Hyperammonemia is a metabolic disturbance characterised by an excess of ammonia in the blood. It 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. It is converted to the less toxic substance urea prior to excretion in urine by the kidneys. The metabolic pathways that synthesize urea involve reactions that start in the mitochondria and then move into the cytosol. The process is known as the urea cycle, which comprises several enzymes acting in sequence. It is greatly exacerbated by common zinc deficiency, which raises ammonia levels further.[1]
^Riggio, O.; Merli, M.; Capocaccia, L.; Caschera, M.; Zullo, A.; Pinto, G.; Gaudio, E.; Franchitto, A.; Spagnoli, R.; D'Aquilino, E. (September 1992). "Zinc supplementation reduces blood ammonia and increases liver ornithine transcarbamylase activity in experimental cirrhosis". Hepatology. 16 (3): 785–789. doi:10.1002/hep.1840160326. ISSN 0270-9139. PMID 1505922. S2CID 1141979.
Hyperammonemia is a metabolic disturbance characterised by an excess of ammonia in the blood. It is a dangerous condition that may lead to brain injury...
presentation, any affected individual is at risk for an episode of hyperammonemia that could still be life-threatening, if presented with the appropriate...
become elevated all the way back up the cycle to NH+ 4, resulting in hyperammonemia (elevated [NH+ 4]P). Although the root cause of NH+ 4 toxicity is not...
November 2017. Rett initially called this syndrome cerebroaatrophic hyperammonemia, but the elevated ammonia levels in the bloodstream were later found...
Ornithine translocase deficiency, also called hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome, is a rare autosomal recessive urea cycle...
Transient hyperammonemia of the newborn (THAN) is an idiopathic disorder occasionally present in preterm newborns but not always symptomatic. Continuous...
consistent results.[medical citation needed] Lactulose is useful in treating hyperammonemia (high blood ammonia), which can lead to hepatic encephalopathy. Lactulose...
blood (hyperammonemia). Likewise, defects in the enzymes responsible for the urea cycle, such as ornithine transcarbamylase, lead to hyperammonemia. Hyperammonemia...
computer game Hungry Hungry Hippos, a tabletop game Hyperornithinemia-hyperammonemia-homocitrullinuria syndrome Search for "hhh" on Wikipedia. HHHR Tower...
an X-linked recessive pattern. OTC deficiency is also accompanied by hyperammonemia and high orotic acid levels. BUN is usually reported in mg/dL in some...
production and diclofenac production. It is also employed to treat type II hyperammonemia to help reduce the amounts of ammonia in a patient's bloodstream by...
storage diseases may show transient exercise-induced alkalosis (high pH), hyperammonemia, and myogenic hyperuricemia. During a non-ischemic forearm exercise...
mutations are known to cause onset of hyperammonemia during the first weeks of life. 70 manifest as hyperammonemia in male patients later in life. Most...
in humans. In another metabolic disorder, in the hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome, first described in 1969, ornithine...
tone. Without treatment, death can occur quickly, due to secondary hyperammonemia, infection, cardiomyopathy, or brain damage. Propionic acidemia can...
arginine to avoid an excess of ammonia in the blood, otherwise known as hyperammonemia, isoleucine, leucine, valine, lysine, methionine as a sulfur-containing...
placebo. Sodium benzoate, along with phenylbutyrate, is used to treat hyperammonemia. Sodium benzoate, along with caffeine, is used to treat postdural puncture...
Isoelectric EEG Respiratory arrest Seizures Multiple organ failure Flaccidity Hyperammonemia (above 300 mg/dL of blood) Death The cause of Reye syndrome, is unknown...
same, while in severe cases symptoms can advance to amino aciduria and hyperammonemia. In a large Asian series of Distal renal Tubular Acidosis in Sjogren's...
Carglumic acid is indicated for the treatment of acute hyperammonemia and chronic hyperammonemia. "Prescription medicines: registration of new chemical...
builds up in the blood plasma as ammonia glutamine (a state called hyperammonemia) due to deficiences in the enzymes carbamoyl phosphate synthetase I...
also prophylactically in high risk patients. Acetyl-L-carnitine lowers hyperammonemia less markedly than L-carnitine. Valproate inhibits CYP2C9, glucuronyl...
people who take megadoses of nutritional supplements and vitamins. Hyperammonemia, elevated level of ammonia in blood (including cerebral edema/intracranial...
It's mainly seen in diseases that cause increased levels of ammonia (hyperammonemia), such as chronic liver disease and Wilson's disease. They are not associated...
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