Biomaterial Database

Ursodeoxycholic acid improves cholestasis in infants with cystic fibrosis. 1997

H Scher, and W P Bishop, and P B McCray

Department of Pediatrics, College of Medicine, University of Iowa, Iowa City 52242, USA.

OBJECTIVE To describe two infants with cholestatic jaundice treated with ursodeoxycholic acid (UDCA). METHODS Two infants with cystic fibrosis (CF)-associated hepatobiliary disease, manifesting as cholestatic jaundice and elevated liver enzymes within the first 6 weeks of life, had improved biochemical indices of liver function following treatment with UDCA 20-40 mg/kg/d. CONCLUSIONS To our knowledge, this is the first report of UDCA treatment in infants with CF-associated cholestatic jaundice. Infants and children require treatment with increased doses of UDCA to compensate for reduced intestinal absorption of bile acid and immaturity of the enterohepatic circulation. CONCLUSIONS UDCA appears to be a cost-effective treatment for CF-associated hepatobiliary disease in infants and children.

UI	MeSH Term	Description	Entries
D007231	Infant, Newborn	An infant during the first 28 days after birth.	Neonate,Newborns,Infants, Newborn,Neonates,Newborn,Newborn Infant,Newborn Infants
D008111	Liver Function Tests	Blood tests that are used to evaluate how well a patient's liver is working and also to help diagnose liver conditions.	Function Test, Liver,Function Tests, Liver,Liver Function Test,Test, Liver Function,Tests, Liver Function
D008297	Male		Males
D002756	Cholagogues and Choleretics	Gastrointestinal agents that stimulate the flow of bile into the duodenum (cholagogues) or stimulate the production of bile by the liver (choleretic).	Choleretics,Cholagogues,Cholagogues, Choleretics,Choleretics and Cholagogues,Hydrocholeretics
D002779	Cholestasis	Impairment of bile flow due to obstruction in small bile ducts (INTRAHEPATIC CHOLESTASIS) or obstruction in large bile ducts (EXTRAHEPATIC CHOLESTASIS).	Bile Duct Obstruction,Biliary Stasis,Bile Duct Obstructions,Biliary Stases,Cholestases,Duct Obstruction, Bile,Duct Obstructions, Bile,Obstruction, Bile Duct,Obstructions, Bile Duct,Stases, Biliary,Stasis, Biliary
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
D005260	Female		Females
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D014580	Ursodeoxycholic Acid	An epimer of chenodeoxycholic acid. It is a mammalian bile acid found first in the bear and is apparently either a precursor or a product of chenodeoxycholate. Its administration changes the composition of bile and may dissolve gallstones. It is used as a cholagogue and choleretic.	Deoxyursocholic Acid,3 alpha,7 beta-Dihydroxy-5 beta-cholan-24-oic Acid,Cholit-Ursan,Cholofalk,Delursan,Destolit,Sodium Ursodeoxycholate,Urdox,Ursacholic Acid,Urso,Urso Heumann,Ursobilane,Ursochol,Ursodiol,Ursofalk,Ursogal,Ursolite,Ursolvan,3 alpha,7 beta Dihydroxy 5 beta cholan 24 oic Acid,Acid, Deoxyursocholic,Acid, Ursacholic,Acid, Ursodeoxycholic,Ursodeoxycholate, Sodium
D016896	Treatment Outcome	Evaluation undertaken to assess the results or consequences of management and procedures used in combating disease in order to determine the efficacy, effectiveness, safety, and practicability of these interventions in individual cases or series.	Rehabilitation Outcome,Treatment Effectiveness,Clinical Effectiveness,Clinical Efficacy,Patient-Relevant Outcome,Treatment Efficacy,Effectiveness, Clinical,Effectiveness, Treatment,Efficacy, Clinical,Efficacy, Treatment,Outcome, Patient-Relevant,Outcome, Rehabilitation,Outcome, Treatment,Outcomes, Patient-Relevant,Patient Relevant Outcome,Patient-Relevant Outcomes