Diagnostic Dilemma and Treatment Outcome in Acute Liver Failure Due to Wilson's Disease

Abstract

BACKGROUND Wilson's disease (WD) manifesting as acute liver failure (ALF) is a life-threatening condition, and spontaneous recovery is rare. Diagnostic scores like the alkaline phosphatase elevation/total bilirubin elevation ratio and aspartate aminotransferase/alanine aminotransferase ratio can distinguish WD from other ALF etiologies. Liver transplantation plays a major role in treating these patients, and the revised Wilson Index is useful in patient selection for this procedure. The aim of this study was to evaluate diagnostic scores, treatments, and outcomes of a large cohort of patients with WD-ALF. MATERIAL AND METHODS Twenty adult patients of a historical cohort admitted from January 2001 to December 2017 were prospectively observed. Demographic, clinical, laboratory, and radiology data, and treatment, time on the waiting list for liver transplantation, and outcomes were recorded. RESULTS No diagnostic laboratory scores were 100% positive in patients with WD-ALF. Cut-off values for the alkaline phosphatase/total bilirubin ratio and aspartate aminotransferase/alanine aminotransferase ratio were met by 65.0% and 80.0% of patients, respectively. All patients met at least 1 criterion for high risk of death (Nazer or revised Wilson Index) and qualified for liver transplantation. In 9 patients, albumin dialysis was used before surgery. Survival after liver transplantation was 85.0% and 74.4% after 1 month and 1 year, respectively. CONCLUSIONS Further research on a novel diagnostic score in WD-ALF is warranted. Adult patients suspected to have WD as the cause of ALF should be treated in the referral liver transplantation unit. Liver transplantation makes long-term survival possible for patients with this critical illness.

Figure 1

Kaplan-Meier survival analysis according to etiology of acute liver failure (Wilson’s disease vs non-Wilson’s disease).