title: An unusual case of severe high anion gap metabolic acidosis.
abstract: We present a case of high anion gap metabolic acidosis with an unusual aetiology in a 75-year-old lady with hypoglycaemia, encephalopathy and relatively preserved renal function. Full toxicology and biochemical analysis suggested that she had an inborn error of metabolism, riboflavin-responsive multiple acyl-CoA dehydrogenase deficiency that can predispose to severe acidosis in situations where calorific intake is reduced. We believe this to be one of the few published cases and is remarkable for the presentation in late adulthood in addition to the requirement for emergency haemodialysis due to the severity of the metabolic disturbance.
authors.affiliation: Department of Renal Medicine, Royal Derby Hospital, Derby, UK.
authors.forename: Riaz V
authors.initials: RV
authors.lastname: Bavakunji
authors.affiliation: Department of Renal Medicine, Royal Derby Hospital, Derby, UK.
authors.forename: Jake D
authors.initials: JD
authors.lastname: Turner
authors.affiliation: Department of Renal Medicine, Royal Derby Hospital, Derby, UK.
authors.forename: Sagar
authors.initials: S
authors.lastname: Jujjavarapu
authors.affiliation: Department of Renal Medicine, Royal Derby Hospital, Derby, UK.
authors.forename: Maarten W
authors.initials: MW
authors.lastname: Taal
authors.affiliation: Department of Renal Medicine, Royal Derby Hospital, Derby, UK.
authors.forename: Richard J
authors.initials: RJ
authors.lastname: Fluck
authors.affiliation: Department of Renal Medicine, Royal Derby Hospital, Derby, UK.
authors.forename: Janson C
authors.initials: JC
authors.lastname: Leung
authors.affiliation: Department of Renal Medicine, Royal Derby Hospital, Derby, UK.
authors.forename: Nitin V
authors.initials: NV
authors.lastname: Kolhe
authors.affiliation: Department of Renal Medicine, Royal Derby Hospital, Derby, UK.
authors.forename: Nicholas M
authors.initials: NM
authors.lastname: Selby
keywords: acyl carnitine; fatty acid metabolism; metabolic acidosis; riboflavin-responsive multiple acylCoA dehydrogenase deficiency