Pediatric Autoimmune Liver Diseases
DOI link for Pediatric Autoimmune Liver Diseases
Pediatric Autoimmune Liver Diseases book
Autoimmune liver disease in childhood is a rare chronic progressive liver disease that includes autoimmune hepatitis (AIH), autoimmune sclerosing cholangitis, overlap syndrome and de novo autoimmune hepatitis after liver transplantation. In pediatric patients with inflammatory bowel disease and primary sclerosing cholangitis, small group series showed improved liver function and improved clinical symptoms with use of vancomycin although this antibiotic has not been evaluated in large patient studies. AIH is a chronic disease of unknown etiology which presents as a progressive inflammatory liver disease characterized by elevated serum liver enzymes and immunoglobulin G with presence of autoantibodies. Physical examination varies from normal findings to the presence of jaundice, splenomegaly, cachexia, and cutaneous stigmata of chronic liver disease. Some patients within the spectrum of autoimmune liver diseases present with characteristics of both AIH and cholestatic liver disease like sclerosing cholangitis. Liver transplantation is indicated in patients who present with fulminant liver failure and those who develop end-stage liver disease despite medical management.