scholarly journals Liver Transplantation for Cirrhosis in Cystic Fibrosis

2006 ◽  
Vol 20 (7) ◽  
pp. 475-478 ◽  
Author(s):  
T Lamireau ◽  
S Martin ◽  
M Lallier ◽  
JE Marcotte ◽  
F Alvarez
Keyword(s):  
Cystic Fibrosis ◽  
Liver Transplantation ◽  
Portal Hypertension ◽  
Pulmonary Function ◽  
Hepatic Failure ◽  
Hepatic Transplantation ◽  
Significant Deterioration ◽  
Postoperative Course ◽  
The Third ◽  
Cystic Fibrosis Population

BACKGROUND: Liver disease is the third most common cause of death in children with cystic fibrosis (CF). Liver transplantation is an effective treatment in children with hepatic failure.AIMS: The objective of the present study was to review the indications and postoperative course of hepatic transplantation in a cystic fibrosis population.PATIENTS: Five children with CF, at a mean age of 16.5 years, underwent liver transplantation.RESULTS: All patients showed cirrhosis, portal hypertension and hepatic failure. The main postoperative complication was ascites refractory to treatment in two patients. No significant deterioration of the pulmonary function was noted. Two patients died, one of Hodgkin lymphoma and the other of progressive pulmonary failure.CONCLUSION: Liver transplantation was indicated in children with CF when hepatic failure and/or severe portal hypertension was present with well-preserved pulmonary function.

Orthotopic Liver Transplantation in Patients With Cystic Fibrosis

PEDIATRICS ◽  
1987 ◽  
Vol 80 (4) ◽  
pp. 571-574
Author(s):  
Kenneth L. Cox ◽  
Richard E. Ward ◽  
Terry L. Furgiuele ◽  
Robert A. Cannon ◽  
Kathleen D. Sanders ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Liver Transplantation ◽  
Intracerebral Hemorrhage ◽  
Liver Transplant ◽  
Orthotopic Liver Transplantation ◽  
Postoperative Management ◽  
Sweat Test ◽  
Transplant Recipients ◽  
Postoperative Course ◽  
Liver Transplant Recipients

An 11-year-old boy who had cystic fibrosis underwent an orthotopic liver transplantation. His immediate postoperative course was not unusually complicated when compared with other liver transplant recipients. Transplantation did not correct abnormalities in the sweat test or the respiratory disease. Cholestasis due to obstruction of the recipient duct with tenacious bile was cleared by instilling N-acetylcysteine into the duct. On the 48th day after the transplantation, he died of an intraventricular and intracerebral hemorrhage caused by an Aspergillus brain abscess. We conclude that certain patients with cystic fibrosis may be appropriate candidates for liver transplantation, but their pre- and postoperative management may need to differ from other liver transplant recipients.

203: Combined Lung Liver Transplantation in Cystic Fibrosis Population: A French Experience Update

2009 ◽  
Vol 28 (2) ◽  
pp. S137
Author(s):  
R. Souilamas ◽  
O. Scatton ◽  
P. Sogni ◽  
Y. Calmus ◽  
R. Kanaan ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Liver Transplantation ◽  
Cystic Fibrosis Population ◽  
French Experience

Handgrip Strength and Pulmonary Function in the Pediatric Cystic Fibrosis Population

2021 ◽  
Vol 121 (9) ◽  
pp. A24
Author(s):  
J. Matsunaga ◽  
B. Bice ◽  
M. Jackson ◽  
J. Wells ◽  
M. Timmerman ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Pulmonary Function ◽  
Handgrip Strength ◽  
Cystic Fibrosis Population

Pulmonary function in individuals who underwent liver transplantation: From the US cystic fibrosis foundation registry

2012 ◽  
Vol 18 (5) ◽  
pp. 585-593 ◽  
Author(s):  
Melissa R. Miller ◽  
Ronald J. Sokol ◽  
Michael R. Narkewicz ◽  
Marci K. Sontag
Keyword(s):  
Cystic Fibrosis ◽  
Liver Transplantation ◽  
Pulmonary Function ◽  
Cystic Fibrosis Foundation ◽  
The Us

Biliary Atresia and Orthotopic Liver Transplantation - 11 Years of Experience in Geneva

Swiss Surgery ◽  
2001 ◽  
Vol 7 (5) ◽  
pp. 199-205 ◽  
Author(s):  
Le Coultre ◽  
Battaglin ◽  
Bugmann ◽  
Genin ◽  
Bachmann ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Survival Rate ◽  
Biliary Atresia ◽  
Hepatic Failure ◽  
Initial Treatment ◽  
Inflammatory Process ◽  
Favourable Outcome ◽  
Initial Operation ◽  
Hepatic Transplantation ◽  
Transplantation Program

Biliary atresia (BA) is a congenital malformation or an evolutive inflammatory process which, without treatment, leads to cirrhosis, hepatic failure and death within two years of birth. The literature gives a survival rate of 60% at five years and 25% to adulthood after an initial operation performed for BA. 30% of children do not survive beyond two years of age. BA has become the most frequent indication for liver transplantation (LT) in children. With LT, survival expectancy is 90%. Results of the operation designed for BA remain unsatisfactory, and seem to depend on the age of the infants, as well as on other factors such as liver histology, and centre experience. Since 1989, onset of the paediatric hepatic transplantation program in Geneva, to July 2000, 20 children have been referred for initial treatment of BA, and 26 for possible hepatic transplantation after initial treatment done in another centre. The aim of the current study is to analyse our own results of the initial operation and to present the results of liver transplantation in this particular group of patients. All the patients with a BA are included in this study. The initial operation for BA yielded 43% favourable outcome at five years and the survival in this group following LT reached 91.3% survival. The importance of the age of the patient at time of initial operation is underlined.

Fast Track Recovery Following En Bloc Heart–Lung–Liver Transplantation in a Patient with Cystic Fibrosis Complicated by Severe Portal Hypertension

2008 ◽  
Vol 17 (2) ◽  
pp. 154-156 ◽  
Author(s):  
Igor E. Konstantinov ◽  
Keith McNeil ◽  
Shinn Yeung ◽  
Jonathan Fawcett ◽  
Daniel Mullany ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Liver Transplantation ◽  
Portal Hypertension ◽  
Fast Track ◽  
En Bloc ◽  
Fast Track Recovery
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