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Wilson disease‐related cirrhosis is associated with increased odds of hemolytic anemia

Wilson disease‐related cirrhosis is associated with increased odds of hemolytic anemia Wilson disease (WD) is an autosomal recessive condition caused by homozygous or compound heterozygous mutations in the ATP7B gene, which encodes a copper‐transporting P‐type ATPase that leads to impaired copper metabolism. As a result, copper accumulates in the liver, brain, cornea, and other organs. The prevalence of WD is reported to be between 2.9 and 3.3 per 100,000 based on epidemiologic studies.1The diagnosis WD is often challenging given the clinical presentation is variable, ranging from biochemical abnormalities without associated symptoms to acute liver failure, and requires a combination of clinical, biochemical, and genetic tests given the lack of any single sensitive and specific diagnostic test.2Several studies have reported the clinical course, diagnostic findings, and outcomes in patients with WD; however, there limited data are available about the disease burden given the rarity of the disease. The primary aim of this study was to evaluate the association of cirrhosis and WD with inpatient mortality. The secondary aims of this study were to examine common reasons for admission, complications of disease, and resource utilization in patients with cirrhosis associated with WD in the United States.METHODSA retrospective, observational study using the National Inpatient Sample (NIS), the largest publicly available inpatient database in http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Journal of Evidence Based Medicine Wiley

Wilson disease‐related cirrhosis is associated with increased odds of hemolytic anemia

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References (11)

Publisher
Wiley
Copyright
© 2022 Chinese Cochrane Center, West China Hospital of Sichuan University and John Wiley & Sons Australia, Ltd
ISSN
1756-5383
eISSN
1756-5391
DOI
10.1111/jebm.12463
Publisher site
See Article on Publisher Site

Abstract

Wilson disease (WD) is an autosomal recessive condition caused by homozygous or compound heterozygous mutations in the ATP7B gene, which encodes a copper‐transporting P‐type ATPase that leads to impaired copper metabolism. As a result, copper accumulates in the liver, brain, cornea, and other organs. The prevalence of WD is reported to be between 2.9 and 3.3 per 100,000 based on epidemiologic studies.1The diagnosis WD is often challenging given the clinical presentation is variable, ranging from biochemical abnormalities without associated symptoms to acute liver failure, and requires a combination of clinical, biochemical, and genetic tests given the lack of any single sensitive and specific diagnostic test.2Several studies have reported the clinical course, diagnostic findings, and outcomes in patients with WD; however, there limited data are available about the disease burden given the rarity of the disease. The primary aim of this study was to evaluate the association of cirrhosis and WD with inpatient mortality. The secondary aims of this study were to examine common reasons for admission, complications of disease, and resource utilization in patients with cirrhosis associated with WD in the United States.METHODSA retrospective, observational study using the National Inpatient Sample (NIS), the largest publicly available inpatient database in

Journal

Journal of Evidence Based Medicine – Wiley

Published: Mar 1, 2022

Keywords: cirrhosis; ESLD; NIS; Wilson disease

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