Prognostic factors for outcomes in patients with cystic fibrosis who are observed at the Federal Clinical Center

Q3 Medicine

Voprosy Detskoi Dietologii Pub Date : 2023-01-01 DOI:10.20953/1727-5784-2023-2-5-12

O. Zonenko, P. Suter, P. Shumilov, S. Krasovsky

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Abstract

Objective. To identify the factors significantly affecting clinical outcomes in patients with cystic fibrosis who are observed at the Federal Clinical Center based on a multivariate analysis of the disease course. Patients and methods. A total of 657 medical records of patients with cystic fibrosis were examined. The survival rate was analyzed depending on the type of genetic mutation and sex of patients. The dynamic model for predicting 2-year survival outcomes based on 36 parameters from the National Registry was developed. Results. The multivariate Cox proportional hazard model applied to dynamic data by a landmarking method revealed a statistically significant association between mortality over a 2-year lifetime with the values of the following factors: FEV1 (relative risk (RR): 0.95; 95% confidence interval (CI): 0.94–0.97); cystic fibrosis-related diabetes (RR: 2.36; CI: 1.47–3.80); Burkholderia cepacia complex infection (RR: 3.22; CI: 2.12–4.91); need for oxygen therapy (RR: 1.9; CI: 1.15–3.12); previous pneumothorax (RR: 2.72; CI: 1.19-6.20); vitamin therapy (RR: 2.72; CI: 1.09–6.81); pancreatic enzyme intake (RR: 0.38; CI: 0.17–0.86). Conclusion. The awareness of prognostic survival factors will allow to optimize treatment and rehabilitation programs for patients with cystic fibrosis, which will lead to an increase in patients’ life expectancy. Key words: cystic fibrosis, children, survival rate

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在联邦临床中心观察到的囊性纤维化患者预后因素

目标。根据病程的多变量分析，确定在联邦临床中心观察到的囊性纤维化患者临床结果的显著影响因素。患者和方法。分析了657例囊性纤维化患者的病历。生存率根据基因突变类型和患者性别进行分析。基于来自国家登记的36个参数，开发了预测2年生存结果的动态模型。结果。采用标记法对动态数据进行多变量Cox比例风险模型分析，结果显示，2年生命期死亡率与以下因素之间存在统计学显著相关:FEV1(相对危险度RR): 0.95;95%置信区间(CI): 0.94-0.97);囊性纤维化相关性糖尿病(RR: 2.36;置信区间:1.47—-3.80);洋葱伯克霍尔德菌复合感染(RR: 3.22;置信区间:2.12—-4.91);需氧量(RR: 1.9;置信区间:1.15—-3.12);既往气胸(RR: 2.72;置信区间:1.19—-6.20);维生素治疗(RR: 2.72;置信区间:1.09—-6.81);胰酶摄入量(RR: 0.38);置信区间:0.17—-0.86)。结论。对预后生存因素的认识将有助于优化囊性纤维化患者的治疗和康复方案，这将导致患者预期寿命的增加。关键词:囊性纤维化，儿童，生存率

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来源期刊

Voprosy Detskoi Dietologii Medicine-Pediatrics, Perinatology and Child Health

CiteScore

1.20

自引率

0.00%

发文量

期刊介绍： The scientific journal Voprosy Detskoi Dietologii is included in the Scopus database. Publisher country is RU. The main subject areas of published articles are Food Science, Pediatrics, Perinatology, and Child Health, Nutrition and Dietetics, Клиническая медицина.