ABSTRACT
Objective: To analyze the clinical pathological characteristics of children with biliary dysplasia (BD), and to explore the related factors affecting the poor prognosis. Methods: 32 children with BD admitted to the Shenzhen Children's Hospital from January 2014 to January 2019 were selected. All the patients underwent cholecystostomy and biliary drainage surgery, liver tissue specimens were biopsied for routine hematoxylin and eosin (HE) and immunohistochemical staining after surgery. The clinical characteristics, cholecystography and liver histopathology of all children with BD were analyzed. The patients were followed up until April 2020, according to the prognosis, the related factors affecting the poor prognosis of children with BD were analyzed by univariate and multivariate logistic regressions. Results: There were 19 males and 13 females in 32 BD children, mostly fullterm children with cesarean section, normal birth weight, the duration of jaundice was (31.5±7.2) days, accompanied by dark urine, light stools, hepatosplenomegaly and mild ascites, often accompanied by congenital heart disease and other symptoms, mostly cytomegalovirus infection in children with BD. Cholangiography showed thin intrahepatic and extrahepatic bile ducts and dysplasia of the gallbladder in BD children. The operative age of children with BD was 30-125 days, the pathological manifestations of liver biopsy were cholestasis, formation of bile embolism, reduction or disappearance of bile ducts in the hepatic interlobular bile duct and portal area, inflammatory cell infiltration, stem cell degeneration, spot-like necrosis, varying degrees of liver fibrosis and intrahepatic bile duct hyperplasia, accompanied by the expressions of epithelial membrane antigen (EMA) and cytokeratin-19 (CK19). Genetic testing showed that Alagille syndrome was associated with JAG1 gene mutation. The median follow-up time of BD children was 50 months, among whom 12 children had poor prognosis and 20 had good prognosis. The age at operation, hepatosplenomegaly, ascites, hepatocyte degeneration, degree of liver fibrosis, total bilirubin (TBIL), EMA and CK19 were significantly increased in the patients with poor prognosis compared with the patients with good prognosis, and the differences were statistically significant (P<0.05). Multivariate logistic regression analysis showed that age at operation, liver fiber, the expression of TBIL, EMA and CK19 were the influencing factors for the poor prognosis of BD children (P<0.05). Conclusion: The age at operation, severity of liver fibrosis, and expressions of TBIL, EMA and CK19 are increased in children with BD, suggesting a poor prognosis.