Pulmonary hypertension as estimated by Doppler echocardiography in adolescent and adult patients with cystic fibrosis and their relationship with clinical, lung function and sleep findings

Ziegler, Bruna; Perin, Christiano; Casarotto, Fernanda Cano; Fagondes, Simone Chaves; Menna-Barreto, Sérgio Saldanha; Dalcin, Paulo de Tarso Roth

doi:10.1111/crj.12590

Cited by 9 publications

(10 citation statements)

References 46 publications

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“…Compared to healthy controls, adults with CF have more sleep fragmentation, a higher frequency of arousals, and a larger number of sleep stage transitions per hour of sleep (41, 42, 45). Both adolescents and adults with CF who have pulmonary hypertension, as compared to those who have normal pulmonary artery pressures, have lower sleep efficiency (21). They also have lower lung function, worse exercise tolerance, and greater abnormalities on chest radiography (21).…”

Section: Poor Sleep Qualitymentioning

confidence: 99%

“…Both adolescents and adults with CF who have pulmonary hypertension, as compared to those who have normal pulmonary artery pressures, have lower sleep efficiency (21). They also have lower lung function, worse exercise tolerance, and greater abnormalities on chest radiography (21). Whether the severe lung disease or the pulmonary hypertension itself predisposes these individuals to poor sleep quality needs further study.…”

Section: Poor Sleep Qualitymentioning

confidence: 99%

“…Nocturnal hypoxemia has been linked to poor subjective sleep quality, a reduced sleep efficiency and total sleep duration among children and adults with CF (38, 41, 43, 55, 56). The frequency of elevated pulmonary artery systolic pressures and pulmonary hypertension is higher among patients with CF who experience desaturations during sleep (21, 41, 49). Patients with pulmonary hypertension also have hypoxemia during exercise (21, 41), and lower daytime oxygen saturations as compared to those without this comorbidity (21).…”

Section: Nocturnal Hypoxemiamentioning

confidence: 99%

“…The frequency of elevated pulmonary artery systolic pressures and pulmonary hypertension is higher among patients with CF who experience desaturations during sleep (21, 41, 49). Patients with pulmonary hypertension also have hypoxemia during exercise (21, 41), and lower daytime oxygen saturations as compared to those without this comorbidity (21). Nocturnal hypoxemia may be the missing piece in a mechanistic link between poor sleep and CF disease severity, given that low oxygen levels during sleep are independently associated with severe CF lung disease and disrupted or fragmented sleep.…”

Section: Nocturnal Hypoxemiamentioning

confidence: 99%

“…Cystic Fibrosis Related Diabetes (CFRD), from a combination of insulin deficiency and insulin resistance, is a common complication, and one that has a negative impact on lung function, nutritional status and survival (20). Pulmonary hypertension, frequently seen among patients with CF who have advanced lung disease, is also associated with significant morbidity and mortality (21). The protein that is affected in CF, the cystic fibrosis transmembrane conductance regulator (CFTR), has been identified in immune cells (lymphocytes) and neural cells (anterior hypothalamus and spinal cord), although understanding of its role in these areas is still emerging (22).…”

Section: Introductionmentioning

confidence: 99%

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Sleep disturbances and their impact in pediatric cystic fibrosis

Shakkottai

O’Brien

Nasr

et al. 2018

Sleep Medicine Reviews

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Cystic fibrosis is a chronic, life-shortening illness that affects multiple systems and results in frequent respiratory infections, chronic cough, fat malabsorption and malnutrition. Poor sleep is often reported by patients with cystic fibrosis. Although objective data to explain these complaints have been limited, they do show poor sleep efficiency and frequent arousals. Abnormalities in gas exchange are also observed during sleep in patients with cystic fibrosis. The potential impact of these abnormalities in sleep on health and quality of life remains largely unstudied. This review summarizes what is known about sleep in children with cystic fibrosis, and implications for clinical practice. This report also highlights new evidence on the impact of sleep problems on disease-specific outcomes such as lung function, and identifies areas that need further exploration.

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Section: Poor Sleep Qualitymentioning

confidence: 99%

Section: Poor Sleep Qualitymentioning

confidence: 99%

Section: Nocturnal Hypoxemiamentioning

confidence: 99%

Section: Nocturnal Hypoxemiamentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Sleep disturbances and their impact in pediatric cystic fibrosis

Shakkottai

O’Brien

Nasr

et al. 2018

Sleep Medicine Reviews

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Risk factors for obstructive sleep apnea in cystic fibrosis

Shakkottai

Irani

O’Brien

et al. 2022

Pediatric Pulmonology

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Introduction: Despite emerging data that suggest a high frequency and severity of obstructive sleep apnea (OSA) among patients with cystic fibrosis (CF), few of them are referred for polysomnography. Little is known about which patients with CF are at increased risk for OSA and which sleep symptoms merit investigation. Methods: A single-center retrospective analysis of clinical and polysomnographic data from 2009, January 1 to October 31, 2020 in referred children and adults with CF.Results: Among 74 patients (42 children, 32 adults) with CF, 39 (53%) had OSA. No age or sex differences emerged in OSA frequency. Mean apnea-hypopnea index (AHI) was higher among overweight/obese adults (n = 16) as compared with adults of normal weight or underweight (11.4 vs. 6.2; p = 0.005). Adults with (n = 10) versus without a crowded oropharynx had 13.0 times greater odds of OSA (95% confidence interval (CI): 1.4, 121.4; p = 0.02). Children with (n = 24) versus without tonsillar hypertrophy had a higher risk for OSA (OR = 5.2; 95% CI: 1.4, 19.8; p = 0.02), as did children with (n = 10) versus without symptomatic chronic sinusitis (OR = 5.8; 95% CI: 1.1, 32.1; p = 0.04). Neither snoring, excessive daytime sleepiness, nor lung disease severity were associated with OSA. Conclusion:Key risk factors for OSA may differ between children and adults with CF: upper airway pathology appears important in children and overweight/obesity or a crowded oropharynx in adults. Given the lack of sensitivity of snoring, daytime sleepiness, and lung disease severity, detection of OSA may require a low threshold for polysomnographic assessment in this vulnerable population.

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