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Liver transplantation for congenital hepatic fibrosis
- Source :
- Transplant International. 34:1281-1292
- Publication Year :
- 2021
- Publisher :
- Frontiers Media SA, 2021.
-
Abstract
- Congenital hepatic fibrosis (CHF) is a hereditary fibrocystic disease that can progress to portal hypertension and recurrent cholangitis requiring liver transplantation (LT). It can be associated with renal pathology and need for kidney transplantation (KT). We describe the clinical characteristics and outcomes of patients undergoing liver transplantation alone (LTA) and simultaneous liver-kidney transplantation (SLKT) for CHF using the Unites States Scientific Registry of Transplant Recipients. A total of 197 patients who received LT for CHF between 2002 and 2018 were identified - 87 (44.2%) received SLKT, 110 (55.8%) received LTA. The 1-, 3- and 5-year patient survival were 99.0%, 96.2% and 94.6%. The 1-, 3- and 5-year liver graft survival were 94.9%, 91.1% and 89.6%. No significant differences in patient or liver graft survival were observed between the SLKT and LTA groups, or between paediatric and adult recipients. 53.3% of patients with CHF necessitating LT also have significant renal disease requiring KT. Kidney graft survival for isolated KT prior to LT were poorer compared with KT performed simultaneously or after LT. Both LTA and SLKT for CHF are associated with excellent long-term outcomes in paediatric and adult patients.
- Subjects :
- Adult
Liver Cirrhosis
medicine.medical_specialty
medicine.medical_treatment
030230 surgery
Liver transplantation
Gastroenterology
03 medical and health sciences
0302 clinical medicine
Internal medicine
medicine
Polycystic kidney disease
Humans
Child
Kidney transplantation
Retrospective Studies
Transplantation
Kidney
business.industry
Graft Survival
Genetic Diseases, Inborn
medicine.disease
Liver Transplantation
Treatment Outcome
medicine.anatomical_structure
Renal pathology
Congenital hepatic fibrosis
Portal hypertension
030211 gastroenterology & hepatology
business
Subjects
Details
- ISSN :
- 14322277 and 09340874
- Volume :
- 34
- Database :
- OpenAIRE
- Journal :
- Transplant International
- Accession number :
- edsair.doi.dedup.....a0882a0c6584a8855548dd3719ca1ec4