Concentration of fractional excretion of nitric oxide (FENO): A potential airway biomarker of restored CFTR function

Kotha, Kavitha; Szczesniak, Rhonda; Naren, Anjaparavanda P.; Fenchel, Matthew; Duan, Leo L.; McPhail, Gary L.; Clancy, John

doi:10.1016/j.jcf.2015.07.002

Cited by 21 publications

(22 citation statements)

References 38 publications

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“…In the studies done by Hofer et al, and Kotha et al, there was no association between FeNO and type of organism (22,23).…”

Section: Discussionmentioning

confidence: 86%

“…The levels of FeNO in these patients did not show any significant difference before and after treatment and discharge. In addition, there was no significant rela-tionship between gender, age, type of micro-organisms obtained from sputum culture, and FeNO level (23). Jobsis et al, studied hydrogen peroxide and nitric oxide in the CF children's expiratory air after antibiotic treatment.…”

Section: Discussionmentioning

confidence: 99%

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Evaluation of Fraction of Exhaled Nitric Oxide (FeNO) in CF Children and its Association with Sputum Culture

et al. 2019

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Background: Exhaled nitric oxide (FeNO) in cystic fibrosis (CF) patients is reduced when compared with healthy people, and it has now been found that FeNO has a relative association with airway clearance index. Studies have shown the role of some infections in changing the FeNO level; however, the role of respiratory infections in FeNO has not yet been thoroughly studied. Objectives: The objective of this study was to investigate the possibility of FeNO usage to monitor the infections in CF patients. Methods: This cross-sectional case-control study was conducted on CF patients between the age of five to 18 with positive sputum culture, through simple non-random census method. FeNO levels were measured in 30 healthy children and 30 CF children with positive sputum culture. CF patients were treated by antibiotic therapy for two weeks; in the case offending negative sputum culture, the FeNO level was re-measured. FeNO levels were re-evaluated in 13 patients after four weeks. Results: There was no statistical difference between both groups in terms of age and weight. The level of FeNO in CF patients was significantly lower than in healthy children (22.1 ± 10.1 versus 30.0 ± 11.0 in the control group and P = 0.003). In 27 children, two weeks after administration of antibiotic therapy, sputum culture was negative. The mean of FeNO in these patients was 16.4 ± 5.5 at the time of the negative sputum culture, which was significantly lower than FeNO before starting the treatment. (P: 0.003). The mean FeNO was 13.0 ± 7.41 in 13 patients who were re-measured four weeks after starting the treatment. There was no significant difference between FeNO level two weeks after treatment and four weeks after starting treatment (P: 0.292). Patients with pseudomona sputum culture were not significantly different from those with non-pseudomona sputum culture in terms of primary FeNO and FeNO changes after the treatment (P value: 0.084 and 0.094, respectively). Conclusions: However, in our study, FeNO was decreased after administration of antibiotic treatment in CF patients, but according to the sample size and conflicting or similar results in other studies, currently, FeNO levels cannot be used as a way of monitoring the treatment of infection in CF patients.

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“…In the studies done by Hofer et al, and Kotha et al, there was no association between FeNO and type of organism (22,23).…”

Section: Discussionmentioning

confidence: 86%

Section: Discussionmentioning

confidence: 99%

Evaluation of Fraction of Exhaled Nitric Oxide (FeNO) in CF Children and its Association with Sputum Culture

et al. 2019

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“…There continues to be concern that these typical endpoints are insensitive to subtle improvements in lung disease in the youngest patients who have minimal detectable lung disease; LCI may be a more sensitive alternative, but is currently not sufficient to support FDA approval [62]. Other novel potential endpoints, such as CT imaging, MRI, hyperpolarized gas and FENO, may prove beneficial in younger CF patients but are not routinely used [70, 71, 85, 86]. Pulmonary exacerbations are not a reliable nor sensitive indicator of improvement in young children with CF given their mild lung disease and rarity of severe exacerbations.…”

Section: Discussionmentioning

confidence: 99%

“…However, improvements in FENO did not correlate with average predicted FEV 1 . In the second smaller, exclusively pediatric-focused study, 5 children with CF (mean age 13.6 years, mean predicted FEV 1 95.2%) had FENO measurements obtained before and after 4 weeks of ivacaftor treatment [86]. FENO increased by 5.2 ppb after ivacaftor treatment (p<0.05).…”

Section: Cftr Modulators To Assist Mutant Cftr Functionmentioning

confidence: 99%

CFTR modulator therapies in pediatric cystic fibrosis: focus on ivacaftor

Kramer

Clancy

2016

Expert Opinion on Orphan Drugs

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Introduction Mutations in the cystic fibrosis transmembrane conductance regulator protein (CFTR) cause cystic fibrosis (CF), a disease with life threatening pulmonary and gastrointestinal manifestations. Recent breakthrough therapies restore function to select disease-causing CFTR mutations. Ivacaftor is a small molecule that increases the open channel probability of certain CFTR mutations, producing clear evidence of bioactivity and efficacy in pediatric CF patients. CFTR modulators represent a significant advancement in CF treatment. Extending these therapies to young CF patients is proposed to have the greatest long term impact, potentially preventing later disease. Areas covered Here we summarize the research experience of CFTR modulators in pediatrics, focusing on ivacaftor and highlighting challenges in pediatric studies. As a result of these studies, ivacaftor has been approved in CF patients age 2 years and older who have one of ten CFTR mutations. Expert Opinion Conducting studies in young CF patients presents unique challenges, including small numbers of patients and difficulty selecting sensitive biomarkers and meaningful outcome measures. Adverse events may be more pronounced in children and deserve special attention. Ongoing efforts must focus on expanding and validating new biomarkers, innovative study design, and thorough monitoring of adverse events in children treated with CFTR modulators.

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“…The mechanisms resulting in reduced airway nitric oxide formation in CF are not completely understood, but recent studies suggest a direct link between cystic fibrosis transmembrane conductance regulator (CFTR) dysfunction and low nitric oxide production from nitric oxide synthases. Changes in F eNO may serve as a biomarker of restored CFTR function in the CF lower airway during CFTR modulator treatment [70]. The effect of ivacaftor on airway nitric oxide has been assessed for the first time by a Canadian research team.…”

Section: Infection and Inflammation In Cfmentioning

confidence: 99%

Key paediatric messages from Amsterdam

Grigg

Barben²,

Bohlin

et al. 2016

ERJ Open Res

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The Paediatric Assembly of the European Respiratory Society (ERS) maintained its high profile at the 2015 ERS International Congress in Amsterdam. There were symposia on preschool wheeze, respiratory sounds and cystic fibrosis; an educational skills workshop on paediatric respiratory resuscitation; a hot topic session on risk factors and early origins of respiratory diseases; a meet the expert session on paediatric lung function test reference values; and the annual paediatric grand round. In this report the Chairs of the Paediatric Assembly's Groups highlight the key messages from the abstracts presented at the Congress.

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Concentration of fractional excretion of nitric oxide (FENO): A potential airway biomarker of restored CFTR function

Abstract: Repeated FENO measurements were stable in CF patients, whereas FENO increased in all patients with CFTR gating mutations treated with ivacaftor. Acute changes in FENO may serve as a biomarker of restored CFTR function in the CF lower airway during CFTR modulator treatment.

Cited by 21 publications

References 38 publications

Evaluation of Fraction of Exhaled Nitric Oxide (FeNO) in CF Children and its Association with Sputum Culture

Evaluation of Fraction of Exhaled Nitric Oxide (FeNO) in CF Children and its Association with Sputum Culture

CFTR modulator therapies in pediatric cystic fibrosis: focus on ivacaftor

Key paediatric messages from Amsterdam

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