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Plasma exchange and chelator therapy rescues acute liver failure in Wilson disease without liver transplantation
Author(s) -
Kido Jun,
Matsumoto Shirou,
Momosaki Ken,
Sakamoto Rieko,
Mitsubuchi Hiroshi,
Inomata Yukihiro,
Endo Fumio,
Nakamura Kimitoshi
Publication year - 2017
Publication title -
hepatology research
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.123
H-Index - 75
eISSN - 1872-034X
pISSN - 1386-6346
DOI - 10.1111/hepr.12711
Subject(s) - medicine , hepatic encephalopathy , liver transplantation , encephalopathy , gastroenterology , liver disease , transplantation , hyperammonemia , disease , surgery , cirrhosis
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.