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 wi...

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Autores principales:	Na Li, Somashekar G. Krishna, Alice Hinton, Darwin L. Conwell, Khalid Mumtaz
Formato:	article
Lenguaje:	EN
Publicado:	Elsevier 2021
Materias:	National inpatient sample Wilson disease Hospitalization Mortality Specialties of internal medicine RC581-951
Acceso en línea:	https://doaj.org/article/bb657af59b5643238f52ea244a0ff708
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spelling	oai:doaj.org-article:bb657af59b5643238f52ea244a0ff7082021-11-18T04:46:07ZCharacteristics and outcomes of hospitalized patients with Wilson’s disease in the United States: A national survey1665-268110.1016/j.aohep.2021.100362https://doaj.org/article/bb657af59b5643238f52ea244a0ff7082021-11-01T00:00:00Zhttp://www.sciencedirect.com/science/article/pii/S1665268121000612https://doaj.org/toc/1665-2681Introduction 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.Na LiSomashekar G. KrishnaAlice HintonDarwin L. ConwellKhalid MumtazElsevierarticleNational inpatient sampleWilson diseaseHospitalizationMortalitySpecialties of internal medicineRC581-951ENAnnals of Hepatology, Vol 25, Iss , Pp 100362- (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	National inpatient sample Wilson disease Hospitalization Mortality Specialties of internal medicine RC581-951
spellingShingle	National inpatient sample Wilson disease Hospitalization Mortality Specialties of internal medicine RC581-951 Na Li Somashekar G. Krishna Alice Hinton Darwin L. Conwell Khalid Mumtaz Characteristics and outcomes of hospitalized patients with Wilson’s disease in the United States: A national survey
description	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.
format	article
author	Na Li Somashekar G. Krishna Alice Hinton Darwin L. Conwell Khalid Mumtaz
author_facet	Na Li Somashekar G. Krishna Alice Hinton Darwin L. Conwell Khalid Mumtaz
author_sort	Na Li
title	Characteristics and outcomes of hospitalized patients with Wilson’s disease in the United States: A national survey
title_short	Characteristics and outcomes of hospitalized patients with Wilson’s disease in the United States: A national survey
title_full	Characteristics and outcomes of hospitalized patients with Wilson’s disease in the United States: A national survey
title_fullStr	Characteristics and outcomes of hospitalized patients with Wilson’s disease in the United States: A national survey
title_full_unstemmed	Characteristics and outcomes of hospitalized patients with Wilson’s disease in the United States: A national survey
title_sort	characteristics and outcomes of hospitalized patients with wilson’s disease in the united states: a national survey
publisher	Elsevier
publishDate	2021
url	https://doaj.org/article/bb657af59b5643238f52ea244a0ff708
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Characteristics and outcomes of hospitalized patients with Wilson’s disease in the United States: A national survey

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