PMID: 2112630Mar 1, 1990Paper

Treatment of acid maltase deficiency with a diet high in branched-chain amino acids

JPEN. Journal of Parenteral and Enteral Nutrition
S MobarhanH Friedman

Abstract

Acid maltase deficiency in adults is associated with progressive muscle weakness and may effect respiratory muscles resulting in respiratory failure. The biochemical and clinical manifestations of acid maltase deficiency arise from a marked deficiency of the lysosomal enzyme alpha-glucosidase (acid maltase), which normally degrades glycogen to free glucose. In the past few years, high-protein diets have provided an alternative energy source for these patients and resulted in improved muscle strength. Recently, we treated a ventilator-dependent acid maltase-deficient patient with a general diet supplemented with branched-chain amino acids. Branched-chain amino acids are the principal amino acids involved in muscle protein synthesis and utilization. While on this diet, the patient had improvement of respiratory function and muscle strength and was able to be weaned from the ventilator during the day. In addition to his nutritional status, levels of serum branched-chain amino acids, showed improvement within 2 months after the diet started. This diet shows potential advantages over a high-protein diet without supplemented branched-chain amino acids for the treatment of acid maltase deficiency. These include theoretical sparing of ...Continue Reading

Citations

Sep 1, 2005·Journal of Neurology·Léon P F WinkelAns T van der Ploeg
Mar 14, 2007·Journal of Neurology·Marloes L C HagemansAns T van der Ploeg
Mar 3, 2010·Cellular and Molecular Life Sciences : CMLS·J M de VriesP A van Doorn
Jan 19, 2012·American Journal of Medical Genetics. Part C, Seminars in Medical Genetics·Angela SchüllerBenedikt Schoser
Jun 1, 1991·Journal of the American College of Nutrition·R Benya, S Mobarhan
May 1, 1991·Nutrition Reviews·S Mobarhan, L S Trumbore
May 7, 2021·Communications Biology·Jason WangNenad Bursac

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