Individuals with inherited hyperammonemias often present developmental and intellectual
deficiencies which are likely to be exaggerated by hyperammonemia episodes in long-term …

Introduction: Hyperammonemia is a marker for a disturbance either in the ammonia
detoxification or less likely in the ammonia production. The symptoms are due mainly to …

Ammonia, normally produced from catabolism of amino acids, is a deadly neurotoxin. As
such, the concentration of free ammonia in the blood is very tightly regulated and is …

Hyperammonaemia is a metabolic disturbance characterized by accumulation of ammonia
in the blood. Entry of ammonia into the brain via the blood-brain barrier leads to …

Ammonia is a neurotoxic compound which is detoxified through liver enzymes from urea
cycle. Several inherited or acquired conditions can elevate ammonia concentrations in …

Hyperammonaemia is a metabolic derangement that may cause severe neurological
damage and even death due to cerebral oedema, further complicating the prognosis of its …

Background: Urea cycle disorders are amongst the most common of the inborn errors of
metabolism. These disorders affect up to 1/25,000 live births in the United States and the …

Human adults produce around 1000 mmol of ammonia daily. Some is reutilized in
biosynthesis. The remainder is waste and neurotoxic. Eventually most is excreted in urine as …

Discussion Malnutrition associated with neoplasia was considered the reason for the
hyperammonemia, after excluding the other main causes. Indeed, protein hypercatabolism …

Background: Symptoms of hyperammonemia occur in patients irrespective of the kind of
metabolic diseases. Age, metabolic and nutritional status, and decompensation factors such …