Wilson’s disease admission increased, mortality rate unchanged

CHICAGO — While the number of admissions for Wilson’s disease has increased, the mortality rate has not changed, which may relate to improved access to health care and disease recognition, according to a study presented at Digestive Disease Week.

“Our primary outcome was in-hospital mortality trend in the past decade, while the secondary outcomes were morbidity, as measured by the presence of acute liver failure, psychiatric disease, hemolytic anemia, acute kidney injury, ICU stay, and liver transplant; hospital length of stay; and hospital cost of stay,” Paul Thomas Kröner, MD, from Mount Sinai St. Luke’s, New York, said in a presentation.

The researchers sought to examine the epidemiology, clinical presentation, mortality and trends in resource use in patients with Wilson’s disease in response to the limited data of disease burden. The retrospective study comprised 15,908 admissions for Wilson’s disease between 2004 and 2013. The information was retrieved from the National Inpatient Sample. Mean patient age was 46.8 years, 55% were women, 71.1% were white and socioeconomic status was mostly similar among all patients.

Hospital admissions increased from 1.9 per 100,000 in 2004 to 5.6 per 100,000 in 2013, while inpatient mortality rate was 2.6% and did not significantly change over the past decade.

The most common manifesting features in the cohort included acute liver failure (17.3%), acute kidney injury (14.9%), neuropsychiatric symptoms in (7.2%), required ICU stay (5.2%) and hemolytic anemia (1.6%). Acute liver failure was the most common indication for the 3.1% of patients who underwent liver transplant. Neuropsychiatric symptoms especially prevalent in women (64%).

Complications that displayed significant association with mortality included acute liver failure (OR = 3.85; 95% CI, 2.01-7.38), acute liver injury (OR = 4.27; 95% CI, 2.62-6.94), hemolytic anemia (OR = 3.57; 95% CI, 1.01-12.96) and ICU stay (OR = 19.28; 95% CI, 11.01-33.77).

Length of additional hospital stay (OR = 2.41; 95% CI, 1.93-2.88) and total additional hospitalization charges (OR = $3,246; 95% CI, $1,116-$5,375) increased incrementally each year.

“In the past decade, Wilson’s disease has seen increasing total hospital charges. This is fairly constant in all other diseases that we see and we think that is due to changing insurance policies and models in the United States,” Kroner said. “Finally, hospital length of stay has increased significantly in the past decade of Wilson’s disease. – by Talitha Bennett

References:

Kröner PT, et al. Abstract 571. Presented at: Digestive Disease Week; May 6-9, 2017; Chicago.

Disclosure : Kröner reports no relevant financial disclosures.