Kuiken, N.S.S. (2012) Growth dependent survival in cholestatic infants listed for liver transplantation. thesis, Medicine.
Full text available on request.Abstract
Background: Liver transplantation has become the standard therapy for children with end stage cholestatic liver disease (ESCLD). More than half of all pediatric liver transplantations are performed in infants younger than 24 months. Although long-term survival rate after transplantation exceeds 80%, mortality is high in those awaiting liver transplantation. Infants with ESCLD are severely jaundiced, have portal hypertension leading to ascites, hypersplenism and risk of esophageal variceal bleeding, and may develop life-threatening systemic infections. Maintaining adequate growth under these circumstances is a challenging objective. We aimed to identify predictors of death in infants with ESCLD awaiting liver transplantation and included the impact of growth failure. Methods: In this single center study we collected data of all infants with ESCLD who were listed for a first transplant before the age of 24 months. Primary outcome was death before transplantation. Multivariate logistic regression analysis was used to identify factors associated with pre-transplantation mortality. Results: Between January 2000 and December 2010 a total of 62 consecutive infants with ESCLD were included. Biliary atresia was diagnosed in 94%. Thirteen infants (21%) died while awaiting transplantation. Significant predictors at the time of listing were total serum bilirubin, blood type B (Odds ratio (OR) 17.78; 95%CI 1.94-163.32) and primary listing without hepatoportoenterostomy (HPE) (OR 17.16; 95%CI 1.87-157.43). In infants who were not transplanted within the first three months after listing, growth failure was a significant predictor of death before transplantation (OR 51.17; 95%CI 1.07-2444.50), next to total serum bilirubin. Conclusions: Significant predictors of waiting list mortality in the Netherlands included high total serum bilirubin, blood type B and primary listing without HPE. In infants not transplanted within 3 months after listing growth failure is of significant importance. Early transplantation with a living related donor organ, or early aggressive nutritional intervention to maintain growth could help to improve the outcome of infants with ESCLD.
| Item Type: | Thesis (Thesis) |
|---|---|
| Supervisor name: | Supervisor: and Rheenen, P.F. van and Department of Pediatric Gastroenterology and Beatrix children’s hospital and University Medical Center Groningen |
| Faculty: | Medical Sciences |
| Date Deposited: | 25 Jun 2020 10:46 |
| Last Modified: | 25 Jun 2020 10:46 |
| URI: | https://umcg.studenttheses.ub.rug.nl/id/eprint/734 |
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