Primary hyperammonemia (Urea cycle disorders)
Definition:
References:
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[1]. Daniel J Wilkinson, et al. Ammonia metabolism, the brain and fatigue; revisiting the link. Prog Neurobiol. 2010 Jul;91(3):200-19. [Content Brief]
[2]. Johannes Häberle, et al. Clinical and biochemical aspects of primary and secondary hyperammonemic disorders. Arch Biochem Biophys. 2013 Aug 15;536(2):101-8. [Content Brief]
[3]. Lindsay C Burrage, et al. Sodium phenylbutyrate decreases plasma branched-chain amino acids in patients with urea cycle disorders. Mol Genet Metab. 2014 Sep-Oct;113(1-2):131-5. [Content Brief]
[4]. Marcel Cerqueira Cesar Machado, et al. Hyperammonemia due to urea cycle disorders: a potentially fatal condition in the intensive care setting. J Intensive Care. 2014 Mar 13;2(1):22. [Content Brief]
[5]. Simon Matoori, et al. Recent advances in the treatment of hyperammonemia. Adv Drug Deliv Rev. 2015 Aug 1;90:55-68. [Content Brief]