Clinical manifestations, most of the children are born at term, the performance of l~2 weeks after birth is not abnormal, often in the physiological jaundice subsides and then appeared sclera, skin yellowing. Jaundice continues to deepen with age, and the color of urine also deepens, even to a strong tea color. Some children’s feces become clay-colored after birth, but there are also many children with normal fetal stools and feces, and with the deepening of jaundice the color of feces gradually becomes lighter, and eventually becomes clay-colored. About 15% of the children have white stools one month after birth. In the longer term, the feces may change from white to yellowish. This is due to the high concentration of bilirubin in the blood, a small amount of bilirubin through the intestinal glands discharged into the intestinal lumen and mixed with the stool. As jaundice worsens, the child’s abdomen swells more obviously, and the liver gradually increases in size and hardens. Usually 3 months the child’s liver can be enlarged flat umbilicus, at the same time the spleen enlarged. In severe cases, there may be abdominal wall vein varices, ascites, esophageal varices rupture bleeding and other manifestations of portal hypertension. The general nutritional status of the children is acceptable in the first 3 months, but with the increase of age, the disease progresses, and nutritional developmental disorders gradually appear. Biliary cirrhosis occurs due to long-term obstruction of the bile ducts, and liver function is impaired, leading to impaired absorption of fats and fat-soluble vitamins. If left untreated at an early stage, most of the children will die of liver failure within the first year of life.