Q000648:ultrastructure*; D008297:Male; D008854:Microscopy, Electron
chemical_list: D003094:Collagen