title: Refeeding syndrome in a young woman with argininosuccinate lyase deficiency.
abstract: A severely chronically protein and calorie restricted young woman with argininosuccinate lyase deficiency developed transient refeeding syndrome (RFS) and hyperammonemia after modest diet liberalization following initiation of glycerol phenylbutyrate (GPB). The patient required IV supportive care and supplementation with potassium, magnesium and calcium. She is now doing well on GPB and an appropriate maintenance diet. Susceptibility to RFS should be considered in chronically nutritionally restricted patients with metabolic disorders after liberalization of diet.
authors.affiliation: Department of Medical and Molecular Genetics, Division of Clinical and Biochemical Genetics, Indiana University School of Medicine, Indianapolis, IN, United States.
authors.forename: M
authors.initials: M
authors.lastname: Stuy
authors.affiliation: Hyperion Therapeutics, Inc., Brisbane, CA, United States.
authors.forename: G-F
authors.initials: GF
authors.lastname: Chen
authors.affiliation: Hyperion Therapeutics, Inc., Brisbane, CA, United States.
authors.forename: J M
authors.initials: JM
authors.lastname: Masonek
authors.affiliation: Hyperion Therapeutics, Inc., Brisbane, CA, United States.
authors.forename: B F
authors.initials: BF
authors.lastname: Scharschmidt
keywords: Argininosuccinate lyase deficiency; Glycerol phenylbutyrate; Hyperammonemia; Refeeding syndrome