Biomaterial Database

Atypical biliary cirrhosis--or sclerosing cholangitis. 1980

J T Galambos, and W S Brooks

Four patients had a characteristic cholestatic syndrome intermediate between primary biliary cirrhosis (PBC) and sclerosing cholangitis (SC). These patients with atypical biliary cirrhosis (ABC) differ from patients with PBC in that they do not have either antimitochondrial antibodies or elevated IgM, are younger, and three of the four are men. Patients with ABC differ from those with SC in that the common duct, hepatic duct, the right and left hepatic ducts and their immediate tributaries show none of the changes characteristic and diagnostic of SC. The findings in ABC which are similar to PBC are: 1) the earliest biopsy finding of the disease is confined to the interlobular duct and is indistinguishable from PBC; 2) late changes on cholangiogram are similar to those in a proportion of patients with PBC; and 3) the incidence of gallstones is increased. ABC has no similarity to SC except that the small intrahepatic ducts are affected by both diseases. The histologic features in fully established ABC suggest extrahepatic bile duct obstruction because of the predominantly PMN leukocytes portal inflammation. The cholangiograms show no evidence of bile duct obstruction, but there is rapid attenuation and narrowing of the smaller intrahepatic bile duct. Cholecystectomy does not improve the clinical course of ABC.

UI	MeSH Term	Description	Entries
D007075	Immunoglobulin M	A class of immunoglobulin bearing mu chains (IMMUNOGLOBULIN MU-CHAINS). IgM can fix COMPLEMENT. The name comes from its high molecular weight and originally was called a macroglobulin.	Gamma Globulin, 19S,IgM,IgM Antibody,IgM1,IgM2,19S Gamma Globulin,Antibody, IgM
D008099	Liver	A large lobed glandular organ in the abdomen of vertebrates that is responsible for detoxification, metabolism, synthesis and storage of various substances.	Livers
D008105	Liver Cirrhosis, Biliary	FIBROSIS of the hepatic parenchyma due to obstruction of BILE flow (CHOLESTASIS) in the intrahepatic or extrahepatic bile ducts (BILE DUCTS, INTRAHEPATIC; BILE DUCTS, EXTRAHEPATIC). Primary biliary cholangitis involves the destruction of small intra-hepatic bile ducts and decreased bile secretion. Secondary biliary cholangitis is produced by prolonged obstruction of large intrahepatic or extrahepatic bile ducts from a variety of causes.	Biliary Cirrhosis,Biliary Cirrhosis, Primary,Biliary Cirrhosis, Secondary,Cholangitis, Chronic Nonsuppurative Destructive,Liver Cirrhosis, Obstructive,Primary Biliary Cholangitis,Biliary Cirrhosis, Primary, 1,Primary Biliary Cirrhosis,Secondary Biliary Cholangitis,Secondary Biliary Cirrhosis,Biliary Cholangitides, Primary,Biliary Cholangitis, Primary,Biliary Cholangitis, Secondary,Cholangitides, Primary Biliary,Cholangitis, Primary Biliary,Cholangitis, Secondary Biliary,Cirrhosis, Biliary,Cirrhosis, Secondary Biliary,Liver Cirrhoses, Biliary,Obstructive Liver Cirrhosis,Primary Biliary Cholangitides,Secondary Biliary Cholangitides
D008297	Male		Males
D008930	Mitochondria, Liver	Mitochondria in hepatocytes. As in all mitochondria, there are an outer membrane and an inner membrane, together creating two separate mitochondrial compartments: the internal matrix space and a much narrower intermembrane space. In the liver mitochondrion, an estimated 67% of the total mitochondrial proteins is located in the matrix. (From Alberts et al., Molecular Biology of the Cell, 2d ed, p343-4)	Liver Mitochondria,Liver Mitochondrion,Mitochondrion, Liver
D009504	Neutrophils	Granular leukocytes having a nucleus with three to five lobes connected by slender threads of chromatin, and cytoplasm containing fine inconspicuous granules and stainable by neutral dyes.	LE Cells,Leukocytes, Polymorphonuclear,Polymorphonuclear Leukocytes,Polymorphonuclear Neutrophils,Neutrophil Band Cells,Band Cell, Neutrophil,Cell, LE,LE Cell,Leukocyte, Polymorphonuclear,Neutrophil,Neutrophil Band Cell,Neutrophil, Polymorphonuclear,Polymorphonuclear Leukocyte,Polymorphonuclear Neutrophil
D002761	Cholangitis	Inflammation of the biliary ductal system (BILE DUCTS); intrahepatic, extrahepatic, or both.	Cholangitides
D003937	Diagnosis, Differential	Determination of which one of two or more diseases or conditions a patient is suffering from by systematically comparing and contrasting results of diagnostic measures.	Diagnoses, Differential,Differential Diagnoses,Differential Diagnosis
D005260	Female		Females
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man