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.
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.
Authors: R Chavez-Cartaya; M Drage; N V Jamieson; P Friend; G Noble-Jamieson; R Levy; N Barnes; R Calne Journal: Transplant Proc Date: 1999-09 Impact factor: 1.066
Authors: G Noble-Jamieson; J Valente; N D Barnes; P J Friend; N V Jamieson; A Rasmussen; R Y Calne Journal: Arch Dis Child Date: 1994-10 Impact factor: 3.791
Authors: Jonathan A Fridell; Geoffrey J Bond; George V Mazariegos; David M Orenstein; Ashokkumar Jain; Rakesh Sindhi; Jonathan D Finder; Ernesto Molmenti; Jorge Reyes Journal: J Pediatr Surg Date: 2003-08 Impact factor: 2.545
Authors: Wen Ye; Michael R Narkewicz; Daniel H Leung; Wikrom Karnsakul; Karen F Murray; Estella M Alonso; John C Magee; Sarah Jane Schwarzenberg; Alexander Weymann; Jean P Molleston Journal: J Pediatr Gastroenterol Nutr Date: 2018-01 Impact factor: 2.839