Hyperammonemia is a condition in which a person has elevated levels of ammonia. Typical symptoms include irritability, vomiting, confusion, and other effects of swelling in the brain.

Higher than normal blood ammonia levels are called hyperammonemia. Acceptable levels depend on age and sex but range from about 42 µg/dL in infants to 200 µg/dL adults assigned male at birth.

Historically, survival among patients with urea-cycle disorders was poor after episodes of hyperammonemia; most children with a severe enzyme deficiency died as neonates — and few survived ...

Tests revealed hyperammonemia of 62 mcmol/L (< 30 mcmol/L). “His ammonia levels continued to increase, and his encephalopathy continued to worsen despite treatment with lactulose and rifaximin ...

Hyperinsulinism and hyperammonemia syndrome has been reported as a cause of moderately severe hyperinsulinism with diffuse involvement of the pancreas. The disorder is caused by gain of function ...

Hyperammonemia. In the newborn period, a normal ammonia level is less than 50 µmol/L. A blood ammonia level between 70 and 100 µmol/L should be viewed in conjunction with clinical findings. An ...

Hyperammonemia can damage muscle and brain. 1 The body converts ammonia to urea in the liver by means of the urea-cycle enzymes, and the urea so generated is subsequently eliminated in the urine ...

Synlogic announced that the Food and Drug Administration (FDA) has granted Fast Track Designation to their investigational drug SYNB1020 for the treatment of hyperammonemia due to urea cycle ...

Recordati Rare Diseases: Carbaglu® (carglumic acid) tablets 200mg receives U.S. FDA approval for a new indication to treat acute hyperammonemia associated with propionic acidemia and ...

Idiopathic hyperammonemia syndrome has been previously described in BMT patients with an incidence as high as 0.5 to 1%.1 2 It is defined as an elevated plasma ammonia concentration in the absence ...

Hyperammonemia is a metabolic condition characterized by an excess of ammonia in the blood. In healthy individuals, ammonia is primarily produced in the intestine as a byproduct of ...

[The authors respond:] We thank Harold Pupko for raising several relevant points concerning our article.[1][1] Valproate-induced hyperammonemic encephalopathy is a well- described phenomenon, but its ...