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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.
Carglumic acid, sold under the brand name Carbaglu among others, is used for the treatment of hyperammonaemia. [ 3 ] [ 6 ] [ 7 ] Carglumic acid is a carbamoyl phosphate synthetase 1 (CPS 1) activator.
During an acute hyperammonemic episode, oral proteins must be avoided and intravenous (I.V.) lipids, glucose and insulin (if needed) should be given to promote anabolism. I.V. nitrogen scavenging therapy (with sodium benzoate and/or sodium phenylacetate) should normalize ammonia levels, but if unsuccessful, hemodialysis is recommended.
Sodium phenylbutyrate is taken orally or by nasogastric intubation as a tablet or powder, and tastes very salty and bitter. It treats urea cycle disorders, genetic diseases in which nitrogen waste builds up in the blood plasma as ammonia glutamine (a state called hyperammonemia) due to deficiences in the enzymes carbamoyl phosphate synthetase I, ornithine transcarbamylase, or argininosuccinic ...
Transient hyperammonemia of the newborn (THAN) is an idiopathic disorder occasionally present in preterm newborns but not always symptomatic. [1] Continuous dialysis or hemofiltration have proven to be the most effective treatment. [1] [2] Nutritional support and sodium benzoate have also been used to treat THAN. [2]
Spoilers ahead! We've warned you. We mean it. Read no further until you really want some clues or you've completely given up and want the answers ASAP. Get ready for all of today's NYT ...
A 29-year-old man’s debilitating night terrors were the first sign of rare autoimmune disorder that rapidly progressed, landing him in the intensive care unit in a “catatonic state.” Ben ...
Hyperinsulinism-hyperammonemia syndrome (HIHA) due to glutamate dehydrogenase 1 gene. Can cause intellectual disability and epilepsy in severe cases. [3] Gastric dumping syndrome (after gastrointestinal surgery) Other congenital metabolic diseases; some of the common include Maple syrup urine disease and other organic acidurias