Failure of Simple Biochemical Indexes to Reliably Differentiate Fulminant Wilson's Disease From Other Causes of Fulminant Liver Failure

Abstract

Serum, urine and tissue biochemical findings were studied in 21 cases of fulminant Wilson's disease with respect to the value of a recently described biochemical index based on serum alkaline phosphatase and total serum bilirubin levels, and these cases were compared with 193 other cases of fulminant liver failure. Serum bilirubin, alkaline phosphatase and AST levels found in fulminant Wilson's disease were significantly different from those found in other cases of fulminant liver failure, but differentiation from other causes of fulminant liver failure on the basis of these biochemical parameters was not possible. The alkaline phosphatase/bilirubin and aspartate AST/bilirubin ratios derived from the above parameters were also significantly lower in fulminant Wilson's disease than in other categories of fulminant liver failure, but distinction between diagnostic categories on this basis was not possible. When ratios that correctly identified all cases of fulminant Wilson's disease were selected, 59/190 (31%) and 84/190 (44%) cases of non-Wilsonian fulminant liver failure would erroneously be assigned a diagnosis of fulminant Wilson's disease, by alkaline phosphatase/bilirubin and AST/bilirubin ratios, respectively. A low alkaline phosphatase-to-bilirubin ratio (< 0.57) in any category of fulminant liver failure suggested a significantly worse prognosis than in cases with higher ratios (chi 2, Yates' corrected = 5.37, p = 0.02). In the Wilson's disease group, serum and hepatic copper and ceruloplasmin concentrations were normal in 4/21, 2/15 and 2/19, respectively, whereas urinary copper level was elevated in 18/18 and was the most valuable test in diagnosis.(ABSTRACT TRUNCATED AT 250 WORDS)

Fulminant Wilson???s disease in children: Appraisal of a critical diagnosis

“…Berman et al (5) identified alkaline phosphatase to total bilirubin ratio of Ͻ2.0 and AST to ALT ratio of Ͼ4.0 for FWD diagnosis. Validity of these two indexes was not confirmed by other studies, which indistinctly analyzed patients with fulminant, subfulminant, acute-on-chronic, and chronic Wilson's disease (15,20,21). In our series, AP to total bilirubin ratio as well as AST to ALT ratio showed significant difference between both groups.…”

“…Of the ten published studies, six were issued from the same few centers, and most analyzed indistinctly adult and pediatric populations. Only Sallie and colleagues (21), in a study addressing diagnostic criteria, clearly identified a pediatric and an adult population: Among the 14 children they mentioned, four required ELT (29%) and all survived (100%). This surprisingly elevated survival rate raises a question about the type of studied population.…”

Factors that predict mortality in children with Wilson disease associated acute liver failure and comparison of Wilson disease specific prognostic indices

“…Assessing the prognosis of patients with ALF WD is critical in order to stratify patients who will improve spontaneously with supportive treatment from those who will need a liver transplantation . The couple of reports involving small number of patients from tertiary centers consist of a mix of both pediatric and adult patients, making comparison between series difficult . Two WD‐specific prognostic models have been published in the last two decades .…”

Liver transplantation for Wilson’s disease: Indications and outcome

“…It is crucial that the entity of wilsonian fulminant hepatitis be clinically recognized because of its uniformly fatal outcome if liver transplantation is not performed in a timely fashion (13)(14)(15)(16)(17)(18). In this form of Wilson's disease, the features of hepatic insufficiency are associated with nonimmune hemolysis and, remarkably, with relatively low serum transaminase and alkaline phosphatase activities, considering the extent of hepatic necrosis (13,17,19).…”