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      Association of vitamin A status with lung function in children and adolescents with cystic fibrosis

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          ABSTRACT

          Importance

          Vitamin A (or retinol) has considerable antioxidative and anti‐inflammatory attributes and it may have protective effects on the respiratory health of patients with cystic fibrosis (CF). This issue, however, remains controversial.

          Objective

          The purpose of the present study was to investigate the relationship between serum retinol levels (SRL) and force expiratory volume in 1 second (FEV1) in patients with CF.

          Methods

          All patients with pancreatic insufficiency attending the CF Department of “Agia Sofia” Children’s Hospital in Athens, Greece, aged 6 to 19 years during the 2012–2016 period, who could perform spirometry effectively, were included in the study. The impact of SRL on FEV1 was examined longitudinally and analyzed with generalized estimating equations.

          Results

          The study included 231 patients and 851 SRL measurements were performed. In 25 (3.2%) cases the SRL were below the 5th percentile of reference distribution; none was above the 95th percentile. The analysis showed that SRL was positively correlated with the FEV1 ( P < 0.001).

          Interpretation

          In this sample of children and adolescents with CF, vitamin A deficiency was rare. Our results suggest a positive relationship between SRL and FEV1.

          Abstract

          Vitamin A serum levels are correlated with pulmonary function in children and adolescents with cystic fibrosis.

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          Most cited references25

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          Inflammation in cystic fibrosis lung disease: Pathogenesis and therapy.

          André Cantin, Dominik Hartl, Michael Konstan … (2015)
          Lung disease is the major cause of morbidity and mortality in patients with cystic fibrosis (CF). Although CF lung disease is primarily an infectious disorder, the associated inflammation is both intense and ineffective at clearing pathogens. Persistent high-intensity inflammation leads to permanent structural damage of the CF airways and impaired lung function that eventually results in respiratory failure and death. Several defective inflammatory responses have been linked to cystic fibrosis transmembrane conductance regulator (CFTR) deficiency including innate and acquired immunity dysregulation, cell membrane lipid abnormalities, various transcription factor signaling defects, as well as altered kinase and toll-like receptor responses. The inflammation of the CF lung is dominated by neutrophils that release oxidants and proteases, particularly elastase. Neutrophil elastase in the CF airway secretions precedes the appearance of bronchiectasis, and correlates with lung function deterioration and respiratory exacerbations. Anti-inflammatory therapies are therefore of particular interest for CF lung disease but must be carefully studied to avoid suppressing critical elements of the inflammatory response and thus worsening infection. This review examines the role of inflammation in the pathogenesis of CF lung disease, summarizes the results of past clinical trials and explores promising new anti-inflammatory options.
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            Lung volumes and forced ventilatory flows.

            G J Tammeling, R Peslin, P Quanjer … (1993)
            Article 0 comments Cited 164 times – based on 0 reviews     Review now
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              Effect of genotype on phenotype and mortality in cystic fibrosis: a retrospective cohort study.

              Nadia Emerson, Edward McKone, Samuel Aitken … (2003)
              Over 1000 mutations of the cystic fibrosis transmembrane conductance regulator gene (CFTR) that cause cystic fibrosis have been identified. We examined the effect of CFTR genotype on mortality and disease phenotype. Using the US Cystic Fibrosis Foundation National Registry, we did a retrospective cohort study to compare standardised mortality rates for the 11 most common genotypes heterozygous for DeltaF508 with those homozygous for DeltaF508. Of the 28455 patients enrolled in the registry at the time of our analysis, 17853 (63%) were genotyped. We also compared the clinical phenotype, including lung function, age at diagnosis, and nutritional measures, of 22 DeltaF508 heterozygous genotypes. Mortality rates and clinical phenotype were also compared between genotypes classified into six classes on the basis of their functional effect on CFTR production. Between 1991 and 1999, genetic and clinical data were available for 17853 patients with cystic fibrosis, which was 63% of the total cohort. There were 1547 deaths during the 9 years of follow-up. In the analysis of the 11 most common genotypes, DeltaF508/R117H, DeltaF508/DeltaI507, DeltaF508/3849+10kbC-->T, and DeltaF508/2789+5G-->A had a significantly lower mortality rate (4.7, 8.0, 11.9, and 4.4, respectively) than the genotype homozygous for DeltaF508 (21.8, p=0.0060). DeltaF508/R117H, DeltaF508/DeltaI507, DeltaF508/ 3849+10 kbC-->T, DeltaF508/2789+5G-->A, and DeltaF508/A455E have a milder clinical phenotype. Outcomes for all functional classes were compared with that of class II (containing DeltaF508 homozygotes) and classes IV and V had a significantly lower mortality rate and milder clinical phenotype. Patients with cystic fibrosis have distinct genetic subgroups that are associated with mild clinical manifestations and low mortality. These differences in phenotype are also related to the functional classification of CFTR genotype.
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                Author and article information

                Contributors
                Konstantinos Douros: costasdouros@gmail.com
                Journal
                Journal ID (nlm-ta): Pediatr Investig
                Journal ID (iso-abbrev): Pediatr Investig
                Journal ID (doi): 10.1002/(ISSN)2574-2272
                Journal ID (publisher-id): PED4
                Title: Pediatric Investigation
                Publisher: John Wiley and Sons Inc. (Hoboken )
                ISSN (Print): 2096-3726
                ISSN (Electronic): 2574-2272
                Publication date (Electronic): 18 June 2021
                Publication date Collection: June 2021
                Volume: 5
                Issue: 2 ( doiID: 10.1002/ped4.v5.2 )
                Pages: 125-129
                Affiliations
                [ 1 ] Cystic Fibrosis Department “Agia Sofia” Children’s Hospital Athens Greece
                [ 2 ] Pediatric Allergy and Respiratory Unit Third Department of Pediatrics “Attikon” University Hospital National and Kapodistrian University of Athens School of Medicine Athens Greece
                Author notes
                [*] [* ] Correspondence

                Konstantinos Douros, Third Department of Pediatrics, “Attikon” University Hospital, 1 Rimini Street, Athens 12464, Greece.

                Email: costasdouros@ 123456gmail.com

                Article
                Publisher ID: PED412270
                DOI: 10.1002/ped4.12270
                PMC ID: 8212758
                SO-VID: 579b11e8-efa9-4464-8b97-43580cf822c6
                Copyright © © 2021 Chinese Medical Association. Pediatric Investigation published by John Wiley & Sons Australia, Ltd on behalf of Futang Research Center of Pediatric Development.
                License:

                This is an open access article under the terms of the http://creativecommons.org/licenses/by-nc-nd/4.0/ License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non‐commercial and no modifications or adaptations are made.

                History
                Date received : 25 November 2020
                Date accepted : 28 March 2021
                Page count
                Figures: 2, Tables: 1, Pages: 4, Words: 3237
                Categories
                Subject: Original Article
                Subject: Original Article
                Custom metadata
                source-schema-version-number 2.0
                cover-date June 2021
                details-of-publishers-convertor Converter:WILEY_ML3GV2_TO_JATSPMC version:6.0.2 mode:remove_FC converted:18.06.2021

                Keywords: cystic fibrosis,vitamin a,retinol,lung function
                Data availability:
                Keywords: cystic fibrosis, vitamin a, retinol, lung function

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