Cystic fibrosis increasingly associated with sleep apnea in children and adolescents

The frequency of obstructive sleep apnea (OSA) may be high among patients with cystic fibrosis (CF). It is a life-shortening, genetic respiratory disease that affects approximately 30,000 Americans. Sleep disorders in cystic fibrosis are known to present before daytime clinical manifestations, lung function impairment, affecting quality of life and disease progression.

A systematic review study by Luisa Pedrada de Sousa and team has revealed that A high prevalence of obstructive sleep apnea was found in children and adolescents with cystic fibrosis, regardless of age and lung function impairment, hence pointing out the importance of investigating sleep-disordered breathing during clinical visits even when lung function is not yet compromised.

The findings of the study are published in Sleep Medicine journal.

The objective of the study was to evaluate the prevalence of obstructive sleep apnea in children and adolescents with cystic fibrosis and preserved lung function or mild impairment, and evaluated its association with clinical variables.

The study was a systematic review with meta-analysis of prevalence was conducted, including observational studies with polysomnographies in patients with cystic fibrosis who presented mean lung function values > 60% predicted. The methodological quality of the studies was analyzed, and a meta-analysis was performed to assess the prevalence of obstructive sleep apnea.

The results of the study were

• A total of 2318 studies identified, 7 were included in the systematic review and 6 in the meta-analysis of prevalence.

• The confounding factors and strategies identified were the items with greatest weakness in the methodological quality assessment.

• Most studies were cross-sectional, and sample size ranged from 9 to 67 individuals. The most frequent criterion for defining obstructive sleep apnea was apnea-hypopnea index (AHI) > 1 per hour.

• The prevalence found ranged from 32.3 to 100% and the pooled prevalence was 65% (I2 = 53.4%), considering AHI>1, and 52% (I2 = 89.4%) for AHI>2 per hour. It was not possible to verify the association between obstructive sleep apnea and clinical variables.

Sousa and team concluded that "A high prevalence of obstructive sleep apnea in children and adolescents with cystic fibrosis was found, regardless of age and lung function impairment, reinforcing the importance of investigating sleep-disordered breathing during clinical visits even when lung function is not yet compromised."

Reference: de Sousa LP, Liberato FMG, Vendrusculo FM, Donadio MVF, Barbosa RRB. Obstructive sleep apnea in children and adolescents with cystic fibrosis and preserved lung function or mild impairment: a systematic review and meta-analysis of prevalence. Sleep Med. 2021 Oct 1;88:36-43. doi: 10.1016/j.sleep.2021.09.017. Epub ahead of print. PMID: 34731826.