title: Anaesthesia for liver transplantation in two infants with an organic acidaemia.
abstract: Liver transplantation is an accepted option of treatment in patients with inborn errors of metabolism limited to or mainly located into hepatocytes who have not responded well to medical treatment. Recurrent metabolic failure and neurological impairment might be an indication for early transplantation in patients with organic acidaemias. We discuss the anaesthetic management and metabolic implications of acidaemia in the first two cases of successfully treated propionic and methylmalonic acidaemia in Italy. A nine and 12 month follow up did not show any further metabolic failure after the procedure, indicating that early liver transplantation improves the quality of life of these patients.
authors.affiliation: Department of Anaesthesia and Intensive Care 1, Ospedali Riuniti di Bergamo, Bergamo, Italy. diegomanzoni@libero.it
authors.forename: Diego
authors.initials: D
authors.lastname: Manzoni
authors.forename: Angelica
authors.initials: A
authors.lastname: Spotti
authors.forename: Bruno
authors.initials: B
authors.lastname: Carrara
authors.forename: Paolo
authors.initials: P
authors.lastname: Gritti
authors.forename: Valter
authors.initials: V
authors.lastname: Sonzogni
mesh_terms: D000758:Anesthesia / Q000379:methods*; D005260:Female; D006801:Humans; D007223:Infant; D016031:Liver Transplantation; D008661:Metabolism, Inborn Errors / Q000601:surgery*; D008764:Methylmalonic Acid / Q000097:blood; D011422:Propionates / Q000097:blood; D011788:Quality of Life
chemical_list: D011422:Propionates; D008764:Methylmalonic Acid; C029658:propionic acid