Amino aciduria (hyperaminoaciduria) is a group of metabolic disorders characterized by excessive excretion of amino acids in the urine and can be classified as prenephrogenic, nephrogenic or mixed. There are more than 100 hereditary disorders caused by amino acid metabolism disorders. New discoveries will continue to be added as biochemical testing techniques advance. We can examine them according to the main clinical features. 1. Hereditary tyrosinemia (1) can be divided into 3 types. The manifestations of each type are different. About more than half of the children have mild to moderate mental decline, and may show self-injurious behavior and limb-motor incoordination, with more prominent language defects. Type II 1 year old or almost 1 year old due to corneal erosion, often causing lacrimation, photophobia and eye redness, neovascularization and corneal clouding, palmar and plantar keratosis, often with excessive sweating and pain, is an inflammatory response due to crystalline tyrosine deposition, and nodules are also the cause of corneal lesions. (2) Type I may have hepatosplenomegaly or liver failure manifestations such as cirrhosis and ascites, and often die after 1 or several years of illness. Tyrosinemia in the neonatal period can lead to liver failure and premature death. Increased blood tyrosine and urine tyrosine levels have diagnostic significance, and blood methionine (methionine) and other amino acids may also be increased. 2, Hartnup disease (1) children born normal, late infancy or early childhood symptoms, the characteristic clinical manifestations are intermittent appearance of red scaly rash, throughout the face, neck, hands and feet, etc., rather like pellagra lesions. (2) There may be growth retardation, episodic personality disorders such as emotional variability, inability to control temper, psychosis, hallucinatory psychosis, episodic cerebellar ataxia (gait instability, intentional tremor, dysarthria, etc.), and occasionally myospasm, vertigo, nystagmus, diplopia, corneal erosion, ptosis, and other signs. (3) Sun exposure, emotion, stress and sulfa drugs can trigger an attack, which lasts about 2 weeks and is followed by a period of episodes of varying duration. The frequency of seizures decreases as the child matures, and some children may have mild persistent mental decline.