Risk factor analysis and nomogram development for advanced-stage hepatic fibrosis in patients with Wilson's disease

Area of Science:

• Hepatology
• Internal Medicine
• Genetics

Background:

• Wilson's disease (WD) is a genetic disorder of copper metabolism.
• Advanced hepatic fibrosis is a serious complication of WD, necessitating early detection.
• Current screening methods for advanced fibrosis in WD require improvement.

Purpose of the Study:

• To investigate risk factors for advanced-stage hepatic fibrosis in Wilson's disease.
• To develop a predictive nomogram for early screening of high-risk WD patients.
• To facilitate timely prevention and intervention strategies for advanced hepatic fibrosis in WD.

Main Methods:

• Retrospective analysis of clinical data from 221 WD patients.
• Identification of independent risk factors using univariate and multivariate Cox regression.
• Development and validation of a clinical predictive nomogram using ROC, calibration curves, and DCA.

Main Results:

• CER, LN, HDL-C, TG, PLT, Sex, and Apo-A1 were identified as independent risk factors.
• The nomogram showed excellent discriminative capacity (AUC > 0.90 in training and validation cohorts).
• Calibration curves confirmed strong agreement between predicted and observed outcomes.

Conclusions:

• The developed nomogram is a highly accurate tool for predicting advanced hepatic fibrosis in WD patients.
• This practical tool aids in early identification and risk stratification.
• Early intervention can be facilitated through timely risk prediction in WD patients.