Hyperdibasic aminoaciduria, type 1 is characterized by increased renal clearance of lysine, ornithine and arginine, in the presence of normal concentrations of cystine. Heterozygous individuals are asymptomatic but homozygotes display intellectual deficit. To date, 25 heterozygotes and one homozygote have been reported. The disease is Monarch Disease Ontology entry MONDO_0009108 (hyperdibasic aminoaciduria type 1). Also known as: dibasic amino aciduria type 1.