Activated L-Arginine/Nitric Oxide Pathway in Pediatric Cystic Fibrosis and Its Association with Pancreatic Insufficiency, Liver Involvement and Nourishment: An Overview and New Results

Clicks: 360
ID: 119534
2020
Free PDF
Article Quality & Performance Metrics
Overall Quality
Not rated
Combines reader engagement with the AI quality analysis. This article has not been analysed, so there is no overall score — reader engagement is measured and shown alongside.
AI Quality Assessment
Not analyzed
Readership in this journal
Emerging

Ranked #48 of 253 articles by views in journal of clinical medicine

Most read Least read

Bar heights use a square-root scale. Only the 120 most-read articles are drawn; the journal has 253 in total.

Mint this article as an NFT
Not yet minted

Create a permanent, verifiable on-chain record of this article on the Scimatic Network. The NFT is held in your Journament account, and you can withdraw it to your own wallet at any time.

5 SUSD one-off · no wallet required
Sign in to mint
Abstract
Cystic fibrosis (CF; OMIM 219700) is a rare genetic disorder caused by a chloride channel defect, resulting in lung disease, pancreas insufficiency and liver impairment. Altered L-arginine (Arg)/nitric oxide (NO) metabolism has been observed in CF patients’ lungs and in connection with malnutrition. The aim of the present study was to investigate markers of the Arg/NO pathway in the plasma and urine of CF patients and to identify possible risk factors, especially associated with malnutrition. We measured the major NO metabolites nitrite and nitrate, Arg, a semi-essential amino acid and NO precursor, the NO synthesis inhibitor asymmetric dimethylarginine (ADMA) and its major urinary metabolite dimethylamine (DMA) in plasma and urine samples of 70 pediatric CF patients and 78 age-matched healthy controls. Biomarkers were determined by gas chromatography–mass spectrometry and high-performance liquid chromatography. We observed higher plasma Arg (90.3 vs. 75.6 µM, p < 0.0001), ADMA (0.62 vs. 0.57 µM, p = 0.03), Arg/ADMA ratio (148 vs. 135, p = 0.01), nitrite (2.07 vs. 1.95 µM, p = 0.03) and nitrate (43.3 vs. 33.1 µM, p < 0.001) concentrations, as well as higher urinary DMA (57.9 vs. 40.7 µM/mM creatinine, p < 0.001) and nitrate (159 vs. 115 µM/mM creatinine, p = 0.001) excretion rates in the CF patients compared to healthy controls. CF patients with pancreatic sufficiency showed plasma concentrations of the biomarkers comparable to those of healthy controls. Malnourished CF patients had lower Arg/ADMA ratios (p = 0.02), indicating a higher NO synthesis capacity in sufficiently nourished CF patients. We conclude that NO production, protein-arginine dimethylation, and ADMA metabolism is increased in pediatric CF patients. Pancreas and liver function influence Arg/NO metabolism. Good nutritional status is associated with higher NO synthesis capacity and lower protein-arginine dimethylation.
Reference Key
brinkmann2020journalactivated Use this key to autocite in the manuscript while using SciMatic Manuscript Manager or Thesis Manager
Authors Folke Brinkmann;Beatrice Hanusch;Manfred Ballmann;Sebene Mayorandan;Alexander Bollenbach;Kristine Chobanyan-Jürgens;Kathrin Jansen;Anjona Schmidt-Choudhury;Nico Derichs;Dimitrios Tsikas;Thomas Lücke;Brinkmann, Folke;Hanusch, Beatrice;Ballmann, Manfred;Mayorandan, Sebene;Bollenbach, Alexander;Chobanyan-Jürgens, Kristine;Jansen, Kathrin;Schmidt-Choudhury, Anjona;Derichs, Nico;Tsikas, Dimitrios;Lücke, Thomas;
Journal journal of clinical medicine
Year 2020
DOI
10.3390/jcm9062012
URL
https://www.mdpi.com/2077-0383/9/6/2012
Keywords
malnutrition nitric oxide cystic fibrosis nutritional status pancreatic disease liver disease l-arginine nitric oxide synthases

Citations

No citations found. To add a citation, contact the admin at info@scimatic.org

Comments

No comments yet. Be the first to comment on this article.