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Living donor liver transplantation in an infant patient with progressive familial intrahepatic cholestasis along with hepatocellular carcinoma: a case report

Authors :
Jung-Man Namgoong
Shin Hwang
Dae-Yeon Kim
Chul-Soo Ahn
Hyunhee Kwon
Suhyeon Ha
Kyung Mo Kim
Seak Hee Oh
Source :
Korean Journal of Transplantation, Vol 36, Iss 1, Pp 73-78 (2022)
Publication Year :
2022
Publisher :
Korean Society for Transplantation, 2022.

Abstract

Progressive familial intrahepatic cholestasis (PFIC) is an autosomal recessive inherited disease requiring liver transplantation (LT). Hepatocellular carcinoma (HCC) is very rare in infants. We present a case of living donor LT using a left lateral section graft performed in a 7-month-old female infant diagnosed with PFIC type II and HCC. No mutation on ABCB11 gene was identified. Because of progressive deterioration of liver function, living donor LT with her mother’s left lateral section graft was performed. Pretransplant serum alpha-fetoprotein (AFP) level was increased to 2,740 ng/mL, but HCC was not taken into account because of its rarity. The explant liver showed micronodular liver cirrhosis, multiple infantile hemangiomas and two HCCs of 0.7 cm and 0.3 cm in size. The patient recovered uneventfully from the LT operation. This patient has been regularly followed up with abdomen ultrasonography and AFP measurement every 6 months. The patient has been continually doing well for 8 years after the LT. In conclusion, LT is currently the only effective treatment for PFIC-associated end-stage liver diseases. HCC can develop at the cirrhotic liver of any cause, thus elevation of HCC tumor markers in pediatric patients is an important clue to perform further investigation before LT.

Details

Language :
English
ISSN :
26718790
Volume :
36
Issue :
1
Database :
Directory of Open Access Journals
Journal :
Korean Journal of Transplantation
Publication Type :
Academic Journal
Accession number :
edsdoj.48c2c08a93b4c4ca5fade2cbbf3f95c
Document Type :
article
Full Text :
https://doi.org/10.4285/kjt.21.0007