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Liver transplantation for hepatic cirrhosis in cystic fibrosis
G Noble-Jamieson1, N Barnes, N Jamieson
1Department of Paediatrics, Addenbrooke's NHS Trust, Cambridge, England.
Insights
A scoring system can identify children with cystic fibrosis (CF) and liver disease eligible for liver transplantation. This procedure improves portal hypertension, nutrition, and respiratory function, significantly enhancing quality of life.
Area of Science:
- Pediatric Gastroenterology
- Hepatology
- Pulmonology
Background:
- Cystic Fibrosis (CF) can lead to hepatic cirrhosis and portal hypertension in 10% of children.
- Worsening portal hypertension causes variceal bleeding, malnutrition, and respiratory decline in pediatric CF patients.
- Pulmonary function can decrease by 50% annually, leading to frequent hospitalizations.
Purpose of the Study:
- To introduce a scoring system for selecting pediatric CF patients with liver disease for liver transplantation assessment.
- To demonstrate that liver transplantation can be successful even in patients with lung function as low as 30% predicted.
Main Methods:
- Development and validation of a simple scoring system to identify suitable candidates.
- Careful pre-transplant assessment and preparation of selected pediatric patients.
Main Results:
- Successful liver transplantation outcomes achieved in children with lung function indices as low as 30% predicted.
- Relief of portal hypertension post-transplantation leads to improved absorption and nutrition.
- Significant improvement in respiratory function and overall quality of life observed after successful liver transplantation.
Conclusions:
- The proposed scoring system aids in selecting pediatric CF patients with liver disease for liver transplantation.
- Liver transplantation is a viable and beneficial treatment option for improving outcomes in this complex patient group.
- Improved graft function post-transplantation leads to multifaceted health benefits, including respiratory and nutritional support.
Abstract:
About 10% of children with CF develop hepatic cirrhosis and progressive portal hypertension. As the portal hypertension worsens these children are likely to develop serious variceal bleeding and other complications including malnutrition and a decline in respiratory function. Indices of lung function may fall as much as 50% in a year and chest infections may require frequent admissions to hospital. The respiratory symptoms are often attributed to CF related lung disease and affected children may therefore be considered unsuitable for liver transplantation. We propose a simple scoring system which can help to select patients who should be referred for assessment of liver transplantation. After careful assessment and preparation children with lung function indices as low as 30% predicted can have a successful outcome after liver transplantation. With good graft function portal hypertension is relieved and absorption, nutrition and respiratory function all improve. The improved quality of life of these children is remarkable.