Respiratory insufficiency in acid maltase deficiency: the effect of high protein diet.

Author: Demey HE, Van Meerbeeck JP, Vandewoude MF, Prove AM, Martin JJ, Bossaert LL

Author affiliation: Department of Intensive Care, University of Antwerp UIA, University Hospital, Belgium.

Publication date & source: 1989.05, JPEN J Parenter Enteral Nutr., 13(3):321-3.

Publication type: Case Reports

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.