Premium
Recovery of severe acute liver failure without transplantation in patients with Wilson disease
Author(s) -
Kido Jun,
Matsumoto Shirou,
Sakamoto Rieko,
Mitsubuchi Hiroshi,
Inomata Yukihiro,
Nakamura Kimitoshi
Publication year - 2018
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/petr.13292
Subject(s) - medicine , liver transplantation , refractory (planetary science) , cirrhosis , surgery , esophageal varices , wilson's disease , transplantation , encephalopathy , liver disease , hyperammonemia , chronic liver disease , portal hypertension , disease , gastroenterology , physics , astrobiology
Wilson disease ( WD ) is a disorder of copper metabolism that leads to liver cirrhosis. WD patients with a NWIS > 11 should receive LT ; however, we encountered 2 WD patients with an NWIS > 11 who recovered from ALF without LT . The present report aimed to analyze cases of WD patients with a high NWIS who recovered from severe ALF and to discuss the clinical manifestations of the patients and the effects of treatments, including zinc (Zn) therapy, chelator therapy, PE, CHDF, and LT . We retrospectively evaluated the medical records of five patients (male, 2; female, 3) diagnosed with WD along with severe ALF . In cases 1, 2, and 3, complete recovery from ALF was noted without LT . In case 4, initial recovery from ALF was noted without LT ; however, ALF worsened owing to bleeding from the esophageal varix. Thus, the patient eventually needed LT . In case 5, recovery from ALF was noted with LT . All cases, except case 2, showed ALF with maximum PELD/MELD scores ≥26 and NWIS s ≥ 11, and had indications for LT . In cases of severe ALF with grade I or II encephalopathy, we recommend evaluations of the effects of Zn and chelator treatments while preparing for LT , as the condition may not improve without LT , and pediatricians or physicians can ask transplant surgeons to perform LT urgently if required.