Outcomes of Acute Liver Injury in Adults Due to Wilson’s Disease: Is Survival Without Transplant Possible?

Abstract

Wilson’s disease (WD) is a rare cause of acute liver failure (ALF) that is thought to have a uniformly fatal outcome without liver transplantation (LT). Previous studies proposed diagnostic and prognostic criteria for WD‐ALF. It is not known whether these apply to WD patients presenting as severe acute liver injury (ALI) without encephalopathy. From 2008 to 2018, 822 patients with ALI in the US Acute Liver Failure Study Group (ALFSG) registry were enrolled and prospectively followed. The diagnosis of WD‐ALI was confirmed in 8 patients. Serum biochemical diagnostic ratios predicting WD‐ALF (alkaline phosphatase [ALP]:total bilirubin(TB) and aspartate aminotransferase [AST]:alanine aminotransferase [ALT]) were determined in these patients, and predictors of prognosis for WD‐ALI were evaluated. Of these 8 ALI‐WD patients, 5 received an LT. Ratios of both ALP:TB of <4 and AST:ALT of >2.2 on study admission were met in 4 LT patients. All LT patients were female. The Model for End‐Stage Liver Disease scores on admission were generally higher in LT patients. All transplanted patients had an initial revised WD score of >11 (>10 predicting poor outcome without LT in WD‐ALF), whereas in non‐LT patients, 2 had scores of 9, and 1 a score of 13. Also, 3 LT patients were started on chelation therapy, 2 were started on plasmapheresis, and 1 was started on Molecular Adsorbent Recirculating System therapy. All non‐LT patients were treated with chelation. At 21 days, all patients were alive and discharged from the hospital. In conclusion, some patients with ALI due to WD may survive without LT. Revised Wilson index scores >10 predict poor outcome in most patients with WD‐ALI, as they do for WD‐ALF, and they correlate positively with the ALI model in this cohort. Biochemical ratios for WD diagnosis appear more applicable to ALF compared with WD‐ALI.

Prognostic Value of the 13C‐Methacetin Breath Test in Adults with Acute Liver Failure and Non‐acetaminophen Acute Liver Injury

“…(5) Over the years, the ALFSG has conducted clinical trials in an effort to improve TFS in both patients with both ALF and ALI. (6,7) The 13 C-methacetin breath test ( 13 C-MBT) is a semiquantitative, noninvasive hepatic metabolic function test that assays the biotransformation of 75 mg of 13 C-labeled methacetin by hepatic cytochrome-P450 1A2 (CYP1A2) to 13 CO 2 and APAP. (8) The 13 C-MBT has been shown to be more accurate than the Model for End-Stage Liver Disease (MELD) score in predicting decompensation and survival in patients with cirrhosis undergoing LT evaluation.…”

Wilson's disease: Revisiting an old friend

“…When a mutation affects the ATP7B transporter, free copper is released into the bloodstream and is removed by urine instead of feces[ 2 ]. Therefore, ATP7B is essential for copper biliary excretion[ 3 ].…”

Management of Acute Wilsonian Hepatitis with Severe Hemolysis: A Successful Combination of Chelation and MARS Dialysis

“…The survival benefit of MARS in acute-on-chronic liver failure is not proven [ 23 ]. Based on few case series [ 5 , 20 , 24 ] and clinical practice guidelines on WD from India [ 25 ], MARS dialysis could stabilize the patient's condition and may serve as a bridge to LT. Our observation demonstrates that MARS sessions administered to target massive copper-overload-associated hemolysis reduced the need for repeated blood transfusions and have contributed to the patient's survival without urgent LT.…”