Evaluation of the Lung Capillary Blood Volume in Children With Sickle Cell Disease
试验速览
- 阶段
- 3 期
- 状态
- 已完成
- 入组人数
- 120
- 试验地点
- 1
- 主要终点
- Study of lung capillary blood volume and alveolar membrane diffusing capacity using NO-CO method
研究概览
简要总结
Sickle cell disease (SCD) is the most common inherited disease of the world affecting African and Caribbean populations. SCD is caused by the homozygous inheritance of the gene for sickle hemoglobin (HbS). Most patients with SCD develop abnormal pulmonary function characterized by airway obstruction, restrictive lung disease, abnormal diffusing capacity, hypoxemia and pulmonary hypertension In healthy subjects, lung capillary blood volume (Qc) and membrane diffusing capacity (Dm) can be accurately measured by the nitric oxide-carbon monoxide (NO-CO) method. We propose to study, for the first time, lung capillary blood volume and alveolar membrane diffusing capacity, using the NO-CO method, in children with SCD aged of at least 6 years Early determination of lung function and pulmonary circulation in children with SCD is very important, not only for the understanding of physiopathologic mechanisms of the disease but also for a better therapeutic management of these children.
详细描述
We propose to study, for the first time, lung capillary blood volume and alveolar membrane diffusing capacity, using the NO-CO method, in children with SCD aged of at least 6 years. We will compare lung function and measurement of Qc and Dm in 2 groups of 120 subjects, one group of SCD children, and the other of normal children matched on age and ethnic origin. Measurement of lung capillary blood will be measured twice, to assess short term reproducibility. The measurement will be done in sitting position and lying down for one part of subjects, and at rest and during a moderate rectangular exercise for the other part of subjects. These different tests are designed to assess the physiological adaptation of pulmonary circulation in these two populations of children. Combined with complete lung function measurements, echocardiographic assessment of pulmonary hemodynamics, and measurement of exhaled nitric oxide, these evaluations will lead to a better understanding of pathophysiology of lung injury in SCD. The study will be completes at Robert Debré Hospital, in close collaboration with Sickle Cell Disease Center and Physiology Department. Children will be included after informed consent signed, as legally prescribed.
研究设计
- 研究类型
- Interventional
- 分配方式
- Non Randomized
- 干预模型
- Parallel
- 主要目的
- Diagnostic
- 盲法
- None
入排标准
- 年龄范围
- 6 Years 至 18 Years（Child, Adult）
- 性别
- All
- 接受健康志愿者
- 是
入选标准
- •Children between 6 and 18 years
- •Sickle cell disease( SS,SC, SBETA O, SDpunjab) and control without sickle cell disease
- •Social insurance
- •Signed informed consent
排除标准
- •Respiratory disease other tha asthma
- •Cardiac disease
- •Encephalopathy
- •G6PD deficiency
- •Consent not signed
研究组 & 干预措施
1:Children with sickle cell disease
NO-CO inhalation and expiration:
Children with sickle cell disease
干预措施: NO-CO inhalation and expiration (Other)
2: Healthy volunteers
NO-CO inhalation and expiration:
Healthy volunteers
干预措施: NO-CO inhalation and expiration (Other)
结局指标
主要结局
Study of lung capillary blood volume and alveolar membrane diffusing capacity using NO-CO method
时间窗: The day of the measure
次要结局
- Respiratory physiopathology's study in sickle cell disease(At the induction of the study)
- Valid alveolar membrane diffusing capacity using NO-CO in children with or without sickle cell disease(At the induction of the study)
- Purpose respiratory function follow up in sickle cell disease child(At the induction of the study)
- Find relationship between these vascular abnormalities and NO metabolism(At the induction of the study)