title: Management of maple syrup urine disease in the peri-operative period.
abstract: Maple syrup urine disease (MSUD) has an incidence of 1:125,000 newborns in Ireland. Patients, when fasting, or in a catabolic state build up toxic metabolites leading to progressive neurological dysfunction. We describe the necessary peri-operative management of a patient with MSUD who developed symptomatic gallstones requiring a laparoscopic cholecystectomy.
authors.affiliation: Trinity Centre, St James's Hospital, Dublin.
authors.forename: E
authors.initials: E
authors.lastname: McCarron
authors.affiliation: Trinity Centre, St James's Hospital, Dublin.
authors.forename: O
authors.initials: O
authors.lastname: McCormack
authors.affiliation: Trinity Centre, St James's Hospital, Dublin.
authors.forename: T
authors.initials: T
authors.lastname: Cronin
authors.affiliation: Trinity Centre, St James's Hospital, Dublin.
authors.forename: A
authors.initials: A
authors.lastname: McGowan
authors.affiliation: Trinity Centre, St James's Hospital, Dublin.
authors.forename: M L
authors.initials: ML
authors.lastname: Healy
authors.affiliation: Trinity Centre, St James's Hospital, Dublin.
authors.forename: D
authors.initials: D
authors.lastname: O'Rourke
authors.affiliation: Trinity Centre, St James's Hospital, Dublin.
authors.forename: E
authors.initials: E
authors.lastname: Crushell
authors.affiliation: Trinity Centre, St James's Hospital, Dublin.
authors.forename: N
authors.initials: N
authors.lastname: Ravi
authors.affiliation: Trinity Centre, St James's Hospital, Dublin.
authors.forename: J V
authors.initials: JV
authors.lastname: Reynolds
mesh_terms: D049448:Cholangiopancreatography, Magnetic Resonance; D017081:Cholecystectomy, Laparoscopic; D005260:Female; D042882:Gallstones / Q000175:diagnosis* / Q000209:etiology* / Q000601:surgery*; D006801:Humans; D008111:Liver Function Tests; D008375:Maple Syrup Urine Disease / Q000150:complications*; D055815:Young Adult