Premature vascular ageing in cystic fibrosis

MacNee, William

doi:10.1183/09031936.00155209

Cited by 10 publications

(5 citation statements)

References 24 publications

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“…In parallel, a variety of related fields [52–54] have observed an increase in the stiffness of various tissues with age. The increased condensation of intracellular and extracellular matrix components observed in aged tissues [55] cause an increase in tissue modulus.…”

Section: Discussionmentioning

confidence: 99%

Organ-derived coatings on electrospun nanofibers as ex vivo microenvironments

et al. 2011

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Idiopathic pulmonary fibrosis (IPF) is an interstitial lung disease characterized by irreversible scarring. Collagen deposition, myofibroblast expansion, and the development of fibroblastic foci are the hallmark pathological events. The origin and mechanism of recruitment of myofibroblasts, the key cell contributing to these events, is unknown. We hypothesize that the fibrotic lung microenvironment causes differentiation of arriving bone marrow-derived cells into myofibroblasts. Therefore, a method of isolating the effects of fibrotic microenvironment components on various cell types was developed. Electrospun nanofibers were coated with lung extracts from fibrotic or nonfibrotic mice and used to determine effects on bone marrow cells from naïve mice. Varying moduli nanofibers were also employed to determine matrix stiffness effects on these cells. At structured time points, bone marrow cell morphology was recorded and changes in fibrotic gene expression determined by real-time PCR. Cells plated on extracts isolated from fibrotic murine lungs secreted larger amounts of extracellular matrix, adopted a fibroblastic morphology, and exhibited increased myofibroblast gene expression after 8 and 14 days; cells plated on extracts from nonfibrotic lungs did not. Similar results were observed when the nanofiber modulus was increased. This ex vivo system appears to recapitulate the three-dimensional fibrotic lung microenvironment.

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Section: Discussionmentioning

confidence: 99%

Organ-derived coatings on electrospun nanofibers as ex vivo microenvironments

et al. 2011

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“…Adult patients with cystic fibrosis (CF) have evidence of haemodynamic alterations within the systemic circulation as depicted by an increase in the vascular parameter, augmentation index (AIx) [1,2]. These alterations are evident at clinical stability [1], appear exaggerated during light exercise [3] and may have deleterious consequences for myocardial work and perfusion of target organs, such as the brain and kidney [4].…”

Section: Introductionmentioning

confidence: 99%

Dynamic vascular changes following intravenous antibiotics in patients with cystic fibrosis

Hull

Garrod

et al. 2013

Journal of Cystic Fibrosis

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“…The negative effects of systemic changes on the cardiovascular system in patients with CF have been well documented [ 85 , 86 ]. MacNee demonstrated increased central arterial stiffening, an indicator of premature vascular aging observed in children with CF by Buehler [ 87 ].…”

Section: Changes Of the Retinal And Choroidal Vasculaturementioning

confidence: 99%

Ocular Changes in Cystic Fibrosis: A Review

Liberski,

Confalonieri,

Cofta

et al. 2024

IJMS

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Cystic fibrosis (CF), also known as mucoviscidosis, is the most common autosomal recessive genetic disease in the Caucasian population, with an estimated frequency of 1:2000–3000 live births. CF results from the mutation of the cystic fibrosis transmembrane conductance regulator (CFTR) gene localized in the long arm of chromosome 7. The product of CFTR gene expression is CFTR protein, an adenosine triphosphate (ATP)-binding cassette (ABC) transporter that regulates the transport of chloride ions (Cl−) across the apical cell membrane. Primary manifestations of CF include chronic lung and pancreas function impairment secondary to the production of thick, sticky mucus resulting from dehydrated secretions. It is well known that CF can cause both anterior and posterior ocular abnormalities. Conjunctival and corneal xerosis and dry eye disease symptoms are the most characteristic manifestations in the anterior segment. In contrast, the most typical anatomical and functional changes relating to the posterior segment of the eye include defects in the retinal nerve fiber layer (RNFL), vascular abnormalities, and visual disturbances, such as reduced contrast sensitivity and abnormal dark adaptation. However, the complete background of ophthalmic manifestations in the course of CF has yet to be discovered. This review summarizes the current knowledge regarding ocular changes in cystic fibrosis.

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Premature vascular ageing in cystic fibrosis

Cited by 10 publications

References 24 publications

Organ-derived coatings on electrospun nanofibers as ex vivo microenvironments

Organ-derived coatings on electrospun nanofibers as ex vivo microenvironments

Dynamic vascular changes following intravenous antibiotics in patients with cystic fibrosis

Ocular Changes in Cystic Fibrosis: A Review

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