Evaluation of the Lung Capillary Blood Volume in Children With Sickle Cell Disease

Last updated: February 1, 2023
Sponsor: Assistance Publique - Hôpitaux de Paris
Overall Status: Completed

Phase

3

Condition

Sickle Cell Disease

Red Blood Cell Disorders

Treatment

N/A

Clinical Study ID

NCT00560261
P061013
2007-A00913-50
  • Ages 6-18
  • All Genders
  • Accepts Healthy Volunteers

Study Summary

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.

Eligibility Criteria

Inclusion

Inclusion Criteria:

  • 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

Exclusion

Exclusion Criteria:

  • Respiratory disease other tha asthma
  • Cardiac disease
  • Encephalopathy
  • G6PD deficiency
  • Consent not signed

Study Design

Total Participants: 120
Study Start date:
February 01, 2008
Estimated Completion Date:
February 29, 2012

Study Description

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.

Connect with a study center

  • Hopital Robert DEBRE

    Paris, 75019
    France

    Site Not Available

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