Evidence of Vascular Endothelial Dysfunction in Young Patients With Cystic Fibrosis

Poore, S.; Berry, Breana; Eidson, Dabney; McKie, Kathleen T.; Harris, Ryan A.

doi:10.1378/chest.12-1934

Cited by 70 publications

(88 citation statements)

References 32 publications

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“…Recently, our group reported conduit vascular endothelial dysfunction in young patients with CF (39). Importantly, this vascular impairment was observed in a population that exhibited preserved spirometric function and exercise capacity, which suggests a systemic consequence prior to the decrease in pulmonary function.…”

Section: For the First Time We Have Identified An Impairment In Micrmentioning

confidence: 89%

Evidence of microvascular dysfunction in patients with cystic fibrosis

Rodriguez‐Miguelez

Thomas

Seigler

et al. 2016

American Journal of Physiology-Heart and Circulatory Physiology

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Rodriguez-Miguelez P, Thomas J, Seigler N, Crandall R, McKie KT, Forseen C, Harris RA. Evidence of microvascular dysfunction in patients with cystic fibrosis. Am J Physiol Heart Circ Physiol 310: H1479 -H1485, 2016. First published April 15, 2016 doi:10.1152/ajpheart.00136.2016.-Cystic fibrosis (CF) is a genetic, multisystemic disorder with broad clinical manifestations apart from the well-characterized pulmonary dysfunction. Recent findings have described impairment in conduit vessel function in patients with CF; however, whether microvascular function is affected in this population has yet to be elucidated. Using laser-Doppler imaging, we evaluated microvascular function through postocclusive reactive hyperemia (PORH), local thermal hyperemia (LTH), and iontophoresis with acetylcholine (ACh). PORH [518 Ϯ 174% (CF) and 801 Ϯ 125% (control), P ϭ 0.039], LTH [1,338 Ϯ 436% (CF) and 1,574 Ϯ 620% (control), P ϭ 0.045], and iontophoresis with ACh [416 Ϯ 140% (CF) and 617 Ϯ 143% (control), P ϭ 0.032] were significantly lower in patients with CF than control subjects. In addition, the ratio of PORH to LTH was significantly (P ϭ 0.043) lower in patients with CF (55.3 Ϯ 5.1%) than control subjects (68.8 Ϯ 3.1%). Significant positive correlations between LTH and forced expiratory volume in 1 s (%predicted) (r ϭ 0.441, P ϭ 0.013) and between the PORH-to-LTH ratio and exercise capacity (r ϭ 0.350, P ϭ 0.049) were observed. These data provide evidence of microvascular dysfunction in patients with CF compared with control subjects. In addition, our data demonstrate a complex relationship between microvascular function and classical markers of disease severity (i.e., pulmonary function and exercise capacity) in CF. CYSTIC FIBROSIS (CF) is an inherited disorder that is predominantly characterized by pulmonary dysfunction; however, broader clinical manifestations, such as gastrointestinal, immune, endocrine, and musculoskeletal dysfunctions, are also present (36). Recently, our group reported conduit vascular endothelial dysfunction in young patients with CF (39). Importantly, this vascular impairment was observed in a population that exhibited preserved spirometric function and exercise capacity, which suggests a systemic consequence prior to the decrease in pulmonary function. Despite these recent observations, there appears to be a lack of understanding of the general functionality of the endothelium in CF.Impairments in endothelial function are highly related to a reduction in the bioavailability of nitric oxide (NO), a key vasodilator involved in the regulation of vessel tone and endothelium integrity (24). Decreases in NO balance have also been shown to contribute to impaired endothelial microvascular vasodilation (44). With use of laser-Doppler imaging technology, cutaneous blood flux can be quantified (45) to represent an accessible model of microvascular function (17). Postocclusive reactive hyperemia (PORH), local thermal hyperemia (LTH), and iontophoresis with acetylcholine (ACh), in combination with laser technology...

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Section: For the First Time We Have Identified An Impairment In Micrmentioning

confidence: 89%

Evidence of microvascular dysfunction in patients with cystic fibrosis

Rodriguez‐Miguelez

Thomas

Seigler

et al. 2016

American Journal of Physiology-Heart and Circulatory Physiology

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“…Although not well-defined, chronic systemic inflammation plays a role with associated alterations of the endothelium (29-34) with differences reported between CF and other lung diseases (31). Recently, endothelial dysfunction was reported in young, healthy CF patients compared with matched control subjects (32). Using a validated technique to evaluate arterial wall integrity (35) in a cross-sectional study involving 50 adult CF patients, Hull and coworkers (33) found that arterial stiffness was significantly increased in the presence of a normal blood pressure and independent of diabetic status, while being associated with systemic inflammation detected by C-reactive protein levels.…”

Section: Discussionmentioning

confidence: 99%

Pulmonary Hypertension in Cystic Fibrosis with Advanced Lung Disease

Hayes

Tobias

Mansour

et al. 2014

Am J Respir Crit Care Med

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“…Few studies have evaluated the effects of PCN on the vascular system to date, and those available are limited to in vitro studies [29,77,78]. However, P. aeruginosa is a rare cause of infective endocarditis and is seen primarily in injection drug users and patients undergoing cardiac procedures [3,4,5,6].…”

Section: Manifestations Of Pcn Exposurementioning

confidence: 99%

Cellular Effects of Pyocyanin, a Secreted Virulence Factor of Pseudomonas aeruginosa

Hall

McDermott

Anoopkumar‐Dukie

et al. 2016

Toxins

325

254

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Pyocyanin has recently emerged as an important virulence factor produced by Pseudomonas aeruginosa. The redox-active tricyclic zwitterion has been shown to have a number of potential effects on various organ systems in vitro, including the respiratory, cardiovascular, urological, and central nervous systems. It has been shown that a large number of the effects to these systems are via the formation of reactive oxygen species. The limitations of studies are, to date, focused on the localized effect of the release of pyocyanin (PCN). It has been postulated that, given its chemical properties, PCN is able to readily cross biological membranes, however studies have yet to be undertaken to evaluate this effect. This review highlights the possible manifestations of PCN exposure; however, most studies to date are in vitro. Further high quality in vivo studies are needed to fully assess the physiological manifestations of PCN exposure on the various body systems.

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Evidence of Vascular Endothelial Dysfunction in Young Patients With Cystic Fibrosis

Cited by 70 publications

References 32 publications

Evidence of microvascular dysfunction in patients with cystic fibrosis

Evidence of microvascular dysfunction in patients with cystic fibrosis

Pulmonary Hypertension in Cystic Fibrosis with Advanced Lung Disease

Cellular Effects of Pyocyanin, a Secreted Virulence Factor of Pseudomonas aeruginosa

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