title: Biliary atresia: Swiss national study, 1994-2004.
abstract: OBJECTIVE
  To determine the epidemiology of biliary atresia (BA) in Switzerland, the outcome of the children from diagnosis, and the prognostic factors.
  
  
  METHODS
  The records of all patients with BA born in Switzerland between January 1994 and December 2004 were analyzed. Survival rates were calculated with the Kaplan-Meier method, and prognostic factors evaluated with the log rank test. Median follow up was 58 months (range, 5-124).
  
  
  RESULTS
  BA was diagnosed in 48 children. Incidence was 1 in 17,800 live births (95% confidence interval 1/13,900-1/24,800), without significant regional, annual, or seasonal variation. Forty-three children underwent a Kasai portoenterostomy (PE) in 5 different Swiss pediatric surgery units. Median age at Kasai PE was 68 days (range, 30-126). Four-year survival with native liver after Kasai PE was 37.4%. Liver transplantation (LT) was needed in 31 in 48 children with BA, including 5 patients without previous Kasai PE. Four patients (8%, all born before 2001) died while waiting for LT, and 29 LT were performed in 27 patients (28 in Geneva and 1 in Paris). All of the transplanted patients are alive. Four-year overall BA patient survival was 91.7%. Four-year survival with native liver was 75% in patients who underwent Kasai PE before 46 days, 33% in patients operated on between 46 and 75 days, and 11% in patients operated on after 75 days (P = 0.02).
  
  
  CONCLUSIONS
  Overall survival of patients with BA in Switzerland compares favorably with current international standards, whereas results of the Kasai operation could be improved to reduce the need for LTs in infancy and early childhood.
authors: University Hospital of Geneva, Switzerland.
authors: Barbara E
authors: BE
authors: Wildhaber
authors: Pietro
authors: P
authors: Majno
authors: Johannes
authors: J
authors: Mayr
authors: Zacharias
authors: Z
authors: Zachariou
authors: Judith
authors: J
authors: Hohlfeld
authors: Marcus
authors: M
authors: Schwoebel
authors: Walter
authors: W
authors: Kistler
authors: Martin
authors: M
authors: Meuli
authors: Claude
authors: C
authors: Le Coultre
authors: Gilles
authors: G
authors: Mentha
authors: Dominique
authors: D
authors: Belli
authors: Christophe
authors: C
authors: Chardot
mesh_terms: D000367:Age Factors; D001656:Biliary Atresia / Q000453:epidemiology* / Q000401:mortality* / Q000601:surgery; D005260:Female; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D053208:Kaplan-Meier Estimate; D016031:Liver Transplantation / Q000401:mortality*; D008297:Male; D011171:Portoenterostomy, Hepatic / Q000401:mortality*; D011379:Prognosis; D012189:Retrospective Studies; D013557:Switzerland / Q000453:epidemiology; D016896:Treatment Outcome