Evolución respiratoria de 4 pacientes con fibrosis quística receptores de trasplante hepático

Liver disease is the second most common cause of death in patients with cystic fibrosis (CF). Improvement in surgical techniques, medical management, and imaging modalities has broadened the range of options for treatment of these patients. Medical management with ursodeoxycholic acid and nutritional support may help decelerate the progression of liver disease. A timely evaluation of CF patients with liver involvement for transplantation is important. Such evaluation should not be delayed until signs of hepatic decompensation occur. Combined lung-liver transplant can be considered for patients with advanced pulmonary disease. Pretransplant management of portal hypertension with a portosystemic shunt procedure is an option for patients with well-preserved synthetic liver function. Improvement in lung function after liver transplantation and no significant risk of pulmonary infection with immunosuppressive therapy have been reported. Review of individual center experiences have shown satisfactory survival and improved quality of life for CF patients undergoing liver transplant.

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Liver transplantation in cystic fibrosis

Genyk

Quiros²,

Jabbour³

et al. 2001

Current Opinion in Pulmonary Medicine

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Liver and combined lung and liver transplantation for cystic fibrosis: Analysis of the UNOS database

Arnon

Annunziato

Miloh

et al. 2011

Pediatric Transplantation

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A proportion of patients with CF develop cirrhosis and portal hypertension. LT and combined LLT are rarely performed in patients with CF. To determine the outcome of LT and LLT in patients with CF. Patients with CF who had LT or LLT between 10/1987 and 5/2008 were identified from UNOS database. A total of 182 children (<18 yr) and 48 adults underwent isolated LT for CF. Seven more children and eight adults with CF underwent combined LLT. One- and five-yr patient and graft survival were not significantly different in patients who underwent LT in comparison with patients who underwent LLT (patient survival: LT; 83.9%, 75.7%, LLT; 80%, 80%; graft survival: LT; 76.1%, 67.0%, LLT; 80.0%, 80.0%, respectively). The two major causes of death after LT were pulmonary disease (15 patients, 22.7%) and hemorrhage (12 patients, 18.2%). Bilirubin was identified as a risk factor for death, and previous liver transplant and prolonged cold ischemic time were identified as risk factors for graft loss in LT patients. LT is a viable option for children and young adults with CF and end-stage liver disease. Outcome of LLT patients with CF was comparable to the outcome of patients with CF who underwent isolated LT.

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Liver transplant in cystic fibrosis: a poll among European centers. A study from the European Liver Transplant Registry

et al. 2006

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Summary Liver Transplant (LTx) has been rarely performed in cystic fibrosis (CF) patients and indications and outcomes are not well defined. A questionnaire was sent to all European CF and LTx centers to collect data on CF transplanted patients. We obtained information regarding 57 CF patients. LTx has been performed prevalently in males and in pediatric age. The main complication of cirrhosis was portal hypertension with hypersplenism. In the majority of cases the decision to transplant was based on the contemporary presence of various factors. Post‐LTx survival was high and comparable with that expected for more common pediatric LTx indications. Poor respiratory function was the main risk factor for early death. In the short‐term, respiratory function significantly improved after LTx. LTx is the appropriate treatment for patients with advanced CF‐related liver disease and preserved pulmonary function (Forced Expiratory Volume at 1 s, FEV1 >50%). This poll reveals that most European liver centers perform LTx prior to the development of end‐stage liver disease or overt pulmonary or other clinical decompensation.

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Evolución respiratoria de 4 pacientes con fibrosis quística receptores de trasplante hepático

Cited by 3 publications

References 10 publications

Liver transplantation in cystic fibrosis

Liver transplantation in cystic fibrosis

Liver and combined lung and liver transplantation for cystic fibrosis: Analysis of the UNOS database

Liver transplant in cystic fibrosis: a poll among European centers. A study from the European Liver Transplant Registry

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