Objectives To determine: (a) prevalence of clinically unsuspected nocturnal hypoventilation (NH) in a clinic population of children with progressive neuromuscular disease; (b) whether NH can be predicted from clinical/laboratory parameters; and (c) change over 1 year in pulmonary function decline, quality of life and attention in children with NH treated with non-invasive positive pressure ventilation (NPPV) compared with children without NH.
Design Prospective cohort study.
Setting Two tertiary-care paediatric neuromuscular clinics.
Patients 46 children (6–17 years) with progressive neuromuscular disease without neurocognitive impairment or dystrophinopathy.
Interventions Polysomnography, pulmonary function, manual muscle strength, quality of life (CHQ-PF50) and Conners questionnaires.
Outcome measures (a) Prevalence of NH; (b) predictive value of surrogate clinical measures for NH; and (c) differences in change over 1 year in pulmonary function, muscle strength, quality of life and attention between children with and without NH.
Results Prevalence of NH was 14.8%, 95% CI 8.0% to 25.7%. Maximal sensitivity and specificity for NH were achieved with thresholds of forced vital capacity <70% and forced expiratory volume in 1 s <65% predicted (sensitivities: 71.4, 71.4; specificities: 64.1, 79.5). Scoliosis also predicted NH (sensitivity 88.9; specificity 80.4). Over 1 year, those with NH had a greater increase in residual volume/total lung capacity (0.075 (−0.003 to 0.168) vs −0.03 (−0.065 to 0.028)), decline in muscle strength (−0.67 (−0.90 to 0.10) vs 0.53 (−0.05 to 0.90)) and worsened perception of health status.
Conclusions 15% of subjects had clinically unsuspected NH, predicted by moderate pulmonary function test impairment and scoliosis. Over 1 year those with NH had increased gas trapping, decline of muscle strength and worse perception of health status, despite NPPV.
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Funding The Hospital for Sick Children Foundation, CHEO Research Institute, Ontario Lung Association, Canadian Nurses Respiratory Society.
Competing interests None.
Ethics approval This study was conducted with the approval of the Children's Hospital of Eastern Ontario Research Ethics Board and Hospital for Sick Children Research Ethics Board.
Provenance and peer review Not commissioned; externally peer reviewed.
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