Protein turnover in acid maltase deficiency before and after treatment with a high protein diet.
A patient with acid maltase deficiency was treated with a high protein diet for 7 months. Protein turnover expressed in terms of lean body mass was shown to be increased in this patient before the diet but was markedly reduced following the diet. The patient improved clinically whilst on the diet both subjectively and in terms of mobility, breathing and reduced peripheral cyanosis at rest.
D000328:Adult; D004044:Dietary Proteins / Q000008:administration & dosage*; D005087:Glucan 1,4-alpha-Glucosidase / Q000172:deficiency*; D005951:Glucose Tolerance Test; D005959:Glucosidases / Q000172:deficiency*; D006008:Glycogen Storage Disease / Q000178:diet therapy*; D006009:Glycogen Storage Disease Type II / Q000178:diet therapy* / Q000201:enzymology; D006801:Humans; D007930:Leucine / Q000097:blood; D008297:Male; D009124:Muscle Proteins / Q000378:metabolism*; D000520:alpha-Glucosidases
D004044:Dietary Proteins; D009124:Muscle Proteins; D005959:Glucosidases; D000520:alpha-Glucosidases; D005087:Glucan 1,4-alpha-Glucosidase; D007930:Leucine