title: Autoimmune hepatitis and primary sclerosing cholangitis in children and adolescents.
abstract: Clinical presentation and histopathology of autoimmune hepatitis (AIH) and primary sclerosing cholangitis (PSC) overlap syndrome (OS) are similar, but their management is different. We conducted a pediatric retrospective cross-sectional study of 34 patients with AIH and PSC. AIH had female predominance (74%) and was lower in PSC (45%). There was a trend toward higher frequency of blacks in PSC/OS (55%) compared to Caucasians (36%) and Hispanics (9%), but not race differences in AIH. Inflammatory bowel disease (IBD) was present in 75% of PSC/OS. Plasma cells were not specific for AIH (found in 42% of PSC). Concentric fibrosis was not reliable for PSC as was found in 46% of AIH.
  
  
  CONCLUSIONS
  A combination of clinical history, laboratory tests, imaging studies and liver biopsy are required to confirm and properly treat AIH and PSC. Liver biopsy should be used to grade severity and disease progression, but cannot be used alone to diagnose these conditions.
authors.affiliation: 1Department of Pathology, University of Miami, Holtz Children's Hospital, Miami, FL, USA.
authors.forename: Claudia Patricia
authors.initials: CP
authors.lastname: Rojas
authors.forename: Rajasekhar
authors.initials: R
authors.lastname: Bodicharla
authors.forename: German
authors.initials: G
authors.lastname: Campuzano-Zuluaga
authors.forename: Lina
authors.initials: L
authors.lastname: Hernandez
authors.forename: Maria Matilde
authors.initials: MM
authors.lastname: Rodriguez
mesh_terms: D000293:Adolescent; D000410:Alanine Transaminase / Q000097:blood; D001219:Aspartate Aminotransferases / Q000097:blood; D001323:Autoantibodies / Q000097:blood; D002648:Child; D002675:Child, Preschool; D015209:Cholangitis, Sclerosing / Q000097:blood / Q000175:diagnosis* / Q000473:pathology; D003430:Cross-Sectional Studies; D003937:Diagnosis, Differential; D005260:Female; D019693:Hepatitis, Autoimmune / Q000097:blood / Q000175:diagnosis* / Q000473:pathology; D006801:Humans; D008099:Liver / Q000473:pathology; D008297:Male; D012189:Retrospective Studies; D005723:gamma-Glutamyltransferase / Q000097:blood
chemical_list: D001323:Autoantibodies; D005723:gamma-Glutamyltransferase; C554782:gamma-glutamyltransferase, human; D001219:Aspartate Aminotransferases; D000410:Alanine Transaminase
keywords: autoantibodies; cholestasis; cirrhosis; hepatitis; liver biopsy; pediatric
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