Year: 2017
Type: dissertation
Study design: Observational Study
Abstract: Introduction: Sleep disordered breathing (SDB) is seen in many children with Duchenne muscular dystrophy (DMD).It results in breathing difficulties during sleep which progress to daytime difficulties as well but can initially be alleviated by non-invasive ventilation.The gold standard for diagnosing SDB is by an overnight sleep study (polysomnogram, PSG) which is expensive and labour intensive.There is no consensus about the optimal timing for the initial overnight PSG in DMD.Aim: To find a daytime predictor or combination or predictors for the best time to commence overnight sleep studies for the investigation of sleep disordered breathing in Duchenne muscular dystrophy.Methods: Twenty boys with DMD, aged 10 -16 years or with symptoms of sleep disordered breathing and not previously diagnosed with nocturnal hypoventilation, had two PSGs 12 months apart.Concurrent clinical information (FVC, SNIP, PCF, age, BMI, symptoms, scoliosis and cardiac involvement) was collected and correlated to the outcomes of sleep studies (RDI, ORDI, CRDI, REM RDI, REM ORDI, REM CRDI, TcpCO2 & SpO2 nadir).Results: 90% of boys had OSA at PSG#1 which was not detected by questions regarding relevant symptoms.None were diagnosed with NH (defined by TcpCO2 > 50 mmHg > 25% TST).At PSG#2 there was a decline in daytime predictor values, although no subjects met diagnostic criteria for NH.Correlations between daytime predictors and PSG outcomes were not strong enough, in part due to the large variability in the data, to predict the future onset of NH.Conclusion: Boys younger than 10 years of age with DMD need to be screened with PSG for OSA due to the 90% incidence in this cohort.Larger numbers are required for future studies to enhance data quality.NH is unlikely to be detected by PSGs in boys with DMD aged<14years.Future studies in this cohort will identify when NH becomes apparent and predictors of this change in this population.PSG -polysomnography, FVC -forced vital capacity, SNIP -sniff nasal inspiratory pressure, PCF -peak cough flow, BMI -body mass index, RDI -respiratory disturbance index, ORDI -obstructive RDI, CRDI -central RDI, REM RDI -RDI in REM, REM ORDI -ORDI in REM, REM CRDI -CRDI in REM, TcpCO2 -transcutaneous partial pressure of carbon dioxide & SpO2 nadir -lowest oxygen saturation 5.3.11SNIP ...........
Author: Mary Roduta Roberts
Language: English
Subfield: Physiology (medical)
Field: Medicine
Domain: Health Sciences
Open Access status: green
OpenAlex ID: W2769642579