title: Biliary atresia: the Brazilian experience.
abstract: OBJECTIVE
  To evaluate epidemiological, clinical and prognostic characteristics of children with biliary atresia.
  
  
  METHODS
  Data regarding portoenterostomy, liver transplantation (LTx), age at last follow-up and survival were collected from the records of patients followed up in six Brazilian centers (1982-2008) and compared regarding decades of surgery.
  
  
  RESULTS
  Of 513 patients, 76.4% underwent portoenterostomy [age: 60-94.7 (82.6±32.8) days] and 46.6% underwent LTx. In 69% of cases, LTx followed portoenterostomy, whereas in 31% of cases LTx was performed as the primary surgery. Patients from the Northeast region underwent portoenterostomy later than infants from Southern (p = 0.008) and Southeastern (p = 0.0012) Brazil, although even in the latter two regions age at portoenterostomy was higher than desirable. Over the decades, LTx was increasingly performed. Overall survival was 67.6%. Survival increased over the decades (1980s vs. 1990s, p = 0.002; 1980s vs. 2000s, p < 0.001; 1990s vs. 2000s, p < 0.001). The 4-year post-portoenterostomy survival, with or without LTx, was 73.4%, inversely correlated with age at portoenterostomy (80, 77.7, 60.5% for ≤ 60, 61-90, > 90 days, respectively). Higher survival rates were observed among transplanted patients (88.3%). The 4-year native liver survival was 36.8%, inversely correlated with age at portoenterostomy (54, 33.3, 26.6% for ≤ 60, 61-90, > 90 days, respectively).
  
  
  CONCLUSIONS
  This multicenter study showed that late referral for biliary atresia is still a problem in Brazil, affecting patient survival. Strategies to enhance earlier referral are currently being developed aiming to decrease the need for liver transplantation in the first years of life.
authors.affiliation: Hospital de Base do Distrito Federal, Brasília, DF, Brazil. elisacarvalho@terra.com.br
authors.forename: Elisa de
authors.initials: Ed
authors.lastname: Carvalho
authors.forename: Jorge Luiz dos
authors.initials: JL
authors.lastname: Santos
authors.forename: Themis Reverbel da
authors.initials: TR
authors.lastname: Silveira
authors.forename: Carlos Oscar
authors.initials: CO
authors.lastname: Kieling
authors.forename: Luciana Rodrigues
authors.initials: LR
authors.lastname: Silva
authors.forename: Gilda
authors.initials: G
authors.lastname: Porta
authors.forename: Irene Kazue
authors.initials: IK
authors.lastname: Miura
authors.forename: Adriana Maria Alves
authors.initials: AM
authors.lastname: De Tommaso
authors.forename: Maria Ângela Bellomo
authors.initials: MÂ
authors.lastname: Brandão
authors.forename: Alexandre Rodrigues
authors.initials: AR
authors.lastname: Ferreira
authors.forename: José Roberto de Deus
authors.initials: JR
authors.lastname: Macêdo
authors.forename: José Tenório de
authors.initials: JT
authors.lastname: Almeida Neto
mesh_terms: D001656:Biliary Atresia / Q000175:diagnosis / Q000453:epidemiology / Q000401:mortality* / Q000601:surgery; D001938:Brazil / Q000453:epidemiology; D002675:Child, Preschool; D004812:Epidemiologic Methods; D005260:Female; D006801:Humans; D007223:Infant; D016031:Liver Transplantation / Q000401:mortality*; D008297:Male; D011171:Portoenterostomy, Hepatic / Q000401:mortality*; D015996:Survival Rate