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

Na Li, Somashekar G. Krishna, Alice Hinton, Darwin L. Conwell, Khalid Mumtaz

Research output: Contribution to journalArticlepeer-review

Abstract

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.

Original languageEnglish
Article number100362
JournalAnnals of Hepatology
Volume25
DOIs
StatePublished - Nov 1 2021

Bibliographical note

Publisher Copyright:
© 2021 Fundación Clínica Médica Sur, A.C.

Keywords

  • Hospitalization
  • Mortality
  • National inpatient sample
  • Wilson disease

ASJC Scopus subject areas

  • Hepatology

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