Organic acid metabolic disorders are also known as organic aciduria or organic acidemia. This group of diseases includes disorders of amino acid metabolism, or abnormal intermediate metabolism of sugar and fat, resulting in the accumulation of organic acids in the body and causing metabolic acidosis. The clinical manifestations of this group of diseases vary widely among individuals and can develop at all ages. If they are not diagnosed and treated in a timely manner, the mortality and neurological disability rates are high. Various types of organic acid metabolic diseases often show similar forms of morbidity, which are broadly as follows: 1. Acute onset in newborns and small infants: more common. It commonly appears from 2 or 3 days after birth with difficulty in breastfeeding, poor response, shortness of breath, and in severe cases with convulsions, vomiting, impaired consciousness, and even death. 2. Intermittent seizures: Everything is normal during the stable period of the disease. Often acute attacks due to infection, diarrhea, hunger and other triggers, vomiting, poor mental health, weakness, convulsions, consciousness barrier ah, often misdiagnosed as recurrent vomiting. 3, sudden infant death: a small number of fat metabolism disorders, such as abnormal fatty acid β-oxidation, in the state of infection, diarrhea, hunger, fatigue, etc., fatty acid metabolism is hyperactive, inducing acute attacks, convulsions, vomiting, impaired consciousness, sudden death. 4.Progressive neurological damage: Some organic acid metabolic diseases are dominated by central nervous system damage, often starting in infancy and childhood, and some patients may start in school age, manifesting as intellectual-motor developmental disorders, epilepsy, hypotonia, tremor, ataxia and other abnormalities, and progressive aggravation. Cranial CT, MRI and other imaging examinations may show brain atrophy or degenerative disease-like lesions. Because the clinical manifestations of organic acid metabolic disease are complex and the symptoms are non-specific, clinical diagnosis is difficult, and confirmation of the diagnosis depends on biochemical tests. Urinary ketone bodies, blood glucose, blood ammonia, electrolytes, liver and kidney function, cardiac enzyme profile, lactate, pyruvate, urinary amino acid screening test can be used as a general screening method. Urinary organic acid analysis using gas chromatography-mass spectrometry (GC-MS) can diagnose most organic acid metabolic diseases.