Digestive outcomes in cystic fibrosis

D Ley, D Turck - Best Practice & Research Clinical Gastroenterology, 2022 - Elsevier
Cystic fibrosis (CF) is the most frequent life-limiting autosomal recessive disease in
Caucasians, affecting the respiratory tract, but also the pancreas, gut, and hepatobiliary tract …

Phase 3 Open-Label Clinical Trial of Elexacaftor/Tezacaftor/Ivacaftor in Children Aged 2–5 Years with Cystic Fibrosis and at Least One F508del Allele

JL Goralski, JE Hoppe, MA Mall… - American journal of …, 2023 - atsjournals.org
Rationale: Elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) has been shown to be safe and
effective in people with cystic fibrosis (CF) aged⩾ 6 years with at least one F508del-CFTR …

[HTML][HTML] Disparities in first evaluation of infants with cystic fibrosis since implementation of newborn screening

SA McColley, SL Martiniano, CL Ren, MK Sontag… - Journal of cystic …, 2023 - Elsevier
Objective We evaluated whether implementation of cystic fibrosis (CF) newborn screening
(NBS) leads to equitable timeliness of initial evaluation. We compared age at first event …

[HTML][HTML] Short-Term Effects of Elexacaftor/Tezacaftor/Ivacaftor Combination on Glucose Tolerance in Young People with Cystic Fibrosis—An Observational Pilot Study

I Korten, E Kieninger, L Krueger, M Bullo… - Frontiers in …, 2022 - frontiersin.org
Background The effect of elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) on glucose
tolerance and/or cystic-fibrosis-related diabetes (CFRD) is not well understood. We …

Neutrophil lymphocyte ratio, mean platelet volume, and immunoreactive trypsinogen as early inflammatory biomarkers for cystic fibrosis in infancy: A retrospective …

T Şişmanlar Eyuboglu, AT Aslan… - Pediatric …, 2023 - Wiley Online Library
Background Airway inflammation starts in early life in cystic fibrosis (CF) and limited,
objective markers are available to help identify infants with increased inflammation. We …

[HTML][HTML] Diversity of CFTR variants across ancestries characterized using 454,727 UK biobank whole exome sequences

JE Ideozu, M Liu, BM Riley-Gillis, SR Paladugu… - Genome Medicine, 2024 - Springer
Background Limited understanding of the diversity of variants in the cystic fibrosis
transmembrane conductance regulator (CFTR) gene across ancestries hampers efforts to …

[HTML][HTML] Case report: Cystic fibrosis with kwashiorkor: A rare presentation in the era of universal newborn screening

AG Wolfe, SP Gilley, SW Waldrop, C Olson… - Frontiers in …, 2023 - frontiersin.org
Background Universal newborn screening changed the way medical providers think about
the presentation of cystic fibrosis (CF). Before implementation of universal screening, it was …

Surgical and medical management of chronic rhinosinusitis in pediatric cystic fibrosis patients: Impact on olfactory symptoms

HD Shan, BJ Vilarello, PT Jacobson, JP Tervo… - International Journal of …, 2024 - Elsevier
Background and purpose Olfactory dysfunction (OD) commonly occurs in patients with
sinonasal dysfunction, but the prevalence and severity of olfactory issues in adolescents …

[HTML][HTML] The Relation between Vitamin D Level and Lung Clearance Index in Cystic Fibrosis—A Pilot Study

M Dediu, IM Ciuca, LL Pop, D Iacob - Children, 2022 - mdpi.com
Background: Life expectancy has increased in cystic fibrosis (CF) patients; however, the rate
of mortality is still high, and in a majority of cases, the cause of death is due to respiratory …

[HTML][HTML] Impact of age at ivacaftor initiation on pulmonary outcomes among people with cystic fibrosis

CA Merlo, LJ McGarry, T Thorat, C Nguyen… - Thorax, 2024 - thorax.bmj.com
Background Ivacaftor (IVA) improves lung function and other extrapulmonary outcomes in
people with cystic fibrosis (CF). However, the effect of initiating IVA at earlier versus later …