Correlation Factors and a Predictive Model of Serum Ceruloplasmin Normalized Wilson’s Disease

Background: The false increase of ceruloplasmin (Cp) in some Wilson’s disease (WD) patients, which overlaps with those in non-WD liver disease patients, decrease the diagnostic accuracy. The aims of our study was to understand the factors affecting WD patients’ Cp normalization, and develop a model using routine predictors to identify WD patients with ambiguous serum Cp.Results: The mixed effects model analysis which executed in longitudinal study revealed that the WD patients’ Cp normalization were significantly associated with the copper burden and liver function indexes, like urinary copper treated with dimercaptopropansulfonate sodium (P=0.000), aspartate aminotransferase (P=0.011), γ-glutamyltransferase (P=0.000), albumin (P=0.000). Multivariate logistic regression analysis in case-control study showed age (P=0.000) and serum creatine (P=0.000) were independent risk factors associated with WD. Based on their regression coefficients, a simplified WD index was derived: 0.001 × age [yr] × Creatine [umol/L]. The AUC value of WD index in total cohort were 0.923 (P=0.000). At a WD index cutoff value of ≤ 1.9 and ≤ 2.5, the positive and negtive predictive value are 88.2% and 89.9% for WD, respectively.Conclusions: The increase of serum Cp in WD patients is related to their excessive copper burden and hepatic injury, common tests can effectively foretell those WD patients with nearly normal serum Cp from other liver injury patients.