Brief Report: Branched-Chain Amino Acid-Free Parenteral Nutrition in the Treatment of Acute Metabolic Decompensation in Patients with Maple Syrup Urine Disease

• Published in: The New England journal of medicine Vol. 324; no. 3; p. 175
• Main Authors: Berry, Gerard T, Heidenreich, Randall, Kaplan, Paige, Levine, Frederick, Mazur, Alice, Palmieri, Michael J, Yudkoff, Marc, Segal, Stanton
• Format: Journal Article
• Language: English
• Published: Boston Massachusetts Medical Society 17.01.1991
• Subjects: Acidosis; Age; Amino acids; Anorexia; Children & youth; Disease; Enzymes; Glucose; Hospitals; Infections; Insulin; Maple syrup urine disease; Metabolism; Nutrition research; Parenteral nutrition; Patients; Peritoneal dialysis; Plasma; Proteins; Urine
• DOI: 10.1056/NEJM199101173240307
• ISSN: 0028-4793

Despite mechanical difficulty with the central venous catheter during the next 9 hours and the fact that the infant had received only 176 kJ (42 kcal) per kilogram per 24 hours and 0.78 g of amino acids per kilogram per 24 hours, the plasma leucine concentration was 1.77 mmol per liter when modified parenteral nutrition was terminated after 11 1/2 hours. Because of hyperglycemia, insulin was administered during the nine-hour period. The plasma leucine level was 1.05 mmol per liter. Since vomiting had ceased shortly after admission, the child was fed maple syrup urine disease formula orally. Because of recurrent vomiting and the development of metabolic acidosis, the formula was discontinued, and calories (502 kJ [120 kcal] per kilogram per 24 hours) were supplied by intravenous glucose and lipid. Important observations in this regard include the following: the beneficial effect of modified parenteral nutrition on the correction of hyperleucinemia ceased or waned if levels of valine or isoleucine became subnormal3; the rate of nitrogen retention in the neonate treated with modified parenteral nutrition by Townsend and Kerr matched the published rates of protein accretion in infants of a similar age3; and even the administration of calories at a rate in excess of the Recommended Dietary Allowance was not sufficient to correct hyperleucinemia in our Patient 3 in the absence of exogenous amino acids.