Effect of splenectomy based on inverse probability weighting of the propensity score on Wilson's disease with hypersplenism: A retrospective cohort study

Abstract

Hypersplenism is a long-term complication of Wilson's disease (WD). Patients often have to stop copper excretion treatment due to the decrease in blood cell count aggravation of abnormal liver function, anaemia, bleeding caused by coagulation dysfunction. The present study aimed to explore the effect of splenectomy on serum, biochemical indicators and neurological function in patients with hypersplenism of WD to evaluate the impact of splenectomy on their survival and prognosis. Due to the non-randomness of splenectomy in patients with hypersplenism in WD in the present study, the propensity scoring model and inverse probability treatment weighting were used to evaluate the age, sex, duration of the disease. A total of 86 patients (40 with and 46 without splenectomy) were included in the present study. The baseline and preoperative data were adjusted by the inverse probability weighting method using the propensity score model. There was no significant difference in distribution of propensity scores between the two groups (P>0.05). There were significant differences in time-weighted PLT levels in patients with hypersplenism of WD [after adjustment, odd ratio (OR)=0.010; 95% CI, 0.0013-0.047; P<0.001]. The time-weighted Child-Pugh scores after adjustment also suggested a significant difference (OR=0.0684; 95% CI, 0.018-0.207; P<0.001). The time-weighted modified Young scale scores demonstrated no statistical significance (after adjustment, OR=0.294; 95% CI, 0.074-1.001; P>0.05). Survival data showed a mean survival time of 11.2±3.15 years with a 10-year survival rate of 64.97% for patients with nonsplenectomy and 12.9±2.62 years with a 10-year survival rate of 92.11% for patients with splenectomy, which was statistically significant (P<0.05). Due to crossover of survival curves at a later stage, the data were analysed using landmark analysis. The results suggested that splenectomy decreased death rate within 10 years by 84% compared with the non-splenectomy group (HR=0.158; 95% Cl, 0.0198-1.2545; P<0.05), but the survival rate of the two groups was not statistically significant after 10 years. (HR=0.445; 95% Cl, 0.2463-0.8022; P>0.05). In conclusion, splenectomy significantly improved levels of PLT and liver function in patients with hypersplenism of WD, neurological function did not deteriorate and survival rate was improved.

The etiology and differential diagnosis of “autoimmune hepatitis-like liver disease” in children: a single-center retrospective study

“…(3) Infectious liver disease ( 11 ): existing etiological evidence of hepatitis virus infection or other viral active infection, or existing clinical and etiological evidence of systemic infection. (4) Gene deficiency diseases including either hepatolenticular degeneration [according to the Leipzig Diagnostic Criteria ( 12 ) and in concordance with the American Association for the Study of Liver Diseases (AASLD) guidelines ( 13 )]. or other genetic liver diseases which can only be diagnosed by genetic testing, including progressive familial intrahepatic cholestasis (PFIC).…”