Respiratory insufficiency in acid maltase deficiency: the effect of high protein diet.
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.
Demey
H E
HE
Department of Intensive Care, University of Antwerp UIA, University Hospital, Belgium.
Van Meerbeeck
J P
JP
Vandewoude
M F
MF
Prové
A M
AM
Martin
J J
JJ
Bossaert
L L
LL
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
D004044:Dietary Proteins; D000520:alpha-Glucosidases; D005087:Glucan 1,4-alpha-Glucosidase