The nutritional management of urea cycle disorders

Journal of Pediatrics

Volumn 138, Issue 1 SUPPL., 2001, Pages

  Leonard, James V ^a  

^a GREAT ORMOND STREET HOSPITAL   (United Kingdom)

Author keywords

[No Author keywords available]

Indexed keywords

ARGINASE; ARGININE; CARNITINE; CITRIC ACID; ESSENTIAL AMINO ACID;

CONFERENCE PAPER; DIET SUPPLEMENTATION; DISORDERS OF AMINO ACID AND PROTEIN METABOLISM; EMERGENCY TREATMENT; HUMAN; PRIORITY JOURNAL; PROTEIN RESTRICTION;

EID: 0035145675     PISSN: 00223476     EISSN: None     Source Type: Journal    
DOI: 10.1067/mpd.2001.111835     Document Type: Conference Paper

References (27)

1. Rates of urea production and hydrolysis and leucine oxidation change linearly over widely varying protein intakes in healthy adults
2. Urea cycle enzymes
3. Use of stable isotopic measurements to evaluate and manage patients with urea cycle disorders
4. In vivo nitrogen metabolism in ornithine transcarbamylase deficiency
5. Protein requirements of infants and children
6. Arginine, an indispensable amino acid for patients with inborn errors of urea synthesis
7. Nitrogen metabolism in neonatal citrullinaemia
8. Arginine therapy of argininosuccinase deficiency
9. Long-term survival of patients with argininosuccinate synthetase deficiency
10. Long-term follow-up of 12 patients with the late-onset variant of argininosuccinic acid lyase deficiency: No impairment of intellectual and psychomotor development during therapy
11. Renal clearance of amino acids. 1. Normal adults and in patients with aminoaciduria
12. Treatment of urea cycle disorders
13. Citrate therapy in argininosuccinate lyase deficiency
14. Sodium citrate supplementation in inborn argininosuccinate lyase deficiency: A study in a 5-year old patient under total parenteral nutrition
15. Effect of 1-carnitine on cerebral and hepatic energy metabolites in congenitally hyperammonemic sparsefur mice and its role during benzoate therapy
16. Prevention of ammonia toxicity by L-carnitine: Metabolic changes in brain
17. L-carnitine protection in ammonia intoxication. Effect of aminocarnitine on carnitine-dependent metabolism and acute ammonia toxicity
18. Protective effect of 1-carnitine in ammonia-precipitated encephalopathy in the portocaval shunted rat
19. Failure of 1-carnitine to protect mice against hyperammonemia induced by ammonium acetate or urease injection
20. Carnitine deficiency associated with ornithine transcarbamylase deficiency
21. A case of carbamylphosphate synthetase deficiency associated with secondary carnitine deficiency - L-carnitine treatment of CPS-I deficiency
22. Plasma glutamine concentration: A guide in the management of urea cycle disorders
23. Blood levels of ammonia and nitrogen scavenging amino acids in patients with inherited hyperammonemia
24. Plasma alpha-ketoglutarate in urea cycle enzymopathies and its role as a harbinger of hyperammonemic coma
25. Intercurrent illness in inborn errors of intermediary metabolism
26. Early recognition of metabolic decompensation