• Home
  1. Home
  2. Biliary atresia: Swiss national study, 1994-2004
Scientific article
English

Biliary atresia: Swiss national study, 1994-2004

Contributors

Wildhaber, Barbara; Majno, Pietro; Mayr, Johannes; Zachariou, Zacharias; Hohlfeld, Judith; Schwoebel, Marcus; Kistler, Walter; Meuli, Martin; Le Coultre, Claude; Mentha, Gilles; Belli, Dominique Charles; Chardot, Christophe

Published in

Journal of pediatric gastroenterology and nutrition, vol. 46, no. 3, p. 299-307

Publication date

2008

Abstract

OBJECTIVES: To determine the epidemiology of biliary atresia (BA) in Switzerland, the outcome of the children from diagnosis, and the prognostic factors. PATIENTS AND 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.

Keywords

  • Age Factors
  • Biliary Atresia/epidemiology/mortality/surgery
  • Female
  • Humans
  • Incidence
  • Infant
  • Infant, Newborn
  • Kaplan-Meiers Estimate
  • Liver Transplantation/mortality
  • Male
  • Portoenterostomy, Hepatic/mortality
  • Prognosis
  • Retrospective Studies
  • Switzerland/epidemiology
  • Treatment Outcome

UNIGE affiliation entities

UNIGE research groups

Citation (ISO format)

WILDHABER, Barbara et al. Biliary atresia: Swiss national study, 1994-2004. In: Journal of pediatric gastroenterology and nutrition, 2008, vol. 46, n° 3, p. 299–307. doi: 10.1097/MPG.0b013e3181633562

Main files (1)

Article (Accepted version)

Identifiers

Additional URL for this publication

http://ovidsp.ovid.com/ovidweb.cgi?T=JS&NEWS=N&PAGE=fulltext&AN=00005176-200803000-00011&LSLINK=80&D=ovft

Journal ISSN

0277-2116
664views
0downloads

Technical informations

Creation

26/02/2009 14:13:00

First validation

26/02/2009 14:13:00

Update

14/03/2023 15:02:45

Status update

14/03/2023 15:02:45

Last indexation

29/10/2024 11:17:49
All rights reserved by Archive ouverte UNIGE and the University of Geneva
OpenAIRE Indexed & Validated