关键词: 胆汁淤积性肝病, 婴儿, 病因, 临床特征

Abstract: Objective To analyze the etiological characteristics of infantile cholestatic hepatopathy (ICH).Methods Data of cases of jaundice in infants admitted to the Department of Gastroenterology of Children's Hospital from January 2014 to July 2018 were collected. Those meeting the ICH diagnostic criteria were enrolled, which was recommended by the American Academy of Pediatrics and was used as the inclusion criteria. The etiology and clinical characteristics of the enrolled cases were retrospectively analyzed.Results A total of 271 ICH patients met the inclusion criteria. Among them, there were 110 cases of infectious diseases, 19 cases of genetic metabolic diseases, 81 cases of structural abnormalities of the biliary tract, 3 cases of drug-related hepatitis, 12 cases of total parenteral nutrition associated cholestasis, 7 cases of congenital hypothyroidism, and 39 cases of unknown etiology. Among infectious diseases, the highest incidence was cytomegalovirus infection, with a total of 61 cases. Among the 19 cases of genetic metabolic diseases, 12 cases were caused by Citrin deficiency. Other etiology included Alagille syndrome in 4 cases, congenital carnitine deficiency in 2 cases, and progressive familial cholestasis in the liver in 1 case. In the disease that the structure of the biliary tract is abnormal, there were 55 cases of congenital biliary atresia which were diagnosed by cholangiography, 32 cases of Kasai surgery were performed for treatment, 17 cases of congenital choledochal cyst, 9 cases of biliary dysplasia.Conclusion On the one hand, in the infectious factor group, cytomegalovirus infection was the main factor. Blood biochemical examination showed that the bilirubin index had varying degrees of increase and the liver enzyme index had the slight rising. Diseases of the biliary tract system were mainly congenital biliary atresia, which was characterized by persistent jaundice that could not be subside, light yellow or terracotta stool and poor nutritional status in the infants. B ultrasound of gallbladder indicated abnormal morphology and poor systolic function and cirrhosis of different degrees. Some cases were relieved after Kasai operation. On the other hand, Citrin deficiency was the main factor in the genetic and metabolic factor group. The clinical features of this disease included jaundice, abdominal distension, growth retardation, abnormal coagulation phase and alpha-fetoprotein index. The etiology and pathogenesis of ICH were complex, and its diagnosis and treatment were very difficult.

Key words: Cholestatic hepatopathy, Infant, Etiology, Clinical features