title: MARS treatment for a patient presenting with acquired hepatic glutamine synthetase deficiency after orthotopic liver transplantation.
abstract: We report a 48-year-old man presenting with refractory hyperammonemia after orthotopic liver transplantation. On investigation he was found to have acquired hepatic glutamine synthetase deficiency, a rare condition that occurs after organ transplantations. The patient was started on continuous venovenous hemofiltration treatment, but the hyperammonemia did not respond. The patient was then subjected to molecular adsorption recirculation system (MARS) therapy and the ammonia level gradually improved with successive treatments. In conclusion, the response was unlikely due to the hemofiltration component of MARS alone but more probably due to the removal of putative albumin-bound toxin by the adsorption circuit that had ameliorated the internal milieu of the graft and reversed the enzyme deficiency.
authors.affiliation: Intensive Care Unit, Queen Mary Hospital, Hong Kong, China.
authors.forename: Alexander
authors.initials: A
authors.lastname: Chiu
authors.forename: Sidney
authors.initials: S
authors.lastname: Tam
authors.forename: Wing Yan
authors.initials: WY
authors.lastname: Au
authors.forename: See Ching
authors.initials: SC
authors.lastname: Chan
authors.forename: Chi Leung
authors.initials: CL
authors.lastname: Liu
authors.forename: Sheung Tat
authors.initials: ST
authors.lastname: Fan
mesh_terms: D001663:Bilirubin / Q000097:blood; D003677:Deficiency Diseases / Q000209:etiology*; D005974:Glutamate-Ammonia Ligase / Q000172:deficiency*; D006440:Hemofiltration; D006801:Humans; D008099:Liver / Q000201:enzymology*; D016031:Liver Transplantation / Q000009:adverse effects*; D008297:Male; D008875:Middle Aged; D012016:Reference Values
chemical_list: D005974:Glutamate-Ammonia Ligase; D001663:Bilirubin