Premium
Transplantation for cystic fibrosis: Outcome following early liver transplantation
Author(s) -
MILKIEWICZ PIOTR,
SKIBA GRZEGORZ,
KELLY DEIRDRE,
WELLER PETER,
BONSER ROBERT,
GUR UFUK,
MIRZA DARIUS,
BUCKELS JOHN,
STABLEFORTH DAVID,
ELIAS ELWYN
Publication year - 2002
Publication title -
journal of gastroenterology and hepatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.214
H-Index - 130
eISSN - 1440-1746
pISSN - 0815-9319
DOI - 10.1046/j.1440-1746.2002.02671.x
Subject(s) - medicine , liver transplantation , cystic fibrosis , transplantation , gastroenterology , liver disease , surgery , pulmonary function testing , lung transplantation , liver function , liver function tests
Background and Aim Life expectancy in patients with cystic fibrosis (CF) has recently improved due to numerous factors, including a multidisciplinary approach to their management. Prolonged survival may have led to an increasing impact of liver disease on the prognosis of CF patients. The aim of this study was to assess the role of liver transplantation in patients with CF. Methods The factors influencing outcome in 24 patients (15 adults and nine children) with CF who have received single liver transplantation, triple heart‐lung‐liver transplantation (tx) or died while being assessed for triple grafting, were analyzed. Results Median age at tx in single liver recipients (13 years) was lower than in triple graft recipients (21 years) and those who died (23 years). All patients who received single liver tx made an excellent recovery, including significant improvement of their respiratory function (mean forced vital capacity (FVC) increased from 61% before transplantation to 82% of expected, 6–9 months after tx). Four out of five patients who received triple tx died (0–2 months) after operation. On the basis of our retrospective review, we propose modifications to an existing scoring system for liver tx assessment in CF by scoring additional points for elevated white blood count, bilirubin, and impaired pulmonary function. These changes will need to be evaluated prospectively to confirm their predictive value. Conclusions Liver transplantation is effective therapy in young patients with cystic fibrosis, portal hypertension and hepatic dysfunction, and is indicated before a critical stage of deteriorating lung function is reached. In patients with both end‐stage liver and lung disease, triple tx has a poor prognosis. Pre‐emptive liver tx in younger patients with CF not only has a better outcome but improves lung function.