ABSTRACT
Background and objective Wilson's disease (WD) is a rare autosomal recessive disease caused by mutations in the ATP7B gene, leading to impairment in copper excretion and subsequent accumulation primarily in the liver and brain. There is scarce data in the literature on the outcomes and cost burden of WD. In light of this, we aimed to assess outcomes, mortality rates, and costs associated with WD patients and their management in the United States (US). Methods We conducted a retrospective cohort study based on data in the National Inpatient Sample (NIS) database from 2007 to 2017. A total of 17,713 patients with a diagnosis of WD were identified using the International Classification of Diseases, Ninth or Tenth Revision (ICD-9/10) codes. Bivariate analyses were performed using t-tests for continuous variables and Pearson's chi-square tests for categorical variables, where two-sided p-values <0.05 were considered statistically significant. Results The majority of the 17,713 identified patients were female. The mean age of the WD cohort was 49 years. WD patients had a higher prevalence of Kayser-Fleischer rings, neuropsychiatric symptoms, and liver-related complications including acute hepatitis, liver failure, portal hypertension, and cirrhosis. Peptic ulcer disease, connective tissue disease, and hemolytic anemia were significantly more common in the WD cohort. Compared to the non-WD cohort, the WD cohort had a significantly higher mortality rate, longer length of stay (LOS), and increased hospitalization costs (p<0.0001). A higher proportion of patients who had undergone orthotopic liver transplantation (OLTx) were in the 18-34 and 35-44-year-old subgroups. On the contrary, the highest proportion of patients with WD who had not undergone OLTx were in the 55-89-year-old subgroup. WD patients who had undergone OLTx had a lower degree of comorbidities, decreased mortality rate, and shorter LOS (all p<0.0001) compared to WD patients who had not undergone OLTx. Conclusion Based on our findings, patients with WD had a higher LOS, mean hospitalization costs, and mortality rate compared to the non-WD cohort. Mortality rate and LOS were significantly lower in WD patients who had undergone OLTx.
ABSTRACT
Hepatocellular carcinoma (HCC) is a highly morbid disease both in the United States and worldwide. Chronic liver inflammation puts people at risk of developing HCC. As chronic liver disease prevalence increases in the United States there can be an expected rise in HCC. Spontaneous regression of HCC is a rare phenomenon but can provide much needed information on how to better understand disease characteristics and progression. The two proposed theories that may explain spontaneous regression are tumor hypoxia and immunologic reaction. In these cases, we describe 3 patients with heavy disease burden at presentation who showed spontaneous regression of cancer. The patient's characteristics correlate most with systemic immunologic reaction resulting in spontaneous regression. Unfortunately, all of these patients had disease recurrence shortly after regression. By studying patient data in cases of spontaneous regression, we can gain a better understanding of disease progression and which exogenous or endogenous factors determine HCC mortality. With this knowledge we hope to better characterize how spontaneous regression occurs, and how we can use this information to help in developing treatment options in the future.
