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3
Published in 2022 at "Journal of pediatric gastroenterology and nutrition"
DOI: 10.1097/mpg.0000000000003459
Abstract: BACKGROUND Alteration of the airway microbiota is a hallmark of cystic fibrosis (CF) pulmonary disease. Dysfunction of cystic fibrosis transmembrane regulator (CFTR) in the intestine also promotes changes in local microbiota such as small intestinal…
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Keywords:
cystic fibrosis;
one month;
luma iva;
lumacaftor ivacaftor ... See more keywords
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2
Published in 2023 at "BMJ Open Respiratory Research"
DOI: 10.1136/bmjresp-2022-001590
Abstract: Background Previous studies showed that the combination of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) corrector and potentiator, lumacaftor–ivacaftor (LUMA–IVA) provides meaningful clinical benefits in patients with cystic fibrosis who are homozygous for the Phe508del CFTR…
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Keywords:
luma iva;
patients cystic;
iva;
study ... See more keywords
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1
Published in 2022 at "Journal of Personalized Medicine"
DOI: 10.3390/jpm12020252
Abstract: Lumacaftor/ivacaftor (LUMA-IVA) therapy is prescribed to people with cystic fibrosis (pwCF) homozygous for the Phe508del-CFTR variant to restore CFTR protein function. There is, however, large inter-individual variability in treatment response. Here, we seek to identify…
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Keywords:
luma iva;
people cystic;
function;
lumacaftor ivacaftor ... See more keywords