Acute respiratory failure due to pulmonary exacerbation in children with cystic fibrosis admitted in a pediatric intensive care unit: outcomes and factors associated with mortality

IF 4.7 2区医学 Q1 RESPIRATORY SYSTEM

Respiratory Research Pub Date : 2024-04-29 DOI:10.1186/s12931-024-02778-2

David Drummond, Charlotte Roy, Matthieu Cornet, Julie Bucher, Véronique Boussaud, Françoise Le Pimpec-Barthes, Margaux Pontailler, Olivier Raisky, Vanessa Lopez, Claudio Barbanti, Romain Guillemain, Sylvain Renolleau, Marion Grimaud, Mehdi Oualha, Laure de Saint Blanquat, Isabelle Sermet-Gaudelus

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Abstract

Children with advanced pulmonary disease due to cystic fibrosis (CF) are at risk of acute respiratory failure due to pulmonary exacerbations leading to their admission to pediatric intensive care units (PICU). The objectives of this study were to determine short and medium-term outcomes of children with CF admitted to PICU for acute respiratory failure due to pulmonary exacerbation and to identify prognosis factors. This retrospective monocentric study included patients less than 18 years old admitted to the PICU of a French university hospital between 2000 and 2020. Cox proportional hazard regression methods were used to determine prognosis factors of mortality or lung transplant. Prior to PICU admission, the 29 patients included (median age 13.5 years) had a severe lung disease (median Forced Expiratory Volume in 1 s percentage predicted at 29%). Mortality rates were respectively 17%, 31%, 34%, 41% at discharge and at 3, 12 and 36 months post-discharge. Survival rates free of lung transplant were 34%, 32%, 24% and 17% respectively. Risk factors associated with mortality or lung transplant using the univariate analysis were female sex and higher pCO2 and chloride levels at PICU admission, and following pre admission characteristics: home respiratory and nutritional support, registration on lung transplant list and Stenotrophomonas Maltophilia bronchial colonization. Children with CF admitted to PICU for acute respiratory failure secondary to pulmonary exacerbations are at high risk of death, both in the short and medium terms. Lung transplant is their main chance of survival and should be considered early.

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儿科重症监护室收治的囊性纤维化患儿因肺部恶化导致的急性呼吸衰竭：结果及与死亡率相关的因素

因囊性纤维化（CF）而患有晚期肺部疾病的儿童有可能因肺部恶化导致急性呼吸衰竭而入住儿科重症监护病房（PICU）。本研究的目的是确定因肺部恶化导致急性呼吸衰竭而入住 PICU 的 CF 患儿的短期和中期预后，并确定预后因素。这项回顾性单中心研究纳入了2000年至2020年期间入住法国一所大学医院PICU的18岁以下患者。研究采用 Cox 比例危险回归法来确定死亡或肺移植的预后因素。29 名患者（中位年龄为 13.5 岁）在入住 PICU 之前患有严重的肺部疾病（中位 1 秒内用力呼气容积预测值为 29%）。出院时、出院后 3 个月、12 个月和 36 个月的死亡率分别为 17%、31%、34% 和 41%。无肺移植的存活率分别为34%、32%、24%和17%。通过单变量分析，与死亡率或肺移植相关的风险因素包括：女性、入院时较高的pCO2和氯化物水平，以及入院前的以下特征：家庭呼吸和营养支持、肺移植名单登记和支气管嗜麦芽血单胞菌定植。因肺部疾病加重导致急性呼吸衰竭而入住 PICU 的 CF 患儿，无论在短期还是中期，死亡风险都很高。肺移植是他们生存的主要机会，应及早考虑。

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来源期刊

Respiratory Research 医学-呼吸系统

自引率

1.70%

发文量

314

期刊介绍： Respiratory Research publishes high-quality clinical and basic research, review and commentary articles on all aspects of respiratory medicine and related diseases. As the leading fully open access journal in the field, Respiratory Research provides an essential resource for pulmonologists, allergists, immunologists and other physicians, researchers, healthcare workers and medical students with worldwide dissemination of articles resulting in high visibility and generating international discussion. Topics of specific interest include asthma, chronic obstructive pulmonary disease, cystic fibrosis, genetics, infectious diseases, interstitial lung diseases, lung development, lung tumors, occupational and environmental factors, pulmonary circulation, pulmonary pharmacology and therapeutics, respiratory immunology, respiratory physiology, and sleep-related respiratory problems.