Albumin Metabolism in Cystic Fibrosis

PEDIATRICS ◽  
1969 ◽  
Vol 44 (2) ◽  
pp. 305-306
Author(s):  
A. Myron Johnson
Keyword(s):  
Cystic Fibrosis ◽  
Inflammatory Response ◽  
Blood Volume ◽  
Acute Inflammatory Response ◽  
Albumin Synthesis ◽  
Compensatory Response ◽  
Normal Albumin

It appears that Dr. Strober and I disagree on the implications of the word "cause" (Pediatrics, 44:144, 1969). The paper in question did not even consider the possibility that some other factor(s) could result in increased blood volume and decreased albumin synthesis concurrently. One possible third factor is the acute inflammatory response. If such be the case, even normal albumin synthesis may represent a compensatory response to the hypervolemia. The arguments in Dr. Strober's reply are based, in part, on the speculation that the liver is incapable of producing albumin at a rate much above the normal, resting rate.

scholarly journals The isoprenoid end product N6-isopentenyladenosine reduces inflammatory response through the inhibition of the NFκB and STAT3 pathways in cystic fibrosis cells

2017 ◽  
Vol 67 (4) ◽  
pp. 315-326 ◽  
Author(s):  
Antonietta Santoro ◽  
Elena Ciaglia ◽  
Vanessa Nicolin ◽  
Alessandra Pescatore ◽  
Lucia Prota ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Inflammatory Response

scholarly journals Vx-809/Vx-770 treatment reduces inflammatory response to Pseudomonas aeruginosa in primary differentiated cystic fibrosis bronchial epithelial cells

2018 ◽  
Vol 314 (4) ◽  
pp. L635-L641 ◽  
Author(s):  
Manon Ruffin ◽  
Lucie Roussel ◽  
Émilie Maillé ◽  
Simon Rousseau ◽  
Emmanuelle Brochiero
Keyword(s):  
Cystic Fibrosis ◽  
Pseudomonas Aeruginosa ◽  
Inflammatory Response ◽  
Respiratory Infections ◽  
Bronchial Epithelial Cell ◽  
Transmembrane Conductance Regulator ◽  
Transmembrane Conductance ◽  
Bronchial Epithelial ◽  
Primary Bronchial Epithelial Cell ◽  
Beneficial Impact

Cystic fibrosis patients exhibit chronic Pseudomonas aeruginosa respiratory infections and sustained proinflammatory state favoring lung tissue damage and remodeling, ultimately leading to respiratory failure. Loss of cystic fibrosis transmembrane conductance regulator (CFTR) function is associated with MAPK hyperactivation and increased cytokines expression, such as interleukin-8 [chemoattractant chemokine (C-X-C motif) ligand 8 (CXCL8)]. Recently, new therapeutic strategies directly targeting the basic CFTR defect have been developed, and ORKAMBI (Vx-809/Vx-770 combination) is the only Food and Drug Administration-approved treatment for CF patients homozygous for the F508del mutation. Here we aimed to determine the effect of the Vx-809/Vx-770 combination on the induction of the inflammatory response by fully differentiated primary bronchial epithelial cell cultures from CF patients carrying F508del mutations, following exposure to P. aeruginosa exoproducts. Our data unveiled that CFTR functional rescue with Vx-809/Vx-770 drastically reduces CXCL8 (as well as CXCL1 and CXCL2) transcripts and p38 MAPK phosphorylation in response to P. aeruginosa exposure through a CFTR-dependent mechanism. These results suggest that ORKAMBI has anti-inflammatory properties that could decrease lung inflammation and contribute to the observed beneficial impact of this treatment in CF patients.

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