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

NCT ID: NCT00560261

Last Updated: 2023-02-03

Basic Information

• Recruitment Status: COMPLETED
• Clinical Phase: PHASE3
• Total Enrollment: 120 participants
• Study Classification: INTERVENTIONAL
• Study Start Date: 2008-02-29
• Study Completion Date: 2012-02-29

Brief 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.

Detailed 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.

Conditions

Sickle Cell Disease

Study Design

• Allocation Method: NON_RANDOMIZED
• Intervention Model: PARALLEL
• Primary Study Purpose: DIAGNOSTIC
• Blinding Strategy: NONE

Study Groups

1:Children with sickle cell disease

NO-CO inhalation and expiration:

Children with sickle cell disease

Group Type: EXPERIMENTAL

NO-CO inhalation and expiration

Intervention Type: OTHER

NO-CO inhalation and expiration

2: Healthy volunteers

NO-CO inhalation and expiration:

Healthy volunteers

Group Type: ACTIVE_COMPARATOR

NO-CO inhalation and expiration

Intervention Type: OTHER

NO-CO inhalation and expiration

Interventions

NO-CO inhalation and expiration

NO-CO inhalation and expiration

Intervention Type: OTHER

NO-CO inhalation and expiration

NO-CO inhalation and expiration

Intervention Type: OTHER

Eligibility Criteria

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 Criteria

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

• Minimum Eligible Age: 6 Years
• Maximum Eligible Age: 18 Years
• Eligible Sex: ALL
• Accepts Healthy Volunteers: Yes

Sponsors

Assistance Publique - Hôpitaux de Paris

OTHER

Sponsor Role: lead

Responsible Party

Responsibility Role: SPONSOR

Principal Investigators

Florence MISSUD, Md

Role: PRINCIPAL_INVESTIGATOR

Assistance Publique - Hôpitaux de Paris

Locations

Hopital Robert DEBRE

Paris, , France

Countries

France

References

Bokov P, Boizeau P, Pautrat J, Missud F, Ba A, Haouari Z, Denjean A, Delclaux C, Benkerrou M. Altered pulmonary capillary blood volume in childhood sickle cell disease. Eur Respir J. 2020 Dec 10;56(6):2000379. doi: 10.1183/13993003.00379-2020. Print 2020 Dec. No abstract available.

Reference Type: RESULT

PMID: 32616593 (View on PubMed)

Other Identifiers

2007-A00913-50

Identifier Type: OTHER

Identifier Source: secondary_id

P061013

Identifier Type: -

Identifier Source: org_study_id