Sleep-related breathing disorder in Duchenne muscular dystrophy: Disease spectrum in the paediatric population


Autoria(s): Suresh, S.; Wales, P.; Dakin, C.; Harris, M. A.; Cooper, D. M.
Data(s)

01/09/2005

Resumo

Duchenne muscular dystrophy (DMD) is a progressive neuromuscular disease with death usually occurring because of respiratory failure. Signs of early respiratory insufficiency are usually first detectable in sleep. Objective: To study the presentation of sleep-related breathing disorder (SRBD) in patients with DMD. Method:> A retrospective review of patients with DMD attending a tertiary paediatric sleep disorder clinic over a 5-year period. Symptoms, lung function and polysomnographic indices were reviewed. Results: A total of 34 patients with DMD were referred for respiratory assessment (1-15 years). Twenty-two (64%) reported sleep-related symptomatology. Forced vital capacity (FVC) was between 12 and 107% predicted (n = 29). Thirty-two progressed to have polysomnography of which 15 were normal studies (median age: 10 years) and 10 (31%) were diagnostic of obstructive sleep apnoea (OSA) (median age: 8 years). A total of 11 patients (32%) showed hypoventilation (median age: 13 years) during the 5-year period and non-invasive ventilation (NIV) was offered to them. The median FVC of this group was 27% predicted. There was a significant improvement in the apnoea/hypopnoea index (AHI) (mean difference = 11.31, 95% CI = 5.91-16.70, P = 0.001) following the institution of NIV. Conclusions: The prevalence of SRBD in DMD is significant. There is a bimodal presentation of SRBD, with OSA found in the first decade and hypoventilation more commonly seen at the beginning of the second decade. Polysomnography is recommended in children with symptoms of OSA, or at the stage of becoming wheelchair-bound. In patients with the early stages of respiratory failure, assessment with polysomnography-identified sleep hypoventilation and assisted in initiating NIV.

Identificador

http://espace.library.uq.edu.au/view/UQ:75604

Idioma(s)

eng

Publicador

Blackwell Publishing Asia

Palavras-Chave #Pediatrics #Duchenne Muscular Dystrophy #Hypoventilation #Non-invasive Ventilation #Obstructive Sleep Apnoea #Polysomnography #Sleep-related Breathing Disorder #Positive-pressure Ventilation #Neuromuscular Disease #Nasal Ventilation #Noninvasive Ventilation #Respiratory-failure #Daytime Predictors #Children #Experience #C1 #321019 Paediatrics #321027 Respiratory Diseases #730204 Child health #730110 Respiratory system and diseases (incl. asthma)
Tipo

Journal Article