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. Like other inherited metabolic disorders, aminoaciduria does not affect fetal growth and development in utero or delivery, and may be asymptomatic in the early stages. Phenylketonuria, tyrosinemia and Hartnup’s disease are 3 clinically important early childhood amino acid disorders that are typically due to biochemical defects. Patients with amino aciduria must pay attention to a low-protein diet, and since a low-protein diet requires long-term adherence, there are some details that require special attention. The usual saying that the details make the difference also applies here. 1. Ensure sufficient energy: Adequate energy intake is a prerequisite to ensure effective protein utilization. Therefore, before implementing a low-protein diet, both doctors and patients should visit a nutrition clinic to have your energy intake determined by a dietitian. 2. Weigh yourself regularly: Regular weight measurement can keep you informed of your nutritional status. 3.Regular monitoring of kidney function and electrolytes: Know the effect of a low protein diet on kidney function and electrolytes. 4.Regular monitoring of albumin and prealbumin: to understand your body’s protein synthesis and nutritional status.