Abstract
There are primary and secondary hyperammonemia. Primary hyperammonemia is a hereditary disorder of the urea formation cycle. Secondary hyperammonemia can occur both in hereditary diseases (mitochondrial diseases, organic aciduria/aminoacidopathies) and in asphyxia suffered in childbirth, liver damage of various etiologies (toxic, infectious, etc.), extrahepatic portal hypertension, renal failure, infections caused by urease-producing microorganisms, diseases accompanied by increased catabolism, etc. transient nature. Regardless of the etiology, hyperammonemia can cause irreversible damage to brain cells, which determines the exceptional importance of early diagnosis and timely therapy.
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