Age and Serum Creatinine Can Differentiate Wilson Disease Patients with Pseudonormal Ceruloplasmin

Int J Clin Pract. 2023 Mar 2:2023:9344891. doi: 10.1155/2023/9344891. eCollection 2023.

Abstract

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.

MeSH terms

  • Case-Control Studies
  • Ceruloplasmin / analysis
  • Ceruloplasmin / metabolism
  • Copper / metabolism
  • Creatinine
  • Hepatolenticular Degeneration* / complications
  • Hepatolenticular Degeneration* / diagnosis
  • Humans
  • Longitudinal Studies
  • Retrospective Studies

Substances

  • Ceruloplasmin
  • Copper
  • Creatinine