title: Mortality of biliary atresia in children not undergoing liver transplantation in the Netherlands.
abstract: In order to further improve the outcome of BA, we characterized the mortality of BA patients who did not undergo OLT in the Netherlands, and compared our results with international data. For this purpose, we analyzed the causes of mortality of non-transplanted BA patients before the age of five yr, using the NeSBAR database. To evaluate trends in mortality, we compared the cohort 1987-1996 (n=99) with 1997-2008 (n=111). We compared clinical condition at OLT assessment with available international data, using the PELD-score. Mortality of non-transplanted BA children was 26% (26/99) in 1987-1996 and 16% (18/111) in 1997-2008 (p=0.09). Sepsis was the prevailing direct cause of death (30%; 13/44). PELD-scores at the time of assessment were higher in non-transplanted BA patients (median 20.5; range 13-40) compared with international data (mean/median between 11.7 and 13.3). Based on our national data, we conclude that pretransplant mortality of BA patients is still considerable, and that sepsis is a predominant contributor. Our results strongly indicate that the prognosis of patients with BA in the Netherlands can be improved by earlier listing of patients for OLT and by improving pretransplant care.
authors.affiliation: Department of Pediatric Gastroenterology and Hepatology, University Medical Center Groningen, University of Groningen, Beatrix Children's Hospital, Groningen, The Netherlands.
authors.forename: Willemien
authors.initials: W
authors.lastname: de Vries
authors.forename: Zacharias J
authors.initials: ZJ
authors.lastname: de Langen
authors.forename: Daniel C
authors.initials: DC
authors.lastname: Aronson
authors.forename: Jan B F
authors.initials: JB
authors.lastname: Hulscher
authors.forename: Paul M J G
authors.initials: PM
authors.lastname: Peeters
authors.forename: Pauline
authors.initials: P
authors.lastname: Jansen-Kalma
authors.forename: Henkjan J
authors.initials: HJ
authors.lastname: Verkade
mesh_terms: D000367:Age Factors; D000714:Anastomosis, Surgical; D001656:Biliary Atresia / Q000175:diagnosis / Q000401:mortality* / Q000601:surgery*; D002423:Cause of Death; D002675:Child, Preschool; D002768:Choledochostomy / Q000379:methods / Q000401:mortality; D015331:Cohort Studies; D016208:Databases, Factual; D013505:Digestive System Surgical Procedures / Q000379:methods* / Q000401:mortality*; D018572:Disease-Free Survival; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007583:Jejunum / Q000601:surgery; D053208:Kaplan-Meier Estimate; D008099:Liver / Q000601:surgery; D008297:Male; D009426:Netherlands; D011171:Portoenterostomy, Hepatic / Q000379:methods / Q000401:mortality; D011183:Postoperative Complications / Q000175:diagnosis / Q000401:mortality; D012189:Retrospective Studies; D018570:Risk Assessment; D016019:Survival Analysis; D016896:Treatment Outcome