Cardiopulmonary Fitness in Children With Cystic Fibrosis Compared to Healthy Children
试验速览
- 阶段
- 不适用
- 状态
- 已完成
- 入组人数
- 345
- 试验地点
- 1
- 主要终点
- Maximum oxygen volume by min (VO2 max) assessed by cardiopulmonary exercise test (CPET)
研究概览
简要总结
Cystic fibrosis is the most common severe genetic disease with autosomal recessive transmission in the Caucasian population.
Its prognosis has improved considerably since the creation of Cystic Fibrosis centers (CF centers) and the improvement of symptomatic management (nutrition, antibiotic therapy, transplantation, etc.). Thus, the median survival rate is now 46 years, whereas it was 5 years in 1963.
The current challenges for cystic fibrosis patients are therefore twofold:
to continue to improve their survival, and to improve their quality of life (QoL) to promote "healthy ageing" with this pathology that begins in childhood.
In 1980, the World Health Organization (WHO) stated that functional capacity explorations best reflected the impact of chronic disease on health-related quality of life. Impairment of physical activity is common to chronic diseases, as in cystic fibrosis, where respiratory impairment and denutrition have been shown to contribute to reduced exercise tolerance and increased dyspnoea.
Measurement of the maximum oxygen consumption (VO2max) by a cardiopulmonary exercise test (CPET) is regarded as the gold standard exercise test in the measurement of aerobic exercise capacity.
In 2005 Pianosi et al. found that for children with cystic fibrosis, the rate of decline of VO2max measured by CPET was predictive of poorer quality of life.
Continuing to study the determinants associated with impaired aerobic fitness in cystic fibrosis offers the hope of considering appropriate therapies to further improve the quality of life of these patients.
In recent years, the arrival and widespread use of CFTR protein modulators in children has been a real turning point and makes it possible to envisage a drastic change in the history of this disease and its prognosis in the long term.
Thus, in this study, the investigators aimed to assess the aerobic fitness, assessed by a CPET, of children with cystic fibrosis, and to compare the results with healthy controls. Secondly, investigators wanted to identify the predictive factors of VO2max in children with cystic fibrosis.
详细描述
This retrospective study was carried out from November 2010 to September 2015 for control children (already published PubMed Identifier (PMID): 29170358) : and July 2018 to December 2019 for cystic fibrosis children.
CPET were performed in the paediatric CPET laboratory of Montpellier University Hospital, France.
Children aged 7 to 17 years old were recruited. Two groups were identified: children with cystic fibrosis and healthy control children.
- The cystic fibrosis group consisted of children followed up every three months in the cystic fibrosis center of Montpellier University and referred to our paediatrician CPET laboratory in 2019 in the framework of an annual check-up. For the few patients who did not have a CPET in 2019, investigators included those who passed a CPET between July and December 2018. From patients' interviews, the absence of any physical activity, apart from physical education at school, was indicative of sedentary lifestyle.
Investigators also collected anthropometric data, radiological findings, CF-related markers of disease severity including cystic fibrosis transmembrane conductance regulator (CFTR) genotype, microbiological infection, pancreatic insufficiency, cystic fibrosis-related diabetes (CFRD), and treatment including modulator data. 2. As in our previous similar CPET controlled paediatric studies, the control group consisted of children referred for non-severe functional symptoms linked to exercise (murmur, palpitation or dyspnoea) or for medical sports certificate. These children were classified as controls after a completely normal check-up, including physical examination, ECG, echocardiography and spirometry. Children with any chronic disease, medical condition (cardiac, neurologic, respiratory, muscular or renal), or medical treatment, and those requiring any further specialised medical consultation were not eligible.
研究设计
- 研究类型
- Observational
- 观察模型
- Case Control
- 时间视角
- Retrospective
入排标准
- 年龄范围
- 7 Years 至 17 Years（Child）
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •Aged 7-17 years old
- •Referred to the paediatric cardiopulmonary exercise test (CPET) laboratory of Montpellier University Hospital, France
- •Have completed a CPET
- •For cystic fibrosis group:
- •To be followed up in the cystic fibrosis center of Montpellier University
- •To be referred to our pediatrician CPET laboratory in 2019, or between July and December 2018 if they have not passed a CPET in 201
- •For control group:
- •To be referred for non-severe functional symptoms linked to exercise (murmur, palpitation or dyspnea) or for medical sports certificate.
- •Having a completely normal check-up, including physical examination, electrocardiogram, echocardiography and spirometry
排除标准
- •Patients < 7 ou > 17 years old
- •Absolute contraindication for CPET : fever, uncontrolled asthma, respiratory failure, acute myocarditis or pericarditis, uncontrolled arrhythmias causing symptoms or hemodynamic compromise, uncontrolled heart failure, acute pulmonary embolus or pulmonary infarction, and children with mental impairment leading to inability to cooperate
- •Parents refuse the use of medical data
- •For control group : children with any chronic disease, medical condition (cardiac, neurologic, respiratory, muscular, or renal), medical treatment, requiring any further specialized medical consultation or with any finding at clinical and paraclinical examination (electrocardiogram, echocardiography)
结局指标
主要结局
Maximum oxygen volume by min (VO2 max) assessed by cardiopulmonary exercise test (CPET)
时间窗: up to 30 oct 2023
次要结局
- Determinants of maximum oxygen volume by min(up to 30 oct 2023)