Inhaled dry powder alginate oligosaccharide in cystic fibrosis: a randomised, double-blind, placebo-controlled, crossover phase 2b study

Autor: Silke van Koningsbruggen-Rietschel, Jane C. Davies, Tacjana Pressler, Rainald Fischer, Gordon MacGregor, Scott H. Donaldson, Knut Smerud, Nils Meland, Jann Mortensen, Marie Ø. Fosbøl, Damian G. Downey, Astrid H. Myrset, Hugo Flaten, Philip D. Rye, The following investigators and their teams (in alphabetical order) conducted this study., Mary Carroll, Jane Davies, Carsten Schwarz, Nico Derichs, Damian Downey, Olaf Eickmeier, Pal Leyell Finstad, Marie Øbro Fossbøl, Marita Gilljam, Lena Hjelte, Alan Knox, Susanne Nährig, Joachim Riethmüller, Felix C. Ringshausen, AS AlgiPharma, Laura Gow, Joy Conway, William Bennett

Publikováno v: ERJ Open Research, Vol 6, Iss 4 (2020)

Background OligoG is a low molecular-weight alginate oligosaccharide that improves the viscoelastic properties of cystic fibrosis (CF) mucus and disrupts biofilms, thereby potentiating the activity of antimicrobial agents. The efficacy of inhaled Oli

Externí odkaz: https://doaj.org/article/bf6a51f4c4a3405ebe8677a3a47a674d

Riociguat for the treatment of Phe508del homozygous adults with cystic fibrosis

Autor: Jennifer L. Taylor-Cousar, Andreas Kaiser, François Vermeulen, Peter Sandner, Clare Saunders, Nico Derichs, Isabelle Fajac, Renee Jensen, Jane C. Davies, Steven M. Rowe, Anja Hoffmann, Damian G. Downey, Christine E. Bear, Karoline Droebner, Soundos Saleh, George M. Solomon, Felix Ratjen, Daniel B. Rosenbluth, Anne Malfroot, Elizabeth Tullis, Stefan Willmann, Dilip Nazareth

Publikováno v: Rio-CF Study Group, Derichs, N, Taylor-Cousar, J L, Davies, J C, Fajac, I, Tullis, E, Nazareth, D, Downey, D G, Rosenbluth, D, Malfroot, A, Saunders, C, Jensen, R, Solomon, G M, Vermeulen, F, Kaiser, A, Willmann, S, Saleh, S, Droebner, K, Sandner, P, Bear, C E, Hoffmann, A, Ratjen, F & Rowe, S M 2021, ' Riociguat for the treatment of Phe508del homozygous adults with cystic fibrosis ', Journal of Cystic Fibrosis, vol. 20, no. 6, pp. 1018-1025 . https://doi.org/10.1016/j.jcf.2021.07.015

BACKGROUND: Riociguat is a first-in-class soluble guanylate cyclase stimulator for which preclinical data suggested improvements in cystic fibrosis transmembrane conductance regulator (CFTR) function. METHODS: This international, multicenter, two-par

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::a372e9b8ce7beb6e7e0a0ae4c60ecd2f
https://pure.qub.ac.uk/en/publications/0aba0b21-9b6d-4143-a70d-193cd26ffef8

Local and Systemic Alterations of the L-Arginine/Nitric Oxide Pathway in Sputum, Blood, and Urine of Pediatric Cystic Fibrosis Patients and Effects of Antibiotic Treatment

Autor: Folke Brinkmann, Kathrin Jansen, Alexander Bollenbach, Anjona Schmidt-Choudhury, Dimitrios Tsikas, Kristine Chobanyan-Jürgens, Anna Wiemers, Beatrice Hanusch, Sebene Mayorandan, Manfred Ballmann, Thomas Lücke, Nico Derichs

Publikováno v: Journal of Clinical Medicine, Vol 9, Iss 3802, p 3802 (2020)
Journal of Clinical Medicine
Volume 9
Issue 12

Alterations in the L-arginine (Arg)/nitric oxide (NO) pathway have been reported in cystic fibrosis (CF
OMIM 219700) as the result of various factors including systemic and local inflammatory activity in the airways. The aim of the present study

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::9ecf0ac063fbe3b9105a1a82d864e39e
https://www.mdpi.com/2077-0383/9/12/3802

Activated L-Arginine/Nitric Oxide Pathway in Pediatric Cystic Fibrosis and Its Association with Pancreatic Insufficiency, Liver Involvement and Nourishment: An Overview and New Results

Autor: Anjona Schmidt-Choudhury, Folke Brinkmann, Manfred Ballmann, Kathrin Jansen, Alexander Bollenbach, Kristine Chobanyan-Jürgens, Nico Derichs, T. Lücke, Sebene Mayorandan, Dimitrios Tsikas, Beatrice Hanusch

Publikováno v: Journal of Clinical Medicine
Journal of Clinical Medicine, Vol 9, Iss 2012, p 2012 (2020)
Volume 9
Issue 6

Cystic fibrosis (CF
OMIM 219700) is a rare genetic disorder caused by a chloride channel defect, resulting in lung disease, pancreas insufficiency and liver impairment. Altered L-arginine (Arg)/nitric oxide (NO) metabolism has been observed in C

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::25716c9fac6462082f79f991c8ad1f9b
https://pubmed.ncbi.nlm.nih.gov/32604946

Cystic Fibrosis Diagnostic Challenges over 4 Decades: Historical Perspectives and Lessons Learned

Autor: Terry B. White, Nico Derichs, Beryl J. Rosenstein, Philip M. Farrell, Carlo Castellani

Objective Because cystic fibrosis (CF) can be difficult to diagnose, and because information about the genetic complexities and pathologic basis of the disease has grown so rapidly over the decades, several consensus conferences have been held by the

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::a6df23dc573248db86c52be7f3e5d37b
http://hdl.handle.net/11562/981404