Article

Osteogenesis imperfecta (OI) is the most common genetic skeletal
disorder. Extraskeletal findings are common but an association with
sleep-disordered breathing (SDB) has never been described. The aim of this study
was to investigate clinical features of children with OI and suspected SDB.
METHODS: A retrospective study of clinical records, signs of SDB and
polysomnographic recordings of children with OI was performed. We paid particular
attention to symptoms that could be associated with SDB in this population -
scoliosis, kyphosis, vertebral arthrodesis, chest wall deformities, basilar
impression, autonomy - as well as data already known to be associated with
obstructive sleep apnea such as body mass index and upper-airway impairment.
RESULTS: We reviewed the clinical charts of 188 patients referred to our genetic
skeletal disorders reference center for OI. Among the 15 patients (8%) with
polysomnographic recordings, 12 (6.4%) had sleep-disordered breathing. We found a
negative correlation between the Brief Assessment of Motor Function score and
Apnea Hypopnea Index (r=-0.68; p=0.01) and Desaturation Index (r=-0.62; p=0.02).
The Apnea Hypopnea Index was higher for non-walkers than walkers (mean [SD]: 6.5
[3.6] vs. 2.4 [1.5]; p=0.02) and with type III versus IV OI.
Two patients were
started on continuous positive airway pressure ventilation, with clinical
improvement. CONCLUSION:
For OI children, symptoms suggesting obstructive sleep
disorders should be searched for systematically, especially in children with
compromised autonomy, high body mass index, trunk deformations, and severe OI
type.
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Langue : ANGLAIS