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    relacionado con: journal cystic fibrosis
  2. Learn how to maintain your emotional well-being throughout your cystic fibrosis journey. Learn techniques such as breathing patterns to cope on the spot when you feel overwhelmed.

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  1. Hace 4 días · The cystic fibrosis (CF) conductance transmembrane regulator (CFTR) gene was identified in 1989 and this finding led to the discovery of the CFTR protein [1-3], which improved our knowledge of the pathogenesis of CF and paved the way for the development of CFTR modulators [2,4]. CFTR modulators have demonstrated substantial benefits including improved lung function and reduction in pulmonary ...

  2. Hace 1 día · Cystic fibrosis (CF) is a genetic disorder resulting in the secretion of highly viscous mucus in the airways, leading to lung infection, respiratory failure, morbidity and mortality. CF is attributed to mutation in the CF Transmembrane Conductance Regulator (CFTR) gene that codes for a chloride transporting channel at the cell plasma membrane. More than 2,000 mutations in the CF gene have been ...

  3. Hace 1 día · Cystic fibrosis transmembrane conductance regulator (CFTR) modulator drugs target the underlying defect and improve CFTR function. They are a part of standard care in many countries, but not all patients are eligible for these drugs due to age and genotype.

  4. Hace 7 horas · People with cystic fibrosis require intensive daily physiotherapy to clear the lungs and airways. Picture: Getty Images As there is currently no cure for CF, people with the condition require intensive daily physiotherapy to clear the lungs and airways, countless medications and frequent hospitalisations.

  5. Hace 1 día · This is reflected in the dramatic increase in the number of pregnancies to people with CF in the United States and worldwide (Cystic Fibrosis Foundation, 2023). However, for pregnant women and breastfeeding mothers, ceasing ETI therapy may cause sudden and serious decline in lung conditions and risk their own health as well as the health of their babies (Trimble & Donaldson, 2018 ).

  6. Hace 1 día · Thus the effect of maternally administered ETI on foetal genetic and structural development was investigated. Experimental approach: Pregnant Sprague Dawley rats were orally treated with ETI (6.7 mg·kg -1 ·day -1 elexacaftor + 3.5 mg·kg -1 ·day -1 tezacaftor + 25 mg·kg -1 ·day -1 ivacaftor) for 7 days from E12 to E19.

  7. Hace 4 días · Background Bronchiectasis is a condition characterized by abnormal and irreversible bronchial dilation resulting from lung tissue damage and can be categorized into two main groups: cystic fibrosis (CF) and non-CF bronchiectasis (NCFB). Both diseases are marked by recurrent infections, inflammatory exacerbations, and lung damage. Given that infections are the primary drivers of disease ...