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MSUD Nutrition Management Guidelines
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February 2013, v.1.46
Current version: v.1.58
Updated: May 2014
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References

Analyzed Literature

ID Reference
G.39
Acosta, P.B., Yannicelli, S. (2001). Maple Syrup Urine Disease (MSUD). In The Ross Metabolic Formula System: Nutrition support Protocols, 4th Edition (pp. 74-102). Columbus, OH: Ross Products Division, Division of Abbott Laboratories.
F.38
Barshop, B.A., & Khanna, A. (Dec 2005). Domino hepatic transplantation in maple syrup urine disease. The New England journal of medicine, 353, 2410-1. doi:10.1056/NEJM200512013532220
G.23
Berry, G.T. (2008, Fall). Medical Management of MSU. MSUD Family Newsletter, 25(2). Retrieved from http://www.msud-support.org/index.php?view=category&id=46%3Avolume-25-2&option=com_content&Itemid=5
F.615
Berry, G.T., Heidenreich, R., Kaplan, P., Levine, F., Mazur, A., Palmieri, M.J., Yudkoff, M., & Segal, S. (Jan 1991). Branched-chain amino acid-free parenteral nutrition in the treatment of acute metabolic decompensation in patients with maple syrup urine disease. The New England journal of medicine, 324, 175-9. doi:10.1056/NEJM199101173240307
F.124
Biggemann, B., Zass, R., & Wendel, U. (1993). Postoperative metabolic decompensation in maple syrup urine disease is completely prevented by insulin. Journal of inherited metabolic disease, 16(5):912-3.
Retrieved from http://www.ncbi.nlm.nih.gov/pubmed/8295418
G.48
Bodamer, O.A. (2010). Overview of Maple Syrup Urine Disease. Retrieved from UpToDate, Inc website www.uptodate.com
F.53
Bodner-Leidecker, A., Wendel, U., Saudubray, J.M., & Schadewaldt, P. (Dec 2000). Branched-chain L-amino acid metabolism in classical maple syrup urine disease after orthotopic liver transplantation. Journal of inherited metabolic disease, 23(8):805-18.
Retrieved from http://www.ncbi.nlm.nih.gov/pubmed/11196106
G.44
Children's Hospital of Michigan Metabolic Clinic (CHMMC), Division of Genetic and Metabolic Disorders. (2008). Maple Syrup Urine Disease Emergency Care Sick Plan. Detroit, MI: University of Michigan.
G.49
Chuang, D.T., Wynn, R.M., Shih, V.E. (2008). Maple Syrup Urine Disease (Branched-Chain Ketoaciduria). In Valle et al, The Online Metabolic and Molecular Basis of Inherited Disease (Chapter 87 pp.1-42). The McGraw-Hill Companies, Inc. 
F.114
Danner, D.J., Wheeler, F.B., Lemmon, S.K., & Elsas, L.J. (Mar 1978). In vivo and in vitro response of human branched chain alpha-ketoacid dehydrogenase to thiamine and thiamine pyrophosphate. Pediatric research, 12, 235-8. doi:10.1203/00006450-197803000-00016
F.83
Dixon, M.A., & Leonard, J.V. (Nov 1992). Intercurrent illness in inborn errors of intermediary metabolism. Archives of disease in childhood, 67(11):1387-91.
Retrieved from http://www.ncbi.nlm.nih.gov/pubmed/1471895
F.127
Duran, M., & Wadman, S.K. (1985). Thiamine-responsive inborn errors of metabolism. Journal of inherited metabolic disease, 8 Suppl 1:70-5.
Retrieved from http://www.ncbi.nlm.nih.gov/pubmed/3930844
F.113
Duran, M., Tielens, A.G., Wadman, S.K., Stigter, J.C., & Kleijer, W.J. (May 1978). Effects of thiamine in a patient with a variant form of branched-chian ketoaciduria. Acta paediatrica Scandinavica, 67(3):367-72.
Retrieved from http://www.ncbi.nlm.nih.gov/pubmed/654914
F.82
Ellerine, N.P., Herring, W.J., Elsas, L.J., McKean, M.C., Klein, P.D., & Danner, D.J. (Jun 1993). Thiamin-responsive maple syrup urine disease in a patient antigenically missing dihydrolipoamide acyltransferase. Biochemical medicine and metabolic biology, 49(3):363-74.
Retrieved from http://www.ncbi.nlm.nih.gov/pubmed/8347380
F.101
Elsas, L.J., Ellerine, N.P., & Klein, P.D. (May 1993). Practical methods to estimate whole body leucine oxidation in maple syrup urine disease. Pediatric research, 33, 445-51. doi:10.1203/00006450-199305000-00005
F.109
Fernhoff, P.M., Lubitz, D., Danner, D.J., Dembure, P.P., Schwartz, H.P., Hillman, R., Bier, D.M., & Elsas, L.J. (Oct 1985). Thiamine response in maple syrup urine disease. Pediatric research, 19, 1011-6. doi:10.1203/00006450-198510000-00012
F.29
Fuentes-Garcia, D., & Falcon-Arana, L. (Jan 2009). Perioperative management of a patient with maple syrup urine disease. British journal of anaesthesia, 102, 144-5. doi:10.1093/bja/aen341
F.100
Giacoia, G.P., & Berry, G.T. (Sep 1993). Acrodermatitis enteropathica-like syndrome secondary to isoleucine deficiency during treatment of maple syrup urine disease. American journal of diseases of children (1960), 147(9):954-6.
Retrieved from http://www.ncbi.nlm.nih.gov/pubmed/8362810
F.78
Grunewald, S., Hinrichs, F., & Wendel, U. (Apr 1998). Pregnancy in a woman with maple syrup urine disease. Journal of inherited metabolic disease, 21(2):89-94.
Retrieved from http://www.ncbi.nlm.nih.gov/pubmed/9584259
G.4
Hartnett, C. et al. (2003, July, 29). MSUD Infant/ Flax seed oil in MSUD/ Intermittent MSUD/ Leucine and Protein Comparison/ Ordering aminoacids. Message posted to Metabolic Dietician List,  <PNO-METAB-L@LISTSERV.CC.EMORY.EDU>
G.7
Hartnett, C. et al. (2009, January, 28). MSUD low leves/ 6 months MSUD/ ILE deficiency/ MSUD protocol for pregnancy/ Leucine blood spots. Message posted to Metabolic Dietician List,  <PNO-METAB-L@LISTSERV.CC.EMORY.EDU>
F.611
Heldt, K., Schwahn, B., Marquardt, I., Grotzke, M., & Wendel, U. (Apr 2005). Diagnosis of MSUD by newborn screening allows early intervention without extraneous detoxification. Molecular genetics and metabolism, 84, 313-6. doi:10.1016/j.ymgme.2004.11.010
F.80
Henriquez, H., el Din, A., Ozand, P.T., Subramanyam, S.B., & al Gain, S.I. (Nov 1994). Emergency presentations of patients with methylmalonic acidemia, propionic acidemia and branched chain amino acidemia (MSUD). Brain & development, 16 Suppl:86-93.
Retrieved from http://www.ncbi.nlm.nih.gov/pubmed/7726386
F.612
Hilliges, C., Awiszus, D., & Wendel, U. (Feb 1993). Intellectual performance of children with maple syrup urine disease. European journal of pediatrics, 152(2):144-7.
Retrieved from http://www.ncbi.nlm.nih.gov/pubmed/8444223
G.35
Hillman, R. (1993, Spring/Summer). Protein Malnutrition. MSUD Support Newsletter, 11(1). Retrieved from http://www.msud-support.org/index.php?option=com_content&view=article&id=33:protein-malnutrition&catid=8:volume-11-1&Itemid=5
F.45
Hmiel, S.P., Martin, R.A., Landt, M., Levy, F.H., & Grange, D.K. (May 2004). Amino acid clearance during acute metabolic decompensation in maple syrup urine disease treated with continuous venovenous hemodialysis with filtration. Pediatric critical care medicine : a journal of the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies, 5(3):278-81.
Retrieved from http://www.ncbi.nlm.nih.gov/pubmed/15115568
F.37
Hoffmann, B., Helbling, C., Schadewaldt, P., & Wendel, U. (Jan 2006). Impact of longitudinal plasma leucine levels on the intellectual outcome in patients with classic MSUD. Pediatric research, 59, 17-20. doi:10.1203/01.pdr.0000190571.60385.34
F.663
Huner, G., Baykal, T., Demir, F., & Demirkol, M. (2005). Breastfeeding experience in inborn errors of metabolism other than phenylketonuria. Journal of inherited metabolic disease, 28, 457-65. doi:10.1007/s10545-005-0457-3
F.63
Jan, D., Poggi, F., Laurent, J., Rabier, D., Jouvet, P., Lacaille, F., Beringer, A., Hubert, P., Revillon, Y., & Saudubray, J.M. (Feb 1994). Liver transplantation: new indications in metabolic disorders?. Transplantation proceedings, 26(1):189-90.
Retrieved from http://www.ncbi.nlm.nih.gov/pubmed/8108935
F.73
Jouvet, P., Jugie, M., Rabier, D., Desgres, J., Hubert, P., Saudubray, J.M., & Man, N.K. (Nov 2001). Combined nutritional support and continuous extracorporeal removal therapy in the severe acute phase of maple syrup urine disease. Intensive care medicine, 27, 1798-806. doi:10.1007/s00134-001-1124-2
F.79
Jouvet, P., Poggi, F., Rabier, D., Michel, J.L., Hubert, P., Sposito, M., Saudubray, J.M., & Man, N.K. (Aug 1997). Continuous venovenous haemodiafiltration in the acute phase of neonatal maple syrup urine disease. Journal of inherited metabolic disease, 20(4):463-72.
Retrieved from http://www.ncbi.nlm.nih.gov/pubmed/9266382
F.69
Kaplan, P., Mazur, A., Field, M., Berlin, J.A., Berry, G.T., Heidenreich, R., Yudkoff, M., & Segal, S. (Jul 1991). Intellectual outcome in children with maple syrup urine disease. The Journal of pediatrics, 119(1 Pt 1):46-50.
Retrieved from http://www.ncbi.nlm.nih.gov/pubmed/2066858
F.23
Khanna, A., Hart, M., Nyhan, W.L., Hassanein, T., Panyard-Davis, J., & Barshop, B.A. (May 2006). Domino liver transplantation in maple syrup urine disease. Liver transplantation : official publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society, 12, 876-82. doi:10.1002/lt.20744
F.68
Koch, S.E., Packman, S., Koch, T.K., & Williams, M.L. (Feb 1993). Dermatitis in treated maple syrup urine disease. Journal of the American Academy of Dermatology, 28(2 Pt 2):289-92.
Retrieved from http://www.ncbi.nlm.nih.gov/pubmed/8436640
F.77
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. Journal of inherited metabolic disease, 21(2):177-8.
Retrieved from http://www.ncbi.nlm.nih.gov/pubmed/9584272
F.614
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. The International journal of neuroscience, 79(1-2):21-45.
Retrieved from http://www.ncbi.nlm.nih.gov/pubmed/7744549
F.24
le Roux, C., Murphy, E., Hallam, P., Lilburn, M., Orlowska, D., & Lee, P. (Feb 2006). Neuropsychometric outcome predictors for adults with maple syrup urine disease. Journal of inherited metabolic disease, 29, 201-2. doi:10.1007/s10545-006-0223-1
F.26
le Roux, C., Murphy, E., Lilburn, M., & Lee, P.J. (Feb 2006). The longest-surviving patient with classical maple syrup urine disease. Journal of inherited metabolic disease, 29, 190-4. doi:10.1007/s10545-006-0204-4
F.20
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. Journal of inherited metabolic disease, 31 Suppl 2, S281-5. doi:10.1007/s10545-008-0859-0
F.616
Lee, P.J. (2006 Apr-Jun). Pregnancy issues in inherited metabolic disorders. Journal of inherited metabolic disease, 29, 311-6. doi:10.1007/s10545-005-0252-1
F.72
Levin, M.L., Scheimann, A., Lewis, R.A., & Beaudet, A.L. (Jan 1993). Cerebral edema in maple syrup urine disease. The Journal of pediatrics, 122(1):167-8.
Retrieved from http://www.ncbi.nlm.nih.gov/pubmed/8419609
F.93
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 paediatrica Taiwanica = Taiwan er ke yi xue hui za zhi, 43(5):281-4.
Retrieved from http://www.ncbi.nlm.nih.gov/pubmed/12607485
G.50
Marriage, B. (2010). Nutrition Management of Patients with Inherited Disorders of Branched- Chain Amino Acid Metabolism. In P.B. Acosta, Nutrition Management of Patients with Inherited Metabolic Disorders (pp. 175-236). Sudbury, MA: Jones and Bartlett Publishers.
F.105
McMahon, Y., & MacDonnell, R.C. (1990). Clearance of branched chain amino acids by peritoneal dialysis in maple syrup urine disease. Advances in peritoneal dialysis. Conference on Peritoneal Dialysis, 6:31-4.
Retrieved from http://www.ncbi.nlm.nih.gov/pubmed/1982835
G.31
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 http://www.msud-support.org/index.php?view=category&id=40%3Avolume-22-2&option=com_content&Itemid=5
F.51
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.
Retrieved from http://www.ncbi.nlm.nih.gov/pubmed/12042535
G.47
MSUD The New England Consortium of Metabolic Programs at Children’s Hospital Boston. (2006). Acute Illness Protocol. Boston, MA: Author.
G.82
New England Consortium. Acute Illness Protocol Organic Acidemias, Maple Syrup Urine Disease (MSUD).  Retrieved from http://newenglandconsortium.org/protocols/acute_illness/organic_acid_disorders/maple-syrup-urine-disease.pdf
F.67
Northrup, H., Sigman, E.S., & Hebert, A.A. (Mar 1993). Exfoliative erythroderma resulting from inadequate intake of branched-chain amino acids in infants with maple syrup urine disease. Archives of dermatology, 129(3):384-5.
Retrieved from http://www.ncbi.nlm.nih.gov/pubmed/8447687
F.76
Nyhan, W.L., Rice-Kelts, M., Klein, J., & Barshop, B.A. (Jun 1998). Treatment of the acute crisis in maple syrup urine disease. Archives of pediatrics & adolescent medicine, 152(6):593-8.
Retrieved from http://www.ncbi.nlm.nih.gov/pubmed/9641714
F.71
Ozturk, Y. (2008 May-Jun). Acrodermatitis enteropathica-like syndrome secondary to branched-chain amino acid deficiency in inborn errors of metabolism. Pediatric dermatology, 25, 415. doi:10.1111/j.1525-1470.2008.00707.x
F.81
Parini, R., Sereni, L.P., Bagozzi, D.C., Corbetta, C., Rabier, D., Narcy, C., Hubert, P., & Saudubray, J.M. (Aug 1993). Nasogastric drip feeding as the only treatment of neonatal maple syrup urine disease. Pediatrics, 92(2):280-3.
Retrieved from http://www.ncbi.nlm.nih.gov/pubmed/8337030
F.126
Parsons, H.G., Carter, R.J., Unrath, M., & Snyder, F.F. (1990). Evaluation of branched-chain amino acid intake in children with maple syrup urine disease and methylmalonic aciduria. Journal of inherited metabolic disease, 13(2):125-36.
Retrieved from http://www.ncbi.nlm.nih.gov/pubmed/2116544
F.52
Puliyanda, D.P., Harmon, W.E., Peterschmitt, M.J., Irons, M., & Somers, M.J. (Apr 2002). Utility of hemodialysis in maple syrup urine disease. Pediatric nephrology (Berlin, Germany), 17, 239-42. doi:10.1007/s00467-001-0801-2
F.47
Riazi, R., Rafii, M., Clarke, J.T., Wykes, L.J., Ball, R.O., & Pencharz, P.B. (Jul 2004). Total branched-chain amino acids requirement in patients with maple syrup urine disease by use of indicator amino acid oxidation with L-[1-13C]phenylalanine. American journal of physiology. Endocrinology and metabolism, 287, E142-9. doi:10.1152/ajpendo.00431.2003
F.87
Ring, E., Zobel, G., & Stockler, S. (Aug 1990). Clearance of toxic metabolites during therapy for inborn errors of metabolism. The Journal of pediatrics, 117(2 Pt 1):349-50.
Retrieved from http://www.ncbi.nlm.nih.gov/pubmed/2380843
F.85
Riviello, J.J., Rezvani, I., DiGeorge, A.M., & Foley, C.M. (Jul 1991). Cerebral edema causing death in children with maple syrup urine disease. The Journal of pediatrics, 119(1 Pt 1):42-5.
Retrieved from http://www.ncbi.nlm.nih.gov/pubmed/2066857
F.89
Ross, L.F. (Jan 2010). An ethical and policy analysis of elective transplantation for metabolic conditions diagnosed by newborn screening. The Journal of pediatrics, 156, 139-44. doi:10.1016/j.jpeds.2009.06.067
F.88
Rutledge, S.L., Havens, P.L., Haymond, M.W., McLean, R.H., Kan, J.S., & Brusilow, S.W. (Jan 1990). Neonatal hemodialysis: effective therapy for the encephalopathy of inborn errors of metabolism. The Journal of pediatrics, 116(1):125-8.
Retrieved from http://www.ncbi.nlm.nih.gov/pubmed/2295952
F.75
Schaefer, F., Straube, E., Oh, J., Mehls, O., & Mayatepek, E. (Apr 1999). Dialysis in neonates with inborn errors of metabolism. Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association, 14(4):910-8.
Retrieved from http://www.ncbi.nlm.nih.gov/pubmed/10328469
F.46
Schonberger, S., Schweiger, B., Schwahn, B., Schwarz, M., & Wendel, U. (May 2004). Dysmyelination in the brain of adolescents and young adults with maple syrup urine disease. Molecular genetics and metabolism, 82, 69-75. doi:10.1016/j.ymgme.2004.01.016
F.119
Scriver, C.R., Mackenzie, S., Clow, C.L., & Delvin, E. (Feb 1971). Thiamine-responsive maple-syrup-urine disease. Lancet, 1(7694):310-2.
Retrieved from http://www.ncbi.nlm.nih.gov/pubmed/4100151
F.422
Shellmer, D.A., DeVito Dabbs, A., Dew, M.A., Noll, R.B., Feldman, H., Strauss, K.A., Morton, D.H., Vockley, J., & Mazariegos, G.V. (Feb 2011). Cognitive and adaptive functioning after liver transplantation for maple syrup urine disease: a case series. Pediatric transplantation, 15, 58-64. doi:10.1111/j.1399-3046.2010.01411.x
F.21
Simon, E., Flaschker, N., Schadewaldt, P., Langenbeck, U., & Wendel, U. (Dec 2006). Variant maple syrup urine disease (MSUD)--the entire spectrum. Journal of inherited metabolic disease, 29, 716-24. doi:10.1007/s10545-006-0276-1
F.91
Simon, E., Wendel, U., & Schadewaldt, P. (2005 Jan-Mar). Maple syrup urine disease-treatment and outcome in patients of Turkish descent in Germany. The Turkish journal of pediatrics, 47(1):8-13.
Retrieved from http://www.ncbi.nlm.nih.gov/pubmed/15884622
G.33
Singh, R. (1994, Fall/Winter). Update of Nutrition Management of MSUD. MSUD Support Newsletter, 12(2). Retrieved from http://www.msud-support.org/index.php?option=com_content&view=article&id=64:update-of-nutrition-management-of-msud&catid=15:volume-12-2&Itemid=5
G.26
Singh, R.H. (1999, April). Advances in Nutrition Management of Patients with MSUD [PowerPoint slides]. Paper presented at the 7th Abbott Nutrition Advances in Management of Inherited Metabolic Disorders. Washington, District of Columbia.
F.65
Skladal, D., Grissenauer, G., Konstantopoulou, V., Felber, S., & Sperl, W. (Jul 2000). Very high plasma leucine concentrations without neurological symptoms in a patient with classical maple syrup urine disease. Journal of inherited metabolic disease, 23(5):513-4.
Retrieved from http://www.ncbi.nlm.nih.gov/pubmed/10947206
G.43
Strauss, K.A, Puffenberger,E.G., Morton, D.H. (2006). Maple Syrup Urine Disease. In Pagon R.A., Bird T.D., Dolan C.R., Stephens K., Adam M.P., editors, GeneReviews™. Seattle, WA: University of Washington, Seattle.
F.613
Strauss, K.A., & Morton, D.H. (Jul 2003). Branched-chain Ketoacyl Dehydrogenase Deficiency: Maple Syrup Disease. Current treatment options in neurology, 5(4):329-341.
Retrieved from http://www.ncbi.nlm.nih.gov/pubmed/12791200
F.35
Strauss, K.A., Mazariegos, G.V., Sindhi, R., Squires, R., Finegold, D.N., Vockley, G., Robinson, D.L., Hendrickson, C., Virji, M., Cropcho, L., Puffenberger, E.G., McGhee, W., Seward, L.M., & Morton, D.H. (Mar 2006). Elective liver transplantation for the treatment of classical maple syrup urine disease. American journal of transplantation : official journal of the American Society of Transplantation and the American Society of Transplant Surgeons, 6, 557-64. doi:10.1111/j.1600-6143.2005.01209.x
F.426
Strauss, K.A., Wardley, B., Robinson, D., Hendrickson, C., Rider, N.L., Puffenberger, E.G., Shellmer, D., Shelmer, D., Moser, A.B., & Morton, D.H. (Apr 2010). Classical maple syrup urine disease and brain development: principles of management and formula design. Molecular genetics and metabolism, 99, 333-45. doi:10.1016/j.ymgme.2009.12.007
F.28
Tabanlio?lu, D., Ersoy-Evans, S., & Karaduman, A. (2009 Mar-Apr). Acrodermatitis enteropathica-like eruption in metabolic disorders: acrodermatitis dysmetabolica is proposed as a better term. Pediatric dermatology, 26, 150-4. doi:10.1111/j.1525-1470.2008.00803.x
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Tain, Y.L., Huang, S.C., Hung, F.C., Wang, H.S., & Sun, P.C. (1996 Sep-Oct). Acrodermatitis enteropathica-like eruption during treatment of maple syrup urine disease: report of one case. Zhonghua Minguo xiao er ke yi xue hui za zhi [Journal]. Zhonghua Minguo xiao er ke yi xue hui, 37(5):357-60.
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F.423
Thimm, E., Hadzik, B., & Hohn, T. (Jul 2010). Continuous venovenous hemofiltration rapidly lowers toxic metabolites in a patient with MSUD and imminent cerebral herniation. Klinische Pädiatrie, 222, 264-5. doi:10.1055/s-0030-1247508
F.86
Thompson, G.N., Butt, W.W., Shann, F.A., Kirby, D.M., Henning, R.D., Howells, D.W., & Osborne, A. (Jun 1991). Continuous venovenous hemofiltration in the management of acute decompensation in inborn errors of metabolism. The Journal of pediatrics, 118(6):879-84.
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G.5
Thompson, S. et al. (2006, August, 17). Intermediate MSUD and Breast feeding/ MSUD Baby/ Calories for MSUD patient/ MSUD (new dx- for newborn). Message posted to Metabolic Dietician List,  <PNO-METAB-L@LISTSERV.CC.EMORY.EDU>
F.96
Tornqvist, K., & Tornqvist, H. (1996). Corneal deepithelialization caused by acute deficiency of isoleucine during treatment of a patient with maple syrup urine disease. Acta ophthalmologica Scandinavica. Supplement, (219):48-9.
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F.125
Treacy, E., Clow, C.L., Reade, T.R., Chitayat, D., Mamer, O.A., & Scriver, C.R. (1992). Maple syrup urine disease: interrelations between branched-chain amino-, oxo- and hydroxyacids; implications for treatment; associations with CNS dysmyelination. Journal of inherited metabolic disease, 15(1):121-35.
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G.3
Ueda, K. et al. (2001, August, 3). MSUD "wobblies"/ MSUD and Carnation Good Start/ BTK Deficiency or Allstrom Syndrome Diet/ E-3 Component Deficiency. Message posted to Metabolic Dietician List,  <PNO-METAB-L@LISTSERV.CC.EMORY.EDU>
G.27
Van Calcar, S. (2003, MONTH). Diet Treatment for Maple Syrup Urine Disease (MSUD) [PowerPoint slides]. Paper presented at the 9th Abbott Nutrition Advances in Management of Inherited Metabolic Disorders.
G.6
Van Calcar, S. et al. (2008, March, 21). Maternal MSUD/ Beta keto thiolase deficiency/ MSUD infant/ Mild MSUD and Protein restriction/ MSUD essential fatty acid intake . Message posted to Metabolic Dietician List,  <PNO-METAB-L@LISTSERV.CC.EMORY.EDU>
F.102
Van Calcar, S.C., Harding, C.O., Davidson, S.R., Barness, L.A., & Wolff, J.A. (Nov 1992). Case reports of successful pregnancy in women with maple syrup urine disease and propionic acidemia. American journal of medical genetics, 44, 641-6. doi:10.1002/ajmg.1320440523
F.92
Wang, I.J., Chu, S.Y., Wang, C.Y., Wang, P.J., & Hwu, W.L. (2003 Jul-Aug). Maple syrup urine disease presenting with neonatal status epilepticus: report of one case. Acta paediatrica Taiwanica = Taiwan er ke yi xue hui za zhi, 44(4):246-8.
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G.21
Wasserstein, M.P. (2006, Winter). Medical Therapy for Maple Syrup Urine Disease. MSUD Family Newsletter, 23(1). Retrieved fromhttp://www.msud-support.org/index.php?view=article&catid=41%3Avolume-23-1&id=314%3Amedical-therapy-for-maple-syrup-urine-disease&option=com_content&Itemid=5
G.30
Wendel, U. (2004/05, Winter). Diagnostics and Treatment of MSUD in Germany. MSUD Support Newsletter, 22(1). Retrieved from http://www.msud-support.org/index.php?view=category&id=39%3Avolume-22-1&option=com_content&Itemid=5
F.54
Wendel, U., Saudubray, J.M., Bodner, A., & Schadewaldt, P. (Dec 1999). Liver transplantation in maple syrup urine disease. European journal of pediatrics, 158 Suppl 2:S60-4.
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F.49
Yoshida, S., & Tanaka, T. (Apr 2003). Postpartum death with maple syrup urine disease. International journal of gynaecology and obstetrics: the official organ of the International Federation of Gynaecology and Obstetrics, 81(1):57-8.
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