Safety, pharmacokinetics, and pharmacodynamics of ivacaftor in patients aged 2–5 years with cystic fibrosis and a CFTR gating mutation (KIWI): an open-label, single …

JC Davies, S Cunningham, WT Harris… - The Lancet …, 2016 - thelancet.com
Background Ivacaftor has been shown to be a safe, effective treatment for cystic fibrosis in
patients aged 6 years or older with a CFTR gating mutation. We aimed to assess the safety, …

Airway microbiota across age and disease spectrum in cystic fibrosis

…, JP Clancy, RL Gibson, WT Harris… - European …, 2017 - Eur Respiratory Soc
Our objectives were to characterise the microbiota in cystic fibrosis (CF) bronchoalveolar
lavage fluid (BALF), and determine its relationship to inflammation and disease status. BALF …

Molecular MR imaging of melanoma angiogenesis with ανβ3‐targeted paramagnetic nanoparticles

…, SD Caruthers, TD Harris, TA Williams… - … in Medicine: An …, 2005 - Wiley Online Library
Neovascularization is a critical component in the progression of malignant melanoma. The
objective of this study was to determine whether α ν β 3 ‐targeted paramagnetic …

[HTML][HTML] An open-label extension study of ivacaftor in children with CF and a CFTR gating mutation initiating treatment at age 2–5 years (KLIMB)

M Rosenfeld, S Cunningham, WT Harris, A Lapey… - Journal of Cystic …, 2019 - Elsevier
Background KIWI (NCT01705145 ) was a 24-week, single-arm, pharmacokinetics, safety, and
efficacy study of ivacaftor in children aged 2 to 5 years with cystic fibrosis (CF) and a CFTR …

[HTML][HTML] Myofibroblast differentiation and enhanced TGF-B signaling in cystic fibrosis lung disease

WT Harris, DR Kelly, Y Zhou, D Wang, M Macewen… - PloS one, 2013 - journals.plos.org
William T Harris also currently participates as site PI for several Vertex multi-center pediatric
studies. His participation with Vertex has not influenced the design, data, or publication …

MicroRNA-145 antagonism reverses TGF-β inhibition of F508del CFTR correction in airway epithelia

…, WT Gerthoffer, SM Rowe, WT Harris - American journal of …, 2018 - atsjournals.org
… Correspondence and requests for reprints should be addressed to William T. Harris, MD,
620 ACC Building, Children’s of Alabama, 1600 7th Avenue South, Birmingham, AL 35233-…

[HTML][HTML] Tgf-beta downregulation of distinct chloride channels in cystic fibrosis-affected epithelia

H Sun, WT Harris, S Kortyka, K Kotha, AJ Ostmann… - PloS one, 2014 - journals.plos.org
Rationale The cystic fibrosis transmembrane conductance regulator (CFTR) and Calcium-activated
Chloride Conductance (CaCC) each play critical roles in maintaining normal …

Transforming growth factor‐β1 in bronchoalveolar lavage fluid from children with cystic fibrosis

WT Harris, MS Muhlebach, RA Oster… - Pediatric …, 2009 - Wiley Online Library
Rationale Transforming factor β 1 (TGF‐β 1 ) genetic polymorphisms have been identified
as a modifier of cystic fibrosis (CF) lung disease severity. However, few data link TGF‐β 1 …

Plasma TGF‐β1 in pediatric cystic fibrosis: Potential biomarker of lung disease and response to therapy

WT Harris, MS Muhlebach, RA Oster… - Pediatric …, 2011 - Wiley Online Library
Introduction Transforming growth factor beta‐1 (TGF‐β 1 ) is an important genetic modifier
of lung disease severity in cystic fibrosis (CF), yet the mechanism behind this disease …

Association of lower airway inflammation with physiologic findings in young children with cystic fibrosis

SL Peterson‐Carmichael, WT Harris… - Pediatric …, 2009 - Wiley Online Library
Background The relationship between lower airway markers of inflammation and infection
with physiologic findings is poorly understood in young children with cystic fibrosis (CF). The …