title: Respiratory insufficiency in acid maltase deficiency: the effect of high protein diet.
abstract: A 27-yr-old woman with the myopathic form of acid maltase deficiency (AMD) developed severe respiratory insufficiency after a crash diet resulting in a 6-kg weight loss. While being maintained on home ventilation, an hypercaloric high-protein, low-carbohydrate diet (1800-2000 cal; 28% carbohydrates, 55% fat, 17% protein with 1.7 g protein/kg body weight) was instituted. This ameliorated her condition up to a level where useful life was possible and ventilation could be diminished.
authors.affiliation: Department of Intensive Care, University of Antwerp UIA, University Hospital, Belgium.
authors.forename: H E
authors.initials: HE
authors.lastname: Demey
authors.forename: J P
authors.initials: JP
authors.lastname: Van Meerbeeck
authors.forename: M F
authors.initials: MF
authors.lastname: Vandewoude
authors.forename: A M
authors.initials: AM
authors.lastname: Prové
authors.forename: J J
authors.initials: JJ
authors.lastname: Martin
authors.forename: L L
authors.initials: LL
authors.lastname: Bossaert
mesh_terms: D000328:Adult; D004033:Diet Fads / Q000009:adverse effects*; D004044:Dietary Proteins / Q000008:administration & dosage*; D005260:Female; D005087:Glucan 1,4-alpha-Glucosidase / Q000172:deficiency*; D006009:Glycogen Storage Disease Type II / Q000628:therapy*; D006801:Humans; D012131:Respiratory Insufficiency / Q000209:etiology*; D000520:alpha-Glucosidases
chemical_list: D004044:Dietary Proteins; D000520:alpha-Glucosidases; D005087:Glucan 1,4-alpha-Glucosidase