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Key clinical point: Exocrine pancreatic insufficiency (EPI) is the major cause of nutrient malabsorption in cystic fibrosis (CF), with the likelihood of restoration of pancreatic function significantly higher if CF transmembrane conductance regulator (CFTR) modulator therapies are initiated as early in life as possible.
Main finding: In the ARRIVAL study, ivacaftor led to a mean decrease of 56% in immunoreactive trypsinogen in 12-24 month old patients with CFTR gating mutation. Similar improvements were observed in older children aged 2-5 years treated with ivacaftor in the KIWI and the open-label extension KLIMB study. However, the results are not as promising in older patients aged 5-61 years.
Study details: These are highlights from a review that summarizes the effects of highly effective CFTR modulators on growth and nutrition in patients with CF.
Disclosures: The manuscript did not receive any additional funding. The authors declared receiving grant support and serving as consultants for various sources.
Source: Bass R et al. Nutrients. 2021(Aug 24);13(9):2907. Doi: 10.3390/nu13092907.
Key clinical point: Exocrine pancreatic insufficiency (EPI) is the major cause of nutrient malabsorption in cystic fibrosis (CF), with the likelihood of restoration of pancreatic function significantly higher if CF transmembrane conductance regulator (CFTR) modulator therapies are initiated as early in life as possible.
Main finding: In the ARRIVAL study, ivacaftor led to a mean decrease of 56% in immunoreactive trypsinogen in 12-24 month old patients with CFTR gating mutation. Similar improvements were observed in older children aged 2-5 years treated with ivacaftor in the KIWI and the open-label extension KLIMB study. However, the results are not as promising in older patients aged 5-61 years.
Study details: These are highlights from a review that summarizes the effects of highly effective CFTR modulators on growth and nutrition in patients with CF.
Disclosures: The manuscript did not receive any additional funding. The authors declared receiving grant support and serving as consultants for various sources.
Source: Bass R et al. Nutrients. 2021(Aug 24);13(9):2907. Doi: 10.3390/nu13092907.
Key clinical point: Exocrine pancreatic insufficiency (EPI) is the major cause of nutrient malabsorption in cystic fibrosis (CF), with the likelihood of restoration of pancreatic function significantly higher if CF transmembrane conductance regulator (CFTR) modulator therapies are initiated as early in life as possible.
Main finding: In the ARRIVAL study, ivacaftor led to a mean decrease of 56% in immunoreactive trypsinogen in 12-24 month old patients with CFTR gating mutation. Similar improvements were observed in older children aged 2-5 years treated with ivacaftor in the KIWI and the open-label extension KLIMB study. However, the results are not as promising in older patients aged 5-61 years.
Study details: These are highlights from a review that summarizes the effects of highly effective CFTR modulators on growth and nutrition in patients with CF.
Disclosures: The manuscript did not receive any additional funding. The authors declared receiving grant support and serving as consultants for various sources.
Source: Bass R et al. Nutrients. 2021(Aug 24);13(9):2907. Doi: 10.3390/nu13092907.