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: H E Demey, J P Van Meerbeeck, M F Vandewoude, A M Prové, J J Martin, L L Bossaert
• Journal: JPEN. Journal of parenteral and enteral nutrition (JPEN J Parenter Enteral Nutr) 1989 May-Jun Vol. 13 Issue 3 Pg. 321-3 ISSN: 0148-6071 [Print] United States
• PMID: 2503645 (Publication Type: Case Reports, Journal Article)

Chemical References

• Dietary Proteins
• alpha-Glucosidases
• Glucan 1,4-alpha-Glucosidase

Topics

• Adult
• Diet Fads (adverse effects)
• Dietary Proteins (administration & dosage)
• Female
• Glucan 1,4-alpha-Glucosidase (deficiency)
• Glycogen Storage Disease Type II (therapy)
• Humans
• Respiratory Insufficiency (etiology)
• alpha-Glucosidases