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Hyperammonemia, or high ammonia levels, is a metabolic disturbance characterised by an excess of ammonia in the blood. Severe hyperammonemia is a dangerous condition that may lead to brain injury and death. It may be primary or secondary. Ammonia is a substance that contains nitrogen. It is a product of the catabolism of protein.
Severe Transient Hyperammonemia is diagnosed when ammonia levels are above 50 μM up to as much as 4000 μM. Severe Transient Hyperammonemia causes neurological problems as ammonia levels in the brain are too high, which can cause infant hyptotonia as well as neonatal seizures. [5]
As a result, propionyl-CoA, propionic acid, ketones, ammonia, and other toxic compounds accumulate in the blood, causing the signs and symptoms of propionic acidemia. Hyperammonemia develops due to the inhibitory effects of propionyl-CoA on N-acetylglutamate synthase , indirectly resulting in slowing of the urea cycle .
[2] [6] It may be supported by blood ammonia levels, an electroencephalogram, or computer tomography (CT scan) of the brain. [4] [6] Hepatic encephalopathy is possibly reversible with treatment. [1] This typically involves supportive care and addressing the triggers of the event. [4] Lactulose is frequently used to decrease ammonia levels. [1]
GS is present predominantly in the brain, kidneys, and liver. [4] [10] GS in the brain participates in the metabolic regulation of glutamate, the detoxification of brain ammonia, the assimilation of ammonia, recyclization of neurotransmitters, and termination of neurotransmitter signals. [4] [14] GS, in the brain, is found primarily in ...
Ornithine translocase deficiency, also called hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome, [1] is a rare autosomal recessive [2] urea cycle disorder affecting the enzyme ornithine translocase, which causes ammonia to accumulate in the blood, a condition called hyperammonemia.
This ammonia will obviously have to be transported out of the neurons and back into the astrocytes for detoxification, as an elevated ammonia concentration has detrimental effects on a number of cellular functions and can cause a spectrum of neuropsychiatric and neurological symptoms (impaired memory, shortened attention span, sleep-wake ...
Protein toxicity is the effect of the buildup of protein metabolic waste compounds, like urea, uric acid, ammonia, and creatinine.Protein toxicity has many causes, including urea cycle disorders, genetic mutations, excessive protein intake, and insufficient kidney function, such as chronic kidney disease and acute kidney injury.