The dietary management consists of a diet low in lysine (i.e. low in native protein) with addition of amino acids supplements free from these amino acids.
Hyperleucinemia are different inherited rare disorders in metabolism of the amino acid leucine. There are several types of Hyperleucinemia, caused by different enzyme defects. Depending on the type of the disorder, this can result in metabolic acidosis, hypoglycaemia, emesis and cramps.