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Joseph M. Pilewski

Researcher at University of Pittsburgh

Publications -  322
Citations -  17263

Joseph M. Pilewski is an academic researcher from University of Pittsburgh. The author has contributed to research in topics: Lung transplantation & Cystic fibrosis. The author has an hindex of 65, co-authored 285 publications receiving 15142 citations. Previous affiliations of Joseph M. Pilewski include Boston Children's Hospital & University of Pennsylvania.

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Long-Term Ivacaftor in People Aged 6 Years and Older with Cystic Fibrosis with Ivacaftor-Responsive Mutations

TL;DR: Ivacaftor was generally safe and well tolerated with no new safety concerns for up to 104 weeks in pwCF with ivACaftor-responsive mutations, and the pattern of improvement across efficacy endpoints was durable and generally consistent with parent-study outcomes.
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Insulin-like growth factor binding protein-4 exerts antifibrotic activity by reducing levels of connective tissue growth factor and the C-X-C chemokine receptor 4

TL;DR: Examining the effects of IGFBP‐4 on ECM production and fibrosis using cell‐based, ex vivo organ culture and in vivo mouse lung fibrosis models found reduced IGFBP-4 levels in SSc lung fibroblasts may contribute to the fibrotic phenotype via loss of IGF BP‐4 antifibrotic activity.
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The status of gene therapy for cystic fibrosis.

TL;DR: Immune responses to recombinant viruses and inflammatory effects of bacterial DNA are only partially understood and appear to limit efficacy, particularly with repeated administration, and recent studies with novel DNA delivery methods appear promising.
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A Prospective Observational Study of Hypogammaglobulinemia in the First Year After Lung Transplantation

TL;DR: It is demonstrated that LT recipients with severe HGG are at increased risk for recurrent pneumonias and more antibiotic courses and the combination of age, severe H GG, and number of acute steroid courses were most predictive of total days of pneumonia.