Respiration Disorders, Sickle Cell Disease
Conditions
Keywords
Sickle Cell Disease, Respiration Disorders, Polysomnography
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.
Interventions
None listed
Sponsors
Assistance Publique - Hôpitaux de Paris
Study design
Observational model
COHORT
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
2 Years to 6 Years
Healthy volunteers
No
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
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Vaso-occlusive and cerebral complications frequency | 18 months | Vaso-occlusive complications frequency and abnormal transcranial doppler (TCD) during the 18 months of follow up (cohort study) 3 subpopulations will be identified upon polysomnographic results: * Group 1: normal polysomnography * Group 2: sleep abnormalities related to obstructive upper airway disease or bronchial disease * Group 3: sleep abnormalities associated with non-obstructive pattern and isolated hypoxemia identified. The therapeutic trial will be done on this group. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Respiratory sleep abnormalities frequency | 18 months | Respiratory sleep abnormalities frequency in a population of young sickle cell disease children. |
| Relationship between sleep abnormalities and transcranial doppler abnormalities | 2 months | Analyze the relationship between sleep respiratory and non-respiratory abnormalities and transcranial doppler abnormalities |
| Polysomnography relevance compared to sleep ventilatory polygraphy | 2 months | Polysomnography relevance compared to sleep ventilatory polygraphy in diagnosis of sleep abnormalities |
Countries
France
Outcome results
None listed