Hepatol Res. 2017 Mar;47(4):359-363. doi: 10.1111/hepr.12711. Epub 2016 May 28.
Plasma exchange and chelator therapy rescues acute liver failure in Wilson disease without liver transplantation.
Hepatology research : the official journal of the Japan Society of Hepatology
Jun Kido, Shirou Matsumoto, Ken Momosaki, Rieko Sakamoto, Hiroshi Mitsubuchi, Yukihiro Inomata, Fumio Endo, Kimitoshi Nakamura
Affiliations
Affiliations
- Department of Pediatrics, Graduate School of Medical Sciences, Kumamoto University, Kumamoto City, Japan.
- Department of Transplantation and Pediatric Surgery, Graduate School of Medical Sciences, Kumamoto University, Kumamoto City, Japan.
PMID: 27007780
DOI: 10.1111/hepr.12711
Abstract
AIM: Wilson disease (WD) in patients with a New Wilson Index (NWI) score ≥ 11 is fatal, and these patients are good candidates for liver transplantation (LT). However, plasma exchange and chelator therapy are indispensable and effective even for WD with a score ≥ 11. Moreover, continuous hemodiafiltration (CHDF) with these treatments is essential for acute liver failure (ALF) in WD with hepatic encephalopathy because CHDF can exclude toxic metabolites that may cause damage to the brain. Here, we describe four rescued patients presenting with ALF in WD and discuss the available treatment options.
METHODS: We have experienced 11 male and 8 female patients presenting with WD at the Department of Pediatrics, Kumamoto University Hospital between 1999 and 2014. A male and 4 female patients were diagnosed as WD with ALF using a combination of clinical findings and biochemical tests.
RESULTS: The NWI score was ≥ 11 in cases 1 to 3. Cases 1 and 2 with hepatic encephalopathy received plasma exchange, CHDF, coagulation factor replacement treatment (CFRT) and LT. Cases 3 and 4 without encephalopathy obtained stable status without LT by plasma exchange, blood infusion, and CFRT.
CONCLUSIONS: It is better to undergo LT for WD patients with a NWI score ≥ 11, however, there is a possibility of remission by plasma exchange and medical therapy even without LT. WD patients with a NWI score ≥ 11can be rescued by conservative therapy when the ALF of WD does not present with ALF and hepatic encephalopathy. Therefore, ALF with hepatic encephalopathy itself is an indication for LT in WD.
© 2016 The Japan Society of Hepatology.
Keywords: Continuous hemodiafiltration; New Wilson Index; prothrombin time
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