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Liver transplantation for hepatic cirrhosis in cystic fibrosis
G Noble-Jamieson1, J Valente, N D Barnes
1Department of Paediatrics and Surgery, Addenbrooke's NHS Trust, Cambridge.
Insights
Liver transplantation offers significant benefits for children with cystic fibrosis and hepatic cirrhosis, improving nutrition and overall health. This procedure was well-tolerated, with stable or improved respiratory function post-transplant.
Area of Science:
- Pediatric Gastroenterology
- Hepatology
- Transplant Surgery
- Cystic Fibrosis Research
Background:
- Cystic Fibrosis (CF) can lead to severe complications, including hepatic cirrhosis and portal hypertension.
- Portal hypertension in CF patients often presents with gastrointestinal bleeding, posing significant risks.
- Limited treatment options exist for advanced liver disease in pediatric CF patients.
Purpose of the Study:
- To evaluate the safety and efficacy of liver transplantation in children with cystic fibrosis and hepatic cirrhosis.
- To assess the impact of liver transplantation on respiratory function, nutritional status, and overall health in this patient population.
- To analyze early and long-term postoperative outcomes, including complications and survival rates.
Main Methods:
- Retrospective analysis of five pediatric patients with cystic fibrosis and hepatic cirrhosis who underwent liver transplantation.
- Assessment of pre-transplant conditions including portal hypertension, variceal bleeding, respiratory function, and infections.
- Monitoring of postoperative complications, respiratory status, infection rates, nutritional status, and long-term follow-up.
Main Results:
- Liver transplantation was well-tolerated in all five pediatric patients.
- No increase in early postoperative respiratory or other complications was observed.
- Four patients showed sustained well-being 14-35 months post-transplant, with improved nutrition and general health; one experienced biliary stricture issues.
- Respiratory function remained stable or improved despite immunosuppression, with no increase in infections.
Conclusions:
- Liver transplantation is a viable and beneficial treatment option for children with cystic fibrosis complicated by hepatic cirrhosis.
- The procedure leads to significant improvements in nutritional status and general health, with stable or improved respiratory function.
- Long-term outcomes are promising, highlighting the potential of transplantation to enhance quality of life in this challenging patient group.
Abstract:
Five children with cystic fibrosis complicated by hepatic cirrhosis received liver grafts. They all had portal hypertension with varices and three had variceal bleeding; respiratory function was only moderately impaired, but four were colonised with pseudomonas and one with aspergillus. Liver transplantation was well tolerated and there was no increase in respiratory or other early postoperative complications. Four of the children were fully well from 14 to 35 months after transplantation; the most recently transplanted had problems from a biliary stricture. In spite of the need for immunosuppression there was no increase in infection and respiratory function improved or remained stable. Once the children were stabilised after transplantation their nutrition and general health were greatly improved.