Document Detail

Pediatric liver transplantation for fibropolycystic liver disease.
MedLine Citation:
PMID:  22360404     Owner:  NLM     Status:  In-Data-Review    
Ko JS, Yi N-J, Suh KS, Seo JK. Pediatric liver transplantation for fibropolycystic liver disease. Pediatr Transplantation 2012: 16: 195-200. © 2012 John Wiley & Sons A/S. Abstract:  Fibropolycystic liver disease includes CHF, Caroli's syndrome, and Caroli's disease. Patients with Caroli's disease and Caroli's syndrome have an increased risk of recurrent cholangitis, intrahepatic calculi, biliary cirrhosis, and cholangiocarcinoma. The aim of this study was to examine the post-transplantation outcomes of children with fibropolycystic liver disease. Of the 158 children transplanted at Seoul National University Hospital, there were four patients with Caroli's syndrome, two patients with CHF, and one patient with Caroli's disease. One patient underwent combined liver/kidney transplantation. Associated renal manifestations included ARPKD in three children and nephronophthisis in one child. The indications for LT were recurrent cholangitis, decompensated cirrhosis, and refractory complications of portal hypertension. Both graft and patient survival rates were 100% at a median follow-up period of two yr after LT. Three children with growth failure achieved catch-up growth after LT. In three patients with ARPKD, mean serum creatinine levels increased from 0.53 mg/dL at the time of LT to 0.91 mg/dL at the last follow-up (p = 0.01). LT is an excellent option for children with complications from fibropolycystic liver disease. Renal function should be monitored cautiously after LT in the patients with ARPKD.
Jae Sung Ko; Nam-Joon Yi; Kyung Suk Suh; Jeong Kee Seo
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Pediatric transplantation     Volume:  16     ISSN:  1399-3046     ISO Abbreviation:  Pediatr Transplant     Publication Date:  2012 Mar 
Date Detail:
Created Date:  2012-02-24     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9802574     Medline TA:  Pediatr Transplant     Country:  Denmark    
Other Details:
Languages:  eng     Pagination:  195-200     Citation Subset:  IM    
Copyright Information:
© 2012 John Wiley & Sons A/S.
Department of Pediatrics Surgery, Seoul National University College of Medicine, Seoul, South Korea.
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