Characteristics and outcomes of hospitalized patients with Wilson's disease in the United States: A national survey.

INTRODUCTION AND

OBJECTIVES

Wilson's disease (WD) is a rare genetic disorder characterized by excessive copper disposition predominantly in the liver and brain. Hospitalization data on patients with WD are scarce. Hence, we sought to examine the inpatient characteristics and outcomes of patients with WD.

PATIENTS AND

METHODS

We utilized the National Inpatient Database (2006-2011) and analyzed all adult patients with a diagnosis of WD.

RESULTS

There were 9046 hospitalizations during the study period. The leading etiologies for admissions were chronic liver disease (8.58%), WD (6.49%) and infections (septicemia 3.10% and pneumonia 2.50%). The overall inpatient mortality rate for WD patients was 2.58%. Independent predictors of mortality in WD patients were acute respiratory failure (OR: 4.53; 95% CI: 2.44-8.42), acute renal failure (OR: 4.09; 95% CI: 2.19-7.65), decompensated liver disease or liver failure (OR: 3.37; 95% CI: 1.72-6.59), and advanced age (every 10 year increase, OR: 1.48; 95% CI: 1.25-1.75). Propensity-score matched analysis revealed better inpatient survival in WD patients compared to matched non-WD patients (2.84% vs. 4.67%, p = 0.01).

CONCLUSIONS

Our study demonstrated the clinical characteristics and outcomes of hospitalized patients with WD. These findings add important knowledge to our understanding of the healthcare utilization and outcomes of this rare disease in the United States.