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

Abstract

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.

Liver disease in adult patients with cystic fibrosis: A frequent and independent prognostic factor associated with death or lung transplantation

“…However, colonization with B. cepacia and the number of IV antibiotic courses per year reflected a more severe pulmonary disease, as probably did severe genotype and age at diagnosis. Male gender has been reported to be associated with CFLD [4,14,19,[23][24][25]. A protective role of hormonal factors has been hypothesized to explain this difference.…”

Cystic Fibrosis Liver Disease: To Transplant or Not to Transplant?

“…Some small series have however advocated 'preemptive' liver transplantation (12), suggesting that this improves lung function as reported previously (6,25). Such studies, however, mainly examined pediatric cohorts and presented only short-term follow-up.…”

Evolution of indications and results of liver transplantation in Europe. A report from the European Liver Transplant Registry (ELTR)

“…Along with reports concerning LT for specific hepatic diseases [3][4][5][6][7][8][9][10][11][12], ELTR has allowed the development of risk models for livertransplantation mortality according to the characteristics of the donor and recipient, and of the transplant procedure [13,14].…”