Biomaterial Database

Hepatobiliary pathology in patients with cystic fibrosis. 1989

M Sinaasappel

Department of Paediatrics, University Hospital/Sophia Children's Hospital, Rotterdam, The Netherlands.

Overt liver disease in cystic fibrosis is a rare condition. Only 1-5% of all patients show a severe disturbance of the liver cell function or portal hypertension. In contrast, liver architecture is much more often disturbed at post mortem examination. The experience is that liver pathology increases with age which will result in rising numbers of patients in the future parallel to the increasing life expectancy of the patients. Bile plugs are commonly found in the portal tract and probably represent the essential abnormality of the liver in CF. Recently new methods have been developed for the investigation of the bile synthesis which will be helpful in the understanding of the CF defect in the liver.

UI	MeSH Term	Description	Entries
D008107	Liver Diseases	Pathological processes of the LIVER.	Liver Dysfunction,Disease, Liver,Diseases, Liver,Dysfunction, Liver,Dysfunctions, Liver,Liver Disease,Liver Dysfunctions
D003550	Cystic Fibrosis	An autosomal recessive genetic disease of the EXOCRINE GLANDS. It is caused by mutations in the gene encoding the CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR expressed in several organs including the LUNG, the PANCREAS, the BILIARY SYSTEM, and the SWEAT GLANDS. Cystic fibrosis is characterized by epithelial secretory dysfunction associated with ductal obstruction resulting in AIRWAY OBSTRUCTION; chronic RESPIRATORY INFECTIONS; PANCREATIC INSUFFICIENCY; maldigestion; salt depletion; and HEAT PROSTRATION.	Mucoviscidosis,Cystic Fibrosis of Pancreas,Fibrocystic Disease of Pancreas,Pancreatic Cystic Fibrosis,Pulmonary Cystic Fibrosis,Cystic Fibrosis, Pancreatic,Cystic Fibrosis, Pulmonary,Fibrosis, Cystic,Pancreas Fibrocystic Disease,Pancreas Fibrocystic Diseases
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000367	Age Factors	Age as a constituent element or influence contributing to the production of a result. It may be applicable to the cause or the effect of a circumstance. It is used with human or animal concepts but should be differentiated from AGING, a physiological process, and TIME FACTORS which refers only to the passage of time.	Age Reporting,Age Factor,Factor, Age,Factors, Age
D001659	Biliary Tract	The BILE DUCTS and the GALLBLADDER.	Biliary System,Biliary Tree,System, Biliary,Tract, Biliary,Tree, Biliary