MSUD Nutrition Management Guidelines
First Edition
February 2013, v.1.50
Current version: v.1.58
Updated: August 2016
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Analyzed Literature
ID Reference
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Hilliges, C., Awiszus, D., & Wendel, U. (Feb 1993). Intellectual performance of children with maple syrup urine disease. Eur. J. Pediatr., 152(2):144-7.
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Koga, Y., Iwanaga, T., Yoshida, I., Yoshino, M., Kaneko, S., & Kato, H. (Apr 1998). Maple syrup urine disease: nutritional management by intravenous hyperalimentation and uneventful course after surgical repair of dislocation of the hip. J. Inherit. Metab. Dis., 21(2):177-8.
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Korein, J., Sansaricq, C., Kalmijn, M., Honig, J., & Lange, B. (Nov 1994). Maple syrup urine disease: clinical, EEG, and plasma amino acid correlations with a theoretical mechanism of acute neurotoxicity. Int. J. Neurosci., 79(1-2):21-45.
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Lee, J.Y., Chiong, M.A., Estrada, S.C., Cutiongco-De la Paz, E.M., Silao, C.L., & Padilla, C.D. (Dec 2008). Maple syrup urine disease (MSUD)--clinical profile of 47 Filipino patients. J. Inherit. Metab. Dis., 31 Suppl 2, S281-5. doi:10.1007/s10545-008-0859-0
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Lin, M.C., Chen, C.H., Fu, L.S., Jan, S.L., Shu, S.G., & Chi, C.S. (2002 Sep-Oct). Management of acute decompensation of neonatal maple syrup urine disease with continuous arteriovenous haemofiltration: report of one case. Acta Paediatr Taiwan, 43(5):281-4.
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Morton, D.H., Strauss, K.A. (2005, Summer). Liver transplant as treatment for maple syrup disease: our perspective as pediatricians. MSUD Support Newsletter, 22(2). Retrieved from
Morton, D.H., Strauss, K.A., Robinson, D.L., Puffenberger, E.G., & Kelley, R.I. (Jun 2002). Diagnosis and treatment of maple syrup disease: a study of 36 patients. Pediatrics, 109(6):999-1008.
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