For patients with Wilson disease (WD) who have liver failure and encephalopathy, liver transplantation (LT) is a life-saving alternative. Patients who do not have encephalopathy can avoid LT and be effectively treated with chelation treatment. Where LT is necessary, it is critical to forecasting the probability of fatal effects. For a study, researchers sought to conduct a prospective critical analysis of the validity of the WD Index using a cohort of WD patients handled at the hospital. The WD Index and other clinical data were examined from 2005 to 2018, which were collected prospectively as part of clinical care.
About 52 children with WD (29 males) with a median age upon diagnosis of 11.69 (range 3.92–17.26) years were evaluated during a 13-year period. According to the PALF criteria, 17 were diagnosed as part of the family screening, 17 had abnormal liver enzymes, and 18 had acute hepatic decompensation (AHD). Patients in the pre-symptomatic group had an abnormal liver enzyme and a WD Index of 11, and none of them required LT. The WD Index is still an excellent predictor of LT in WD patients with AHD, with a sensitivity of 80%, a specificity of 100%, a positive predictive value of 100%, and a negative predictive value of 80%, respectively. Patients with an index of 8–10 needed LT for a median of 58 days (IQR 48–135). LT was required for patients with WD with AHD and an index of 11. Children with a WD Index of 8 to 10 within the first two months of admission need to be closely monitored since LT may be required.
Reference:journals.lww.com/jpgn/Abstract/2022/04000/Re_evaluation_of_King_Wilson_Index_in_Children.16.aspx
