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Pediatric liver transplantation for fibropolycystic liver disease
Author(s) -
Ko Jae Sung,
Yi NamJoon,
Suh Kyung Suk,
Seo Jeong Kee
Publication year - 2012
Publication title -
pediatric transplantation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.457
H-Index - 69
eISSN - 1399-3046
pISSN - 1397-3142
DOI - 10.1111/j.1399-3046.2012.01661.x
Subject(s) - medicine , liver transplantation , congenital hepatic fibrosis , cirrhosis , transplantation , gastroenterology , liver disease , portal hypertension , kidney disease , hepatorenal syndrome , biliary cirrhosis , disease , autoimmune disease
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.