As survival has improved with liver transplantation (LT), long-term health outcomes have become more important. Reported 5-year survival rate after pediatric LT in North America is >85%. More data on long-term health consequences are provided in a review of 167 10-year survivors from a North American Database (Studies of Pediatric Liver Transplantation –SPLIT) (J Pediatr 2012; 160: 820-6).
Ng VL et al report on frequency of comorbidities as well as quality of life. Of the 10-year survivors who were included in this study: 85 (50.9%) were transplanted in the first year of life; 69 (41.3%) received transplants between 1-7.9 years. Biliary atresia accounted for 55.1% of the transplanted cohort; the remainder were due to the following: metabolic liver disease 23 (13.8%), acute liver failure 18 (10.8%), other cholestatic conditions 17 (10.2%), tumor 6 (3.6%), and other 11 (6.6%).
First allograft survival rates were 94% at 1 year and 88% at 10 years. Health-related quality of life (HRQOL) as assessed by the PedsQL 4.0 Generic Core Scales revealed lower patient self-reported total scale scores for LT survivors compared with healthy children (77.2 vs 84.9, P<.001). 14% had HRQOL >2 SDs below that of a matched healthy population. Other specific post-LT morbidities included the following:
- Impaired linear growth (23% <10th percentile); ongoing steroid therapy was associated with increased risk of poor linear growth.
- Renal dysfunction (9%) –defined as calculated glomerular filtration rate <90 mL/min/1.73 m2.
- Hyperlipidemia: 20% with hypercholesterolemia, and 26% with hypertriglyciridemia
- Lymphoproliferative disease (5%). EBV seroconversion occurred in 46 (47%) of 97 who had been EBV-negative prior to LT. 25 (15%) developed symptomatic EBV infection.
- School performance: 32 (23%) had repeated a grade or were held back at least 1 school year.
- Liver fibrosis: at 10 years, elevated aminotransferases were noted in 11% and increased gamma gluatmyl transpeptidase in 15%. Previous studies from SPLIT indicate fibrosis is common in long-term survivors even with good clinical outcomes.
Alive and well? While survival has improved remarkably, better outcomes are still needed.
- -Pediatrics 2008; 122: 1128-35. Outcomes of 461 pediatric LT.
- -Am J Transplant 2008; 8: 2506-13. Improving long-term outcomes of LT.
- -Hepatolog 2009; 49: 880-6. LT-Liver fibrosis at 10 year followup.
- -JPGN 2008; 47: 165. ~50% below 1.3 SD of adult height. Many show partial catch up growth.
- -Liver Transplant 2006; 12: 1310. Review article on nutrition for OLTx patient.