Narrowing in on Early Cystic Fibrosis Lung Disease

Zemanick, Edith T.; Hoffman, Lucas R.; Rosenfeld, Margaret

doi:10.1164/rccm.201410-1929ed

Cited by 5 publications

(4 citation statements)

References 20 publications

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“…It has long been appreciated that newborns with CF can have serious pancreatic insufficiency and gastrointestinal complications related to their disease, as discussed in two recent reviews [7,8]. In contrast, only fairly recently reviews [9][10][11] and critical analyses [12,13] have begun to suggest that abnormalities of the respiratory system consistent with early disease progression can be observed early in life in children with CF. To date, a comprehensive review of the ages of earliest disease manifestation and progression across organ systems is lacking, which represents a knowledge gap.…”

Section: Introductionmentioning

confidence: 99%

Cystic fibrosis in young children: A review of disease manifestation, progression, and response to early treatment

VanDevanter

Kahle²,

O’Sullivan

et al. 2016

Journal of Cystic Fibrosis

122

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Section: Introductionmentioning

confidence: 99%

Cystic fibrosis in young children: A review of disease manifestation, progression, and response to early treatment

VanDevanter

Kahle²,

O’Sullivan

et al. 2016

Journal of Cystic Fibrosis

122

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“…With the recent development of disease-modifying treatments for CF, there is a need for noninvasive techniques to monitor disease development and progression in young children [3,4]. While lung function tests offer an alternative to invasive surveillance, studies correlating disease outcomes and respiratory function have predominantly been limited to those using spirometry [5][6][7] or techniques that require sedation [8][9][10][11][12][13][14], and there have been relatively few studies in preschool-aged children [8,[15][16][17][18][19][20].…”

Section: Introductionmentioning

confidence: 99%

Impact of lung disease on respiratory impedance in young children with cystic fibrosis

et al. 2015

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This study aimed to evaluate the ability of the forced oscillation technique (FOT) to detect underlying lung disease in preschool children with cystic fibrosis (CF) diagnosed following newborn screening.184 children (aged 3-6 years) with CF underwent lung function testing on 422 occasions using the FOT to assess respiratory resistance and reactance at the time of their annual bronchoalveolar lavage collection and chest computed tomography scan. We examined associations between FOT outcomes and the presence and progression of respiratory inflammation, infection and structural lung disease.Children with CF who had pronounced respiratory disease, including free neutrophil elastase activity, infection with pro-inflammatory pathogens and structural lung abnormalities had similar FOT outcomes to those children without detectable lung disease. In addition, the progression of lung disease over 1 year was not associated with worsening FOT outcomes.We conclude that the forced oscillation technique is relatively insensitive to detect underlying lung disease in preschool children with CF. However, FOT may still be of value in improving our understanding of the physiological changes associated with early CF lung disease. @ERSpublications Forced oscillation technique is insensitive in detecting lung disease in preschool children with cystic fibrosis http://ow.ly/R9rSU

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“…Colonisation by A. fumigatus is defined as the presence of this fungus in laboratory cultures of the respiratory tract without clinical symptoms or deterioration in lung function (table 1 [13,40,41]) [16,25,42]. The exact prevalence of colonisation in PwCF is not well known and reports vary at between 3.4% and 57% [11,38,43].…”

Section: Colonisationmentioning

confidence: 99%

Aspergillus-related lung disease in people with cystic fibrosis: can imaging help us to diagnose disease?

Elders

Warris

et al. 2021

Eur Respir Rev

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In people with cystic fibrosis (PwCF), viscous sputum and dysfunction of the mucociliary escalator leads to early and chronic infections. The prevalence of Aspergillus fumigatus in sputum is high in PwCF and the contribution of A. fumigatus to the progression of structural lung disease has been reported. However, overall, relatively little is known about the contribution of A. fumigatus to CF lung disease. More knowledge is needed to aid clinical decisions on whether to start antifungal treatment. In this review, we give an overview of A. fumigatus colonisation and infection in PwCF and the different types of pulmonary disease caused by it. Furthermore, we discuss the current evidence for structural lung damage associated with A. fumigatus in PwCF on chest computed tomography and magnetic resonance imaging. We conclude that radiological outcomes to identify disease caused by A. fumigatus can be important for clinical studies and management.

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Narrowing in on Early Cystic Fibrosis Lung Disease

Cited by 5 publications

References 20 publications

Cystic fibrosis in young children: A review of disease manifestation, progression, and response to early treatment

Cystic fibrosis in young children: A review of disease manifestation, progression, and response to early treatment

Impact of lung disease on respiratory impedance in young children with cystic fibrosis

Aspergillus-related lung disease in people with cystic fibrosis: can imaging help us to diagnose disease?

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