Progress in pediatric hepatology

In the past year there have been important advances in pediatric hepatology; we summarize these findings and indicate the clinical and pathophysiologic implications. Initially, we review recent advances in metabolic liver disorders, including the use of molecular genetics techniques to identify the genetic defect of patients with Gilbert syndrome, the use of an enzymatic inhibitor to abolish the mortality of transgenic mice with hereditary tyrosinemia, the use of hepatocellular transplantation to correct the phenotype of histidinemia, the unique clinical and biochemical features that characterize patients with progressive intrahepatic cholestasis due to 3-β-hydroxy-C27 steroid dehydrogenase-isomerase deficiency, and the milder phenotype of patients with mainly hepatic involvement due to specific mitochondrial respiratory chain defects. We then review infectious diseases, focusing on epidemiologic studies regarding the vaccination and morbidity associated with hepatitis viruses and articles that highlight the high morbidity associated with opportunistic infections in immunocompromised transplant recipients.