Concentrations of isoleucine fell to levels that made this amino acid limiting for protein synthesis and hence therapeutic effect. Rates of fall were comparable to those obtained with intravenous therapy. Results In each instance, a progressive fall in leucine concentration was obtained. Clinical status closely mirrored the concentration of leucine. Main Outcome Measures Effects on the concentrations of leucine and the other branched-chain amino acids. Four different mixtures were used and a fifth was designed on the basis of this experience. Intervention Studies were carried out for 4 to 11 days, during which there was no intake of leucine. Data were collected during the management of 3 episodes of metabolic imbalance. Patients Two patients with maple syrup urine disease. They were designed to be used in the management of the acute crisis. Needs for water and calories were to be met intravenously. Objective To develop enteral mixtures suitable for administration by nasogastric drip in minimal volume.ĭesign Mixtures of amino acids were designed containing no leucine, isoleucine, or valine for administration by nasogastric drip. However, these intravenous mixtures are not generally available. Experience with intravenous mixtures of amino acids indicates that this can be accomplished by the synthetic forces of protein synthesis. Shared Decision Making and Communicationīackground The acute crisis of metabolic decompensation in maple syrup urine disease is a potentially lethal medical emergency that requires reduction in concentrations of leucine and other branched-chain amino acids in plasma.Scientific Discovery and the Future of Medicine.Health Care Economics, Insurance, Payment.Clinical Implications of Basic Neuroscience. Challenges in Clinical Electrocardiography.
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