Pediatric hepatobiliary disease: Genetic and clinical manifestations

In children, hepatobiliary diseases are rarely and mainly due to congenital defects during formation, development of the liver biliary tract or disorders metabolism. The infant have incomplete physiology perinatal until childhood period. process complete child's tract, there important changes affected by genetic environmental factors. Therefore, very vulnerable leading in children. Disorders formation bile ducts, secretion, hepatocellular metabolism, disturbances metabolism all lead associated with diseases. Based on pathophysiology, children can be divided into two groups: structure function disruption metabolic processes cells. secondary effects disease threaten a life, is cause such as hypoglycemia, coagulation disorder low concentration vitamin K-dependent factors intracranial hemorrhage infections caused immunodeficiency, malnutrition, increased portal venous pressure severe gastrointestinal bleeding... should detected treated early avoid adverse complications. context this paper, we focus causes Genetic research situation World Vietnam will also mentioned. information about clinical manifestations aggregated contribute general understanding orientate accurate effective treatment for patients.