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

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.

Study Overview

• Status: Completed
• Conditions: Sickle Cell Disease
• Intervention / Treatment: Other: NO-CO inhalation and expiration

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.

• Study Type: Interventional
• Enrollment (Actual): 120
• Phase: Phase 3

Contacts and Locations

Study Locations

• France
  • Paris, France, 75019
    • Hopital Robert Debre

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 6 years to 18 years (Child, Adult)
• Accepts Healthy Volunteers: Yes
• Genders Eligible for Study: All

Description

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

Study Plan

How is the study designed?

Design Details

• Primary Purpose: Diagnostic
• Allocation: Non-Randomized
• Interventional Model: Parallel Assignment
• Masking: None (Open Label)

Number of Arms

2

Arms and Interventions

Participant Group / Arm	Intervention / Treatment
Experimental: 1:Children with sickle cell disease; NO-CO inhalation and expiration: Children with sickle cell disease	Other: NO-CO inhalation and expiration; NO-CO inhalation and expiration
Active Comparator: 2: Healthy volunteers; NO-CO inhalation and expiration: Healthy volunteers	Other: NO-CO inhalation and expiration; NO-CO inhalation and expiration

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Time Frame
Study of lung capillary blood volume and alveolar membrane diffusing capacity using NO-CO method	The day of the measure

Secondary Outcome Measures

Outcome Measure	Time Frame
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

Collaborators and Investigators

• Sponsor: Assistance Publique - Hôpitaux de Paris
• Investigators: Principal Investigator: Florence MISSUD, Md, Assistance Publique - Hôpitaux de Paris

Publications and helpful links

General Publications

• 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.

Study record dates

Study Major Dates

• Study Start: February 1, 2008
• Primary Completion (Actual): February 1, 2012
• Study Completion (Actual): February 1, 2012

Study Registration Dates

• First Submitted: November 16, 2007
• First Submitted That Met QC Criteria: November 16, 2007
• First Posted (Estimate): November 19, 2007

Study Record Updates

• Last Update Posted (Actual): February 3, 2023
• Last Update Submitted That Met QC Criteria: February 1, 2023
• Last Verified: July 1, 2012

More Information

Terms related to this study

• Keywords: Children; Sickle cell disease;; Lung capillary blood volume
• Additional Relevant MeSH Terms: Hematologic Diseases; Genetic Diseases, Inborn; Anemia; Anemia, Hemolytic, Congenital; Anemia, Hemolytic; Hemoglobinopathies; Anemia, Sickle Cell
• Other Study ID Numbers: P061013; 2007-A00913-50 (Other Identifier: ID-RCB)