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[Isolated hepatic transplant in patient with cystic fibrosis]
M Berenguer1, M Prieto, F Orbis
1Servicio de Medicina Digestiva, Hospital Universitario La Fe, Valencia.
Insights
Cystic fibrosis (CF) patients with liver cirrhosis can benefit from liver transplantation. This procedure improved the patient's nutritional status and lung function, offering a new treatment option.
Area of Science:
- Hepatology
- Pulmonology
- Genetics
Background:
- Cystic fibrosis (CF) is a genetic disorder affecting multiple organs, primarily the lungs and pancreas.
- Liver involvement, including focal biliary cirrhosis and portal hypertension, is increasingly recognized in CF patients due to improved survival.
- The prognosis for CF patients with liver cirrhosis is poor, with high mortality rates within 4-5 years.
Observation:
- Liver involvement in CF is infrequent in young children but increases with age.
- Effective management of pulmonary complications has led to increased survival rates in CF patients.
- A growing number of CF patients are developing liver complications.
Findings:
- Isolated liver transplantation is a viable option for CF patients with liver cirrhosis and stable pulmonary function.
- A 14-year-old CF patient who underwent liver transplantation showed significant improvement in nutritional status and pulmonary function.
- Post-transplant follow-up of 8 months demonstrated positive outcomes for the patient.
Implications:
- Liver transplantation offers a promising therapeutic strategy for CF-associated liver disease.
- Improved pulmonary function post-transplantation suggests a potential systemic benefit beyond liver restoration.
- This case highlights the importance of considering liver transplantation in advanced CF liver disease.
Abstract:
Cystic fibrosis (CF) is a recessive autosomic disease with multiorgan, although predominantly pancreatic and pulmonary, involvement. Liver involvement is infrequent in children under the age of 5 years, but increases progressively with time. It is characterized by the development of focal biliary cirrhosis with eventual appearance of portal hypertension. During the last few years the more effective control of the pulmonary complications, which are the main cause of mortality, has led to an increase in the survival of these patients and thus the number of patients with CF and liver involvement is greater every day. In these cases, the prognosis is bad and most patients die in 4 to 5 years. Isolated liver transplantation is a recently proposed alternative for patients who have developed liver cirrhosis but who maintain acceptable pulmonary function. The case of a 14-years-old patient in whom liver transplantation was performed with good results after 8 months of follow-up is presented. Improvement in the nutritive state and pulmonary function was observed.