Sijsling, M.I. (2012) Early deterioration of PELD score in young children with biliary atresia predicts poor outcome. thesis, Medicine.
Full text available on request.Abstract
Background: The Pediatric End-stage Liver Disease (PELD) score is designed to prioritize children with biliary atresia for liver transplantation, based on the severity of chronic liver disease. High scores at listing predict poor outcome, including death. Periodic calculation of the PELD score in children awaiting liver transplantation may be an important additional predictor of pre-transplantation mortality. We aimed to determine the PELD change or cut-off score in young children with biliary atresia (BA) on the waiting list that could assist clinicians in identifying those at high risk of dying before transplantation. Methods: A national cohort of children younger than 5 years with BA, screened for liver transplantation between 2000 and 2012 was retrospectively analyzed. PELD scores and change scores were calculated at listing and then bi-monthly until death or liver transplantation. Results: A total of 71 children with BA-associated end-stage liver disease were included, of which 12 (17%) died before transplantation. At the time of listing the optimum PELD score cut point to differentiate high from low risk patients was 21 points. For children with a PELD score of 21 points or higher mortality risk before transplantation increased to 40% (10/25), whereas a score of 20 points or lower reduced this risk to 4% (2/46). Two months after listing, when 47 patients were still waiting for a donor liver, the optimum cut point to differentiate high from low risk patients was 24 points. Children with a score of 24 or higher had a 56% (9/16) risk of death, whereas a score of 23 or lower gave a 0% risk (0/31; 95% confidence interval (CI) 0 to 9%). A deterioration of PELD score of 5 points or more in the first two months after listing indicated a 50% (9/18) risk of death before transplantation, whereas a change score of 4 points or less reduced mortality to 0% (0/29; 95% CI 0 to 10%). Conclusions: Periodic calculation of the PELD score in young children with BA-associated end-stage liver disease awaiting transplantation facilitates recognition of those with a high risk of pre-transplantation demise. Earlier listing of BA patients and/or adaptation of priority rules on the waiting list could help to decrease mortality and thus increase the prognosis of BA.
| Item Type: | Thesis (Thesis) |
|---|---|
| Supervisor name: | Supervisors: and Rheenen, Dr. P.F. van and Verkade, Prof. Dr. H.J. and Department Pediatric Gastroenterology, Hepatology and Nutrit and Organization:Beatrix Children’s Hospital and University Medical Center Groningen |
| Faculty: | Medical Sciences |
| Date Deposited: | 25 Jun 2020 11:03 |
| Last Modified: | 25 Jun 2020 11:03 |
| URI: | https://umcg.studenttheses.ub.rug.nl/id/eprint/2307 |
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