Clinical trial NCT01565954: Sleep Respiratory Disorders Evaluation in Sickle Cell Disease Children
Official title: Systematic Evaluation of Sleep Respiratory Disorders During Sleep in Children With Sickle Cell Disease
Study type: OBSERVATIONAL
Overall status: COMPLETED
Conditions: Sickle Cell Disease
Respiration Disorders
Keywords: Sickle Cell Disease
Respiration Disorders
Polysomnography
Lead sponsor: Assistance Publique - Hôpitaux de Paris
Sponsor class: OTHER
First posted: 2012-03-29
Enrollment: 62
Sex: ALL
Minimum age (years): 2
Maximum age (years): 6
Standard ages: CHILD
Accepts healthy volunteers: no
Observational model: COHORT
Time perspective: PROSPECTIVE
Sampling method: PROBABILITY_SAMPLE
Study population: The objective of this study is to provide an early curative treatment for homozygous sickle cell children in age group of 2 to 6 years based on the anomalies identified in polysomnography. The choice of the age of 2 to 6 is linked to the peak risk of onset of cerebrovascular occlusive complications in this age

Brief summary: In Sickle cell disease children, sleep respiratory abnormalities are risk factors for vaso-occlusive complications, as well as cerebral vasculopathy.

A 18 months follow-up children with sickle cell disease evaluating sleep respiratory problems frequency and etiology, as well as their influence on sickle cell disease complications.

Detailed description: * Inclusion visit with physical examination. A 2.9 ml blood sample will be necessary, if not done within the framework of care within 6 months for: CBC (Cell Blood Count), reticulocytes counts (1.2 ml of blood), liver enzymes, electrolytes, urea, creatininemia (1,2 ml of blood) and fetal hemoglobin (0.5 ml of blood). - A complete standardized examination including nasopharynx endoscopy, if not yet done in usual care, to identified upper airway obstruction
* A standardized pneumology evaluation to identified obstructive lung symptomatology
* An ambulatory polysomnography will be performed within days following inclusion, during sleep and will allow electroencephalogram recording, oculomotricity, muscles (mentalis muscles and tibialis anterior) movements, electrocardiogram and respiratory activity recording.

The steering committee will classify the children in three populations based on their exam results:

* Group 1: no abnormality in polysomnography
* Group 2: obstructive sleep-related disorder
* Group 3: isolated sleep hypoxemia for group3, patients will have a new ambulatory polysomnography.

Eligibility criteria: Inclusion Criteria:

* Sickle cell disease children with either sickle cell anemia, compound heterozygozity SB0-thalassemia, or SDPunjab,
* no prior transfusion program within 3 months,
* no prior treatment with hydroxycarbamide within 3 months,
* Aged 2 to 6 years,
* Parents or tutors signed informed consent,
* Prior physical examination,
* Social security insurance.

Exclusion criteria:

* Encephalopathy
* Other disease that could interfere with protocol exams realization
* Nitrous oxide analgesia contre-indication
* Conventional hospitalization in the past 3 weeks

Primary outcomes:
measure: Vaso-occlusive and cerebral complications frequency
timeFrame: 18 months
Secondary outcomes:
measure: Respiratory sleep abnormalities frequency
timeFrame: 18 months
measure: Relationship between sleep abnormalities and transcranial doppler abnormalities
timeFrame: 2 months
measure: Polysomnography relevance compared to sleep ventilatory polygraphy
timeFrame: 2 months
Other outcomes:
Locations:
city: Paris
country: France
facility: Assistance Publique - Hôpitaux de Paris
Study officials:
affiliation: Assistance Publique - Hôpitaux de Paris
name: Malika BENKERROU, MD, PhD
role: PRINCIPAL_INVESTIGATOR
References: