Pediatric transplantationPediatric liver transplantation for cystic fibrosis
Section snippets
Aim
Determine the outcome of cystic fibrosis patients who undergo liver transplantation (OLTx).
Methods
Data prospectively collected on 291 OLTx performed in 265 children from 10/1984 to 12/1999. All patients received cyclosporine or tacrolimus based immunosuppression. Demographic characteristics, pulmonary function tests, growth charts, nutritional status, and pretransplant morbidities were considered.
Results
There were 9 children (8 boys and 1 girl) who were transplanted for end stage liver disease secondary to CF. Five are currently alive. All those who died were <5% for height and weight. Pulmonary complications accounted for three deaths.
Conclusions
CF constitutes an indication for 4% of all pediatric liver transplants. Transplantation should be undertaken promptly after the diagnosis of end stage liver disease. Most patients have a history of variceal bleeds , meconium ileus, and exocrine pancreatic insufficiency Table 1. Poor growth and nutrition were associated with high mortality. Most deaths in our series were related to pulmonary complications, and occurred less than 2 years after transplantation. The presence of preoperative
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