Biomaterial Database

Hepatic copper accumulation in primary biliary cirrhosis. 1979

G D Benson

Hepatic copper accumulation is a regular feature of primary biliary cirrhosis (PBC). The levels are directly related to the clinical stage of the disease. Since the copper values in PBC are comparable to Wilson's disease, there is the potential for copper toxicity, although this is speculative since the two diseases differ in the binding, distribution, and intracellular localization of the copper. The involvement of copper toxicity in the progression of PBC is supported by the observation that the highest values occur in association with the hepatic failure that occurs in the advanced stage.Corticosteroid therapy appears to decrease hepatic copper levels in PBC. Although this therapy does not invariably lower the hepatic Cu content in patients with PBC, it does so in many individuals. Therapeutic trials with d-penicillamine are in progress. When results are available they will guide us in the management of individual patients with PBC. In the meantime, dietary copper should be restricted as is done in management of Wilson's disease.

UI	MeSH Term	Description	Entries
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
D010396	Penicillamine	3-Mercapto-D-valine. The most characteristic degradation product of the penicillin antibiotics. It is used as an antirheumatic and as a chelating agent in Wilson's disease.	Dimethylcysteine,Mercaptovaline,beta,beta-Dimethylcysteine,Copper Penicillaminate,Cuprenil,Cuprimine,D-3-Mercaptovaline,D-Penicillamine,Metalcaptase,D 3 Mercaptovaline,D Penicillamine,Penicillaminate, Copper,beta,beta Dimethylcysteine
D003300	Copper	A heavy metal trace element with the atomic symbol Cu, atomic number 29, and atomic weight 63.55.	Copper-63,Copper 63
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000305	Adrenal Cortex Hormones	HORMONES produced by the ADRENAL CORTEX, including both steroid and peptide hormones. The major hormones produced are HYDROCORTISONE and ALDOSTERONE.	Adrenal Cortex Hormone,Corticoid,Corticoids,Corticosteroid,Corticosteroids,Cortex Hormone, Adrenal,Hormone, Adrenal Cortex,Hormones, Adrenal Cortex