title: Metabolic liver disease.
abstract: Diagnosis of metabolic liver disease requires a high level of diagnostic suspicion. Diet is usually the primary treatment for metabolic liver disease. Where indicated, liver transplantation provides lifelong functional correction of liver-based metabolic defects. Liver cell therapy warrants further study for the future treatment of metabolic liver disease. All families should receive genetic advice and pre-emptive management of future affected siblings.
authors.affiliation: Liver Unit, Birmingham Children's Hospital, Birmingham, United Kingdom. Pat.Mckiernan@bch.nhs.uk
authors.forename: Pat
authors.initials: P
authors.lastname: McKiernan
mesh_terms: D017485:1-Deoxynojirimycin / Q000031:analogs & derivatives / Q000627:therapeutic use; D003512:Cyclohexanones / Q000627:therapeutic use; D004032:Diet; D004791:Enzyme Inhibitors / Q000627:therapeutic use; D056947:Enzyme Replacement Therapy; D022781:Hepatocytes / Q000637:transplantation; D006529:Hepatomegaly / Q000209:etiology; D006801:Humans; D015160:Hydrops Fetalis; D007223:Infant; D007231:Infant, Newborn; D007567:Jaundice, Neonatal / Q000150:complications; D008107:Liver Diseases / Q000209:etiology* / Q000628:therapy*; D016031:Liver Transplantation; D008661:Metabolism, Inborn Errors / Q000150:complications* / Q000628:therapy; D009579:Nitrobenzoates / Q000627:therapeutic use; D013163:Splenomegaly / Q000209:etiology
chemical_list: D003512:Cyclohexanones; D004791:Enzyme Inhibitors; D009579:Nitrobenzoates; D017485:1-Deoxynojirimycin; C059896:miglustat; C077073:nitisinone