Concepedia

Treatment of acute decompensation of maple syrup urine disease in adult patients with a new parenteral amino‐acid mixture

Aude Servais, Jean Baptiste Arnoux, Catherine Lamy, Aurélie Hummel, Nathalie Vittoz, I. Katerinis, V. Bazzaoui, Sandrine Dubois, Christine Broissand, Marie‐Caroline Husson,

Journal of Inherited Metabolic Disease · 2012 · 21 citations · 6 references

DOI

Abstract

This new parenteral amino-acid mixture is safe and allows efficient Leu concentration decrease during acute MSUD decompensation episodes in adults. Its use avoids the need for nasogastric tube insertion.

References

6

Kinetics of Neutral Amino Acid Transport Across the Blood‐Brain Barrier

Quentin R. Smith, Seiji Momma, Masaki Aoyagi et al. · Journal of Neurochemistry · 1987 · 450 citations

Classical maple syrup urine disease and brain development: Principles of management and formula design

Kevin A. Strauss, Bridget Wardley, Christine Hendrickson et al. · Molecular Genetics and Metabolism · 2009 · 191 citations · Full text

, , +7

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

Gerard T. Berry, Randall A. Heidenreich, Paige Kaplan et al. · New England Journal of Medicine · 1991 · 56 citations · Full text

Treatment of the Acute Crisis in Maple Syrup Urine Disease

William L. Nyhan, M Rice-Kelts, Bruce A. Barshop · Archives of Pediatrics and Adolescent Medicine · 1998 · 40 citations · Full text

Total parenteral nutrition therapy of toxic maple syrup urine disease

Irene R. Townsend, DS Kerr · American Journal of Clinical Nutrition · 1982 · 12 citations

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