Liver transplantation for cystic fibrosis in adults.
OBJECTIVE
To expand our knowledge on liver transplantation for cirrhosis associated with cystic fibrosis in adults.


METHODS
Five patients who underwent a liver transplantation due to cystic fibrosis were reviewed. The outcome of the patients in terms of age, immunosuppression regimen, patient and graft survival, and pre- and post-transplant complications were investigated.


RESULTS
Five adult liver transplant patients had cystic fibrosis (0.2%). These included 4 men and 1 woman with a mean age of 31 +/- 10, ranging from 22 to 52 years old at the time of transplantation. All patients had lung problems. Four patients had exocrine and two had endocrine pancreatic insufficiency. Two are currently alive with a follow-up of 5.8 years and 4 months after transplantation, respectively. There were three deaths from pulmonary embolism at 4.5 years, myocardial infarction with cyclosporine nephrotoxicity at 10.7 years, and lymphoproliferative disorder at 5 months after transplantation. No deaths occurred from lung infection. Only one patient had postoperative pulmonary infectious complications, which were successfully treated with antibiotics and did not result in mortality.


CONCLUSIONS
Adult liver transplantation for end-stage liver disease associated with cystic fibrosis offers encouraging results with a rapid general improvement after surgery and it is now considered to be a safe and acceptable treatment for this disease population.
Ikegami
Toru
T
Baylor University Medical Center, Transplantation Services, 3500 Gaston Avenue, Dallas, TX 75246, USA.
Sanchez
Edmund Q
EQ
Uemura
Tadahiro
T
Narasimhan
Gomathy
G
Masannat
Ousama
O
Chinnakotla
Srinath
S
McKenna
Gregory J
GJ
Randall
Henry B
HB
Levy
Marlon F
MF
Goldstein
Robert M
RM
Klintmalm
Goran B
GB
D000328:Adult; D003550:Cystic Fibrosis / Q000150:complications / Q000401:mortality / Q000601:surgery*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D017093:Liver Failure / Q000209:etiology / Q000401:mortality / Q000601:surgery*; D016031:Liver Transplantation / Q000379:methods*; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D015996:Survival Rate; D013781:Texas / Q000453:epidemiology; D013997:Time Factors; D016896:Treatment Outcome