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- Outcome and survival of 88 patients with urea cycle disorders: a retrospective evaluation.Eur J Pediatr. 2003; 162: 410-416
- Neonatal onset ornithine transcarbamylase deficiency: a retrospective analysis.J Pediatr. 1999; 134: 268-272
- Neurodevelopmental outcome of long-term therapy of urea cycle disorders in Japan.J Inherit Metab Dis. 1998; 21: 151-159
- An integrated approach to the diagnosis and prospective management of partial ornithine transcarbamylase deficiency.Pediatrics. 2002; 109: 150-152
- In vivo urea cycle flux distinguishes and correlates with phenotypic severity in disorders of the urea cycle.Proc Natl Acad Sci USA. 2000; 97: 8021-8026
- Problems in the management of urea cycle disorders.Mol Genet Metab. 2004; 81: S86-S91
- Proceedings of a consensus conference for the management of patients with urea cycle disorders.J Pediatr. 2001; 138: S6-S10
- Laboratory evaluation of urea cycle disorders.J Pediatr. 2001; 138: S21-S29
- Protein and amino acid requirements and the composition of complementary foods.J Nutr. 2003; 133: 2953S-2961S
- Laboratory support of the clinical nutrition service.Clin Chem Lab Med. 2000; 38: 693-714
- Mechanisms of hyperammonemia.Clin Chem Lab Med. 2002; 40: 653-662
- Glutamate transporter and receptor function in disorders of ammonia metabolism.Ment Retard Dev Disabil Res Rev. 2001; 7: 276-279
- Localized proton MR spectroscopy in infants with urea cycle defect.AJNR Am J Neuroradiol. 2001; 22: 834-837
- Arginases I and II: do their functions overlap?.Mol Genet Metab. 2004; 81: S38-S44
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- Differential utilization of systemic and enteral ammonia for urea synthesis in control subjects and ornithine transcarbamylase deficiency carriers.Am J Clin Nutr. 2003; 78: 749-755
- Exercise promotes BCAA catabolism: effects of BCAA supplementation on skeletal muscle during exercise.J Nutr. 2004; 134: 1583S-1587S
- Metabolic bases of amino acid requirements in acute diseases.Curr Opin Clin Nutr Metab Care. 2002; 5: 189-197
- Effect of alternative pathway therapy on branched chain amino acid metabolism in urea cycle disorder patients.Mol Genet Metab. 2004; 81: S79-S85
- Regulation of skeletal muscle protein metabolism in catabolic states.Curr Opin Clin Nutr Metab Care. 2005; 8: 61-65
- Restoration of ureagenesis in N-acetylglutamate synthase deficiency by N-carbamylglutamate.J Pediatr. 2004; 145: 552-554
- The nutritional management of urea cycle disorders.J Pediatr. 2001; 138: S40-S45
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Dr. Summar acknowledges the support of NIH grants MOI-RR-0095 and U54-RR-019453.
Complete financial disclosure information for each author is provided in the frontmatter of this supplement on page iii.