Primary hyperammonemia (Urea cycle disorders)

Definition:

Hyperammonemia is a metabolic condition characterized by elevated levels of ammonia in the blood, and may result in irreversible brain damage if not treated early and thoroughly. Hyperammonemia can be classified into primary or secondary hyperammonemia depending on the underlying pathophysiology. Detoxification of ammonia is mainly accomplished by the urea cycle in periportal hepatocytes. If the urea cycle is directly affected by a defect of any of the involved enzymes or transporters, this results in primary hyperammonemia.

References:

[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]

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