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PubMed ID:
18798336
Public Release Type:
Journal
Publication Year: 2008
Affiliation: Montefiore Medical Center, Bronx, NY, USA.
DOI:
https://doi.org/10.1002/hep.22446
Authors:
Hassenein T,
Campbell M,
Fontana RJ,
Barakat F,
Smith A,
Squires RH Jr,
Schiodt FV,
Webster J,
Balko J,
Volenberg I,
Korman JD,
Lee WM,
Schilsky ML,
Pediatric and Adult Acute Liver Failure Study Groups,
Lee WM,
Polson J,
Pezzia C,
Lalani E,
Hynan LS,
Reisch JS,
Larson AM,
Do H,
Crippin JS,
Gerstle L,
Davern TJ,
Partovi K,
Emre S,
McCashland TM,
Bernard T,
Hay J,
Groettum C,
Murray N,
Coultrup S,
Shakil AO,
Morton D,
Blei AT,
Gottstein J,
Zaman A,
Schwartz J,
Ingram K,
Han S,
Peacock V,
Fontana RJ,
Welch S,
McGuire B,
Avant L,
Chung R,
Casson D,
Brown R Jr,
Schilsky M,
Senkbeil L,
Harrison ME,
Rush R,
Reuben A,
Huntley N,
Munoz S,
Misra C,
Stravitz T,
Salvatori J,
Rossaro L,
Prosser C,
Satyanarayana R,
Taylor W,
Reddy R
Studies:
Acute Liver Failure Study Group: Adult Acute Liver Failure Study
Acute liver failure (ALF) due to Wilson disease (WD) is invariably fatal without emergency liver transplantation. Therefore, rapid diagnosis of WD should aid prompt transplant listing. To identify the best method for diagnosis of ALF due to WD (ALF-WD), data and serum were collected from 140 ALF patients (16 with WD), 29 with other chronic liver diseases and 17 with treated chronic WD. Ceruloplasmin (Cp) was measured by both oxidase activity and nephelometry and serum copper levels by atomic absorption spectroscopy. In patients with ALF, a serum Cp <20 mg/dL by the oxidase method provided a diagnostic sensitivity of 21% and specificity of 84% while, by nephelometry, a sensitivity of 56% and specificity of 63%. Serum copper levels exceeded 200 microg/dL in all ALF-WD patients measured (13/16), but were also elevated in non-WD ALF. An alkaline phosphatase (AP) to total bilirubin (TB) ratio <4 yielded a sensitivity of 94%, specificity of 96%, and a likelihood ratio of 23 for diagnosing fulminant WD. In addition, an AST:ALT ratio >2.2 yielded a sensitivity of 94%, a specificity of 86%, and a likelihood ratio of 7 for diagnosing fulminant WD. Combining the tests provided a diagnostic sensitivity and specificity of 100%.
