Division of Occupational and Environmental Medicine, Epidemiology, Biostatistics and Prevention Institute, University of Zurich and University Hospital Zurich, Hirschengraben 84, 8001, Zurich, Switzerland.
Department of Internal Medicine, University Hospital Zurich, Zurich, Switzerland.
Orphanet J Rare Dis. 2021 Feb 27;16(1):105. doi: 10.1186/s13023-021-01737-0.
Little is known about the impact of rare diseases on inpatient outcomes.
To 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.
Retrospective, cross-sectional study.
165,908 inpatients, Swiss teaching hospital.
Primary outcome: in-hospital mortality.
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.
Patients 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]).
Rare 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.
关于罕见病对住院患者结局的影响知之甚少。
比较 0 种、1 种或>1 种罕见病患者的住院结局。应用了 628 种 ICD-10 编码的罕见病目录来计算罕见病。
回顾性、横断面研究。
165908 名住院患者，瑞士教学医院。
主要结局：院内死亡率。
住院时间（LOS）、重症监护病房（ICU）入院、ICU LOS 和 30 天再入院。通过多变量回归分析单一和联合罕见病与结局的相关性。
患有 1 种罕见病的患者院内死亡风险增加（优势比[OR]：1.80；95%置信区间[CI]：1.67，1.95），联合罕见病的相关性更强（OR 2.78；95% CI 2.39，3.23）。患有 1 种罕见病的女性院内死亡的 OR 为 1.69（95% CI 1.50，1.91），患有联合罕见病的女性 OR 为 2.99（95% CI 2.36，3.79）。男性分别为 1.85（95% CI 1.68，2.04）和 2.61（95% CI 2.15，3.16）。罕见病与 LOS 延长（1 种和>1 种罕见病分别增加 28%和 49%）、ICU 入院（1 种和>1 种：OR 1.64[95% CI 1.57，1.71]和 2.23[95% CI 2.01，2.48]）、ICU LOS 延长（1 种和>1 种罕见病分别增加 14%和 40%）和 30 天再入院（1 种和>1 种：OR 1.57[95% CI 1.47，1.68]和 1.64[95% CI 1.37，1.96]）相关。
罕见病与住院患者结局不良独立相关。这可能是第一项表明联合罕见病与院内死亡、LOS 延长、ICU 入院、ICU LOS 延长和 30 天再入院的相关性更强的研究。
