Occasionally, an individual may inherit a mild form of the disorder in which ammonia accumulates in the bloodstream only during periods of illness or other stress.

It processes excess nitrogen, generated when protein is used by the body, to make a compound called urea that is excreted by the kidneys.

[citation needed] This condition is inherited in an autosomal recessive pattern, which means two copies of the gene in each cell are altered.

Newborn screening for ASA is available in the U.S. and parts of Australia, and is considered in several European countries[citation needed] During an acute hyperammonemic episode, oral proteins must be avoided and intravenous (I.V.)

In those with frequent episodes of metabolic decompensation or with hyperammonemia even when following a protein-restricted diet, daily oral nitrogen scavenging therapy may be successful.

Arterial hypertension can be treated by restoring nitric oxide deficiency Due to the rarity of the disease, it is hard to estimate mortality rates or life expectancy.