Pseudomonas aeruginosa Lung function Risk factors Genetic modifiers Children Cystic fibrosis 1. Introduction People with cystic fibrosis (pwCF) experience a decrease in lung function over the course of the disease, which is worsened by pulmonary exacerbations [1]. End-stage lung disease is caused by...
Albert Iron9, Herve´ Mittre10, Delphine Feldmann11, Caroline Guittard3, Mireille Claustres1,2,3 and Marie des Georges3 Among the 1700 mutations reported in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, a missense mutation, p.Ser1235Arg, is a relatively frequent finding....
Liver disease, which included hepatomegaly, liver cysts, Caroli disease, congenital hepatic fibrosis, and signs of portal hypertension was assessed on abdominal US and, if indicated, on MRI scans. In selected patients with high suspicion who did not meet the above-mentioned clinical criteria, the...
Although this is astounding progress, these cystic fibrosis transmembrane conductance regulator (CFTR) modulators are expensive and require lifetime treatment. A one-time treatment administered early in life for people with CF might prevent the onset of lung disease. Figure 1 outlines the six classes...
The Phosphoinositide 3-Kinase (PI3K) Pathway Is Involved in the Dexa-Stimulated Cystic Fibrosis Transmembrane Conductance Regulator Activity To determine the signaling pathway of dexa action we used different kinase inhibitors. First, dexa increased CFTR activity in Ussing chamber measurements, as ...