title: Clinical Characteristics and Prognosis in Children and Adolescents With Autoimmune Hepatitis and Overlap Syndrome.
abstract: This is a cohort study of 134 children and adolescents with a known diagnosis of autoimmune hepatitis (AIH). During follow-up, some of them developed autoimmune sclerosing cholangitis (ASC). This study describes the characteristics of the patients upon diagnosis, and their response to treatment and any complications, and compares the patients who developed ASC during follow-up (ASC group) with those who did not (AIH group).
  
  
  
  A total of 73.1% of the patients were girls with a median age upon diagnosis of 10.41 (7.41-12.53) years.
  
  
  
  Of 134 patients, 28 (20.9%) developed cholestatic manifestations, with features of ASC. A few differences were observed between the AIH and ASC groups when they were analyzed by χ test, such as the smaller predominance of girls in ASC group (P = 0.04), and more common asymptomatic presentation in the ASC group (P = 0.01). Cirrhosis was observed in 68% of biopsies, with no significant difference between groups (P = 0.43). Of 16 deaths, 15 were in the AIH group and 1 in the ASC group (P = 0.22). Of 11 transplants, 10 were in the AIH group and one in the ASC group (P = 0.53). The presence of cirrhosis at baseline was associated with a smaller survival probability (P = 0.015). The survival rate by Kaplan-Meier method was 94% at 5 years and 80% at 10 years, and was similar in both the groups (P = 0.08).
  
  
  
  No statistically significant difference was observed between the groups in relation to prognosis and response to treatment.
authors.affiliation: *Clinical Hospital†Department of Pediatrics, Faculty of Medicine‡Faculty of Medicine§Department of Pediatrics, Interdisciplinary Laboratory of Medical Investigation, Faculty of Medicine||Faculty of Medicine¶Department of Surgery, Faculty of Medicine, Federal University of Minas Gerais (UFMG), Brazil.
authors.forename: Adriana T
authors.initials: AT
authors.lastname: Rodrigues
authors.forename: Priscila M F
authors.initials: PM
authors.lastname: Liu
authors.forename: Eleonora D T
authors.initials: ED
authors.lastname: Fagundes
authors.forename: Thaís C N
authors.initials: TC
authors.lastname: Queiroz
authors.forename: Pamela
authors.initials: P
authors.lastname: de Souza Haueisen Barbosa
authors.forename: Soraya L C
authors.initials: SL
authors.lastname: Silva
authors.forename: Ana C
authors.initials: AC
authors.lastname: Simões E Silva
authors.forename: Débora M
authors.initials: DM
authors.lastname: Miranda
authors.forename: Alexandre R
authors.initials: AR
authors.lastname: Ferreira
authors.forename: Luiz R
authors.initials: LR
authors.lastname: Alberti
mesh_terms: D000293:Adolescent; D001379:Azathioprine / Q000627:therapeutic use; D001938:Brazil; D002648:Child; D002662:Child Health Services; D015209:Cholangitis, Sclerosing / Q000150:complications*; D015331:Cohort Studies; D005260:Female; D019693:Hepatitis, Autoimmune / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy / Q000401:mortality*; D006801:Humans; D008297:Male; D011241:Prednisone / Q000627:therapeutic use; D011379:Prognosis; D012737:Sex Factors; D016019:Survival Analysis
chemical_list: D001379:Azathioprine; D011241:Prednisone