L’indice de clairance pulmonaire dans la mucoviscidose

Poncin, William; Lebecque, Patrick

doi:10.1016/j.rmr.2018.03.007

Cited by 4 publications

(2 citation statements)

References 135 publications

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“…The gold standard for diagnosis of SRDs is polysomnography, but respiratory polygraphy has been shown to be an attested substitute for well-studied sleep in these patients [45,46]. In fact, daytime measurements of oxygen saturation, lung function as FEV1 or LCI, and arterial blood gas measurements are not useful alone in predicting SRDs [15][16][17][18].…”

Section: Discussionmentioning

confidence: 99%

“…Recently, the Lung Clearance Index (LCI), reflecting the overall degree of ventilation inhomogeneity, obtained from a multiple-breath washout of an inert gas marker, has been widely used in CF and PCD children as it studies the distal airways and detects lung damage more accurately than spirometry [17]. It has been shown that LCI has a high effectiveness in predicting sleep disorder breathing, especially nocturnal hypoxemia, in stable CF patients compared with standard parameters of lung function variables, such as FEV1 [18].…”

Section: Srds In Cf Patientsmentioning

confidence: 99%

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Sleep Respiratory Disorders in Children and Adolescents with Cystic Fibrosis and Primary Ciliary Dyskinesia

Papale,

Manti,

Presti

et al. 2023

Children

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Cystic fibrosis (CF) and primary ciliary dyskinesia (PCD) are genetic respiratory diseases featured by chronic upper and lower airway inflammation and infection, mainly due to impaired mucociliary clearance due to genetic mutations. Sleep is crucial to healthy children’s normal physical and psychological development and has an important value in chronic respiratory diseases. Impaired sleep quality, such as sleep deprivation or insufficient sleep during the night, and sleep respiratory disorders (SRDs) are common in 5% to 30% of the general population. Sleep disruption leads to attention deficits, daytime sleepiness, fatigue and mood disorders and correlates to a worsened quality of life. Furthermore, sleep respiratory disorders (SRSs) are under-recognized comorbidities in CF and PCD patients. SRSs include a spectrum of symptoms ranging from primary snoring through upper airway resistance to obstructive sleep apnea (OSA), nocturnal hypoventilation and hypoxemia occurring in people with moderate to severe lung disease and damaging the disease-related outcomes and quality of life. Effective screening during sleep with polysomnography is very important for the timely initiation of efficacious treatments and to prevent worsened respiratory, metabolic and cardiovascular outcomes. However, the impact of SRDs on health and quality of life is still underinvestigated.

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Section: Discussionmentioning

confidence: 99%

Section: Srds In Cf Patientsmentioning

confidence: 99%

Sleep Respiratory Disorders in Children and Adolescents with Cystic Fibrosis and Primary Ciliary Dyskinesia

Papale,

Manti,

Presti

et al. 2023

Children

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The intermittent intrapulmonary deflation technique for airway clearance in patients with cystic fibrosis: A randomized trial

de Macedo,

Aubriot,

Reychler

et al. 2024

Respiratory Medicine and Research

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Will Airway Gene Therapy for Cystic Fibrosis Improve Lung Function? New Imaging Technologies Can Help Us Find Out

Parsons

Donnelley

2020

Human Gene Therapy

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The promise of genetic therapies has turned into reality in recent years, with new first-line treatments for fatal diseases now available to patients. The development and testing of genetic therapies for respiratory diseases such as cystic fibrosis (CF) has also progressed. The addition of gene editing to the genetic agent toolbox, and its early success in other organ systems, suggests we will see rapid expansion of gene correction options for CF in the future. Although substantial progress has been made in creating techniques and genetic agents that can be highly effective for CF correction in vitro, physiologically relevant functional in vivo changes have been largely prevented by poor delivery efficiency within the lungs. Somewhat hidden from view, however, is the absence of reliable, accurate, detailed, and noninvasive outcome measures that can detect subtle disease and treatment effects in the lungs of humans or animal models. The ability to measure the fundamental function of the lung-ventilation, the effective transport of air throughout the lung-has been constrained by the available measurement technologies. Without sensitive measurement methods, it is difficult to quantify the effectiveness of genetic therapies for CF. The mainstays of lung health assessment are spirometry, which cannot provide adequate disease localization and is not sensitive enough to detect small early changes in disease; and computed tomography, which provides structural rather than functional information. Magnetic resonance imaging using hyperpolarized gases is increasingly useful for lung ventilation assessment, and it removes the radiation risk that accompanies X-ray methods. A new lung imaging technique, X-ray velocimetry, can now offer highly detailed regional lung ventilation information well suited to the diagnosis, treatment, and monitoring needs of CF lung disease, particularly after the application of genetic therapies. In this review, we discuss the options now available for imaging-based lung function measurement in the generation and use of genetic and other therapies for treating CF lung disease.

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L’indice de clairance pulmonaire dans la mucoviscidose

Cited by 4 publications

References 135 publications

Sleep Respiratory Disorders in Children and Adolescents with Cystic Fibrosis and Primary Ciliary Dyskinesia

Sleep Respiratory Disorders in Children and Adolescents with Cystic Fibrosis and Primary Ciliary Dyskinesia

The intermittent intrapulmonary deflation technique for airway clearance in patients with cystic fibrosis: A randomized trial

Will Airway Gene Therapy for Cystic Fibrosis Improve Lung Function? New Imaging Technologies Can Help Us Find Out

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