Background and Aim. Significantly reduced serum ceruloplasmin (Cp) is the most important clue in the diagnosis of Wilson’s disease (WD) and is well known to clinicians. The false increase in Cp in some WD patients, which overlaps with that in non-WD liver disease patients, decreases the diagnostic accuracy. The aims of our study were to understand the factors associated with Cp normalisation in WD patients and identify these WD patients using usual predictors. Methods. We retrospectively screened individuals with serum Cp ≥ 140 mg/L from 1032 WD patients who were hospitalised for the first time. Logistic regression analyses were performed in a case-control study between the WD cohort and another liver disease cohort to explore the independent risk factors for WD diagnosis and establish a regression model to identify them. The follow-up medical records of the WD cohort were subjected to mixed-effects model analysis in a longitudinal study to discover factors associated with Cp normalisation. Results. Eighty-six WD patients and their 353 medical records and another 98 non-WD liver disease patients were included in the present study. Cp normalisation was significantly associated with the copper burden and liver function indexes, such as urinary copper, γ-glutamyltransferase, and albumin ( p ≤ 0.001 ). Logistic regression analysis showed that age and serum creatinine ( p ≤ 0.001 ) were independent risk factors associated with WD. The AUC value of the regression model in the total cohort was 0.926 ( p ≤ 0.001 ). At a cutoff value of ≥0.617 and ≥−1, the positive and negative predictive values were both 90.8% for WD. Conclusion. Increased serum Cp in WD patients is related to excessive copper burden and hepatic injury, and common tests can effectively distinguish WD patients from other liver injury patients.

Background and aim: Significantly reduced serum ceruloplasmin (Cp) is the most important clue in the diagnosis of Wilson disease (WD) and is well known to clinicians. The false increase in Cp in some WD patients, which overlaps with that in non-WD liver disease patients, decreases the diagnostic accuracy. The aims of our study were to understand the factors associated with Cp normalization in WD patients and to identify these WD patients using usual predictors. Methods:We retrospectively screened individuals with serum Cp ≥140 mg/L from 1032 WD patients who were hospitalised for the first time. Logistic regression analyses were performed in a case-control study between the WD cohort and another liver disease cohort to explore the independent risk factors for WD diagnosis and establish a regression model to identify them. The follow-up medical records of the WD cohort were subjected to mixed effects model analysis in a longitudinal study to discover factors associated with Cp normalisation. Results:Eighty-six WD patients and 353 of their medical records and another 98 non-WD liver disease patients were included in the present study. Cp normalisation was significantly associated with the copper burden and liver function indexes, such as urinary copper, γ-glutamyltransferase, and albumin (p≤0.001). Logistic regression analysis showed that age and serum creatinine (p≤0.001) were independent risk factors associated with WD. The AUC value of regression model in total cohort was 0.926 (p≤0.001). At a cutoff value of ≥ 0.617 and ≥ -1, the positive and negative predictive values were both 90.8% for WD. Conclusion:Increased serum Cp in WD patients is related to excessive copper burden and hepatic injury, and common tests can effectively distinguish WD patients from other liver injury patients.

Background: Significantly reduced serum ceruloplasmin (Cp) is the most important clue in the diagnosis of Wilson disease (WD) and is well known to clinicians. The false increase of Cp in some WD patients decrease the diagnostic accuracy. The aims of our study was to understand the factors affecting WD patients’ Cp normalization, and identify these WD patients using routine predictors. Methods:We retrospectively screened people with serum Cp ≥140mg/L from 1032 WD patients, and followed their medical records to execute mixed effects model analysis in longitudinal study. Logistic regression analyses were performed in a case-control study between WD cohort and another liver diseases cohort to explore the independent risk factors for Cp normalization and establish a regression model. Results:Eighty-six WD patients and their 352 medical records over more than nine years and another 98 non-WD liver disease patients entered into present study. The Cp normalization were significantly associated with the copper burden and liver function indexes, such as urinary copper, γ-glutamyltransferase, albumin (P< 0.001). Logistic regression analysis showed age and serum creatine (P< 0.001) were independent risk factors associated with WD. Its AUC value of regression model in total cohort was 0.926 (P< 0.001). At a cutoff value of ≥ 0.617 and ≥ -1, the positive and negtive predictive value both are 90.8% for WD, respectively. Conclusion: Increased serum Cp in WD patients is related to excessive copper burden and hepatic injury, common tests can effectively foretell those WD patients from other liver injury patients.