Abstract
BackgroundLittle is known about the impact of rare diseases on inpatient outcomes.ObjectiveTo compare outcomes of inpatients with 0, 1, or > 1 rare disease. A catalogue of 628 ICD-10 coded rare diseases was applied to count rare diseases.DesignRetrospective, cross-sectional study.Subjects165,908 inpatients, Swiss teaching hospital.Main measuresPrimary outcome: in-hospital mortality. Secondary outcomes: length of stay (LOS), intensive care unit (ICU) admissions, ICU LOS, and 30-day readmissions. Associations with single and combined rare diseases were analyzed by multivariable regression.Key resultsPatients with 1 rare disease were at increased risk of in-hospital death (odds ratio [OR]: 1.80; 95% confidence interval [CI]: 1.67, 1.95), combinations of rare diseases showed stronger associations (OR 2.78; 95% CI 2.39, 3.23). Females with 1 rare disease had an OR of 1.69 (95% CI 1.50, 1.91) for in-hospital death, an OR of 2.99 (95% CI 2.36, 3.79) if they had a combination of rare diseases. Males had an OR of 1.85 (95% CI 1.68, 2.04) and 2.61 (95% CI 2.15, 3.16), respectively. Rare diseases were associated with longer LOS (for 1 and > 1 rare diseases: increase by 28 and 49%), ICU admissions (for 1 and > 1: OR 1.64 [95% CI 1.57, 1.71] and 2.23 [95% CI 2.01, 2.48]), longer ICU LOS (for 1 and > 1 rare diseases: increase by 14 and 40%), and 30-day readmissions (for 1 and > 1: OR 1.57 [95% CI 1.47, 1.68] and 1.64 [95% CI 1.37, 1.96]).ConclusionsRare diseases are independently associated with worse inpatient outcomes. This might be the first study suggesting even stronger associations of combined rare diseases with in-hospital deaths, increased LOS, ICU admissions, increased ICU LOS, and 30-day readmissions.
Highlights
Rare diseases are a diverse group of diseases with a low prevalence
We studied associations of rare diseases with (i) in-hospital mortality, (ii) increased length of stay (LOS), (iii) intensive care unit (ICU) admissions, (iv) increased ICU LOS, and (v) 30-day readmissions
Primary end point The unadjusted logistic regression model indicated an increased in-hospital mortality associated with the presence of a single rare disease, and with the presence of combined rare diseases
Summary
Rare diseases are a diverse group of diseases with a low prevalence. The defined prevalence thresholds of rare diseases vary across references from 5 to 76 cases/100,000 people [1], that is, rare diseases affect a Blazsik et al Orphanet J Rare Dis (2021) 16:105 small fraction of the population. The fraction of studies into general health indicators and clinical outcomes (i.e., mortality or health-care utilization) of rare diseases in comparison with more frequent conditions is rather small [9]. Little is known about the clinical impact of rare diseases among inpatients. No study investigated the effect of combinations of rare diseases on clinical outcomes in the inpatient setting. Little is known about the impact of rare diseases on inpatient outcomes
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