March 2018

Editorial Board
Published in issue: March 2018
ii

Editorials

In statistics we trust: Towards the careful derivation and interpretation of meaningful survival estimates in cystic fibrosis
• N. Mayer-Hamblett,
• D. Polineni,
• S.L. Heltshe
Published online: February 03, 2018
p133-134
A comorbidity of CF in need of our attention and activity: Attention Deficit Hyperactivity Disorder!
• Jurgen Lemiere,
• Trudy Havermans
Published online: February 14, 2018
p135-136
New directions on lung clearance index variability and feasibility
• Katherine O’Neill,
• Clare Saunders
Published in issue: March 2018
p137-139

News Article

News
Published online: February 04, 2018
p140-142

Review

Mechanisms of humoral immune response against Pseudomonas aeruginosa biofilm infection in cystic fibrosis
• Renan Marrichi Mauch,
• Peter Østrup Jensen,
• Claus Moser,
• Carlos Emilio Levy,
• Niels Høiby
Published online: October 21, 2017
p143-152

Standards of Care

ECFS best practice guidelines: the 2018 revision
• Carlo Castellani,
• Alistair J.A. Duff,
• Scott C. Bell,
• ...
• Thomas O.F. Wagner,
• Susan P. Wolfe,
• Pavel Drevinek
Published online: March 03, 2018
p153-178

CFTR/Basic Science

Ivacaftor restores CFTR-dependent sweat gland fluid secretion in cystic fibrosis subjects with S945L alleles
• Jeeyeon Kim,
• Zoe Davies,
• Colleen Dunn,
• Jeffrey J. Wine,
• Carlos Milla
Published online: December 23, 2017
p179-185

Diagnosis/Screening

Ratiometric sweat secretion optical test in cystic fibrosis, carriers and healthy subjects
• Gabriella Bergamini,
• Gloria Tridello,
• Elisa Calcaterra,
• ...
• Baroukh Maurice Assael,
• Claudio Sorio,
• Paola Melotti
Published online: December 29, 2017
p186-189

Immunology

FAM13A is a modifier gene of cystic fibrosis lung phenotype regulating rhoa activity, actin cytoskeleton dynamics and epithelial-mesenchymal transition
• Harriet Corvol,
• Nathalie Rousselet,
• Kristin E. Thompson,
• ...
• Craig A. Hodges,
• Pierre-Yves Boëlle,
• Loic Guillot
Published online: November 24, 2017
p190-203

Microbiology

16S rRNA gene sequencing reveals site-specific signatures of the upper and lower airways of cystic fibrosis patients
• Sarah K. Lucas,
• Robert Yang,
• Jordan M. Dunitz,
• Holly C. Boyer,
• Ryan C. Hunter
Published online: August 18, 2017
p204-212

Epidemiology/Models of Care

A guide to interpreting estimated median age of survival in cystic fibrosis patient registry reports
• Ruth H. Keogh,
• Sanja Stanojevic
Published online: February 02, 2018
p213-217

Up-to-date and projected estimates of survival for people with cystic fibrosis using baseline characteristics: A longitudinal study using UK patient registry data
• Ruth H. Keogh,
• Rhonda Szczesniak,
• David Taylor-Robinson,
• Diana Bilton
Published online: January 06, 2018
p218-227

Clinical Trials

Lumacaftor/ivacaftor in patients with cystic fibrosis and advanced lung disease homozygous for F508del-CFTR
• Jennifer L. Taylor-Cousar,
• Manu Jain,
• Tara Lynn Barto,
• ...
• David Waltz,
• Joseph Pilewski
• on behalf of the VX14-809-106 Investigator Group
Published online: November 08, 2017
p228-235

Pulmonology

Variability of lung clearance index in clinically stable cystic fibrosis lung disease in school age children
• Marcus Svedberg,
• Per M. Gustafsson,
• Paul D. Robinson,
• Monica Rosberg,
• Anders Lindblad
Published online: August 16, 2017
p236-241

Variability of monthly nitrogen multiple-breath washout during one year in children with cystic fibrosis
• Kent Green,
• Thomas Kongstad,
• Marianne Skov,
• ...
• Per Gustafsson,
• Tacjana Pressler,
• Kim Gjerum Nielsen
Published online: December 19, 2017
p242-248

Three-center feasibility of lung clearance index in infants and preschool children with cystic fibrosis and other lung diseases
• Mirjam Stahl,
• Simon Y. Graeber,
• Cornelia Joachim,
• ...
• Matthias V. Kopp,
• Lutz Naehrlich,
• Marcus A. Mall
Published online: August 12, 2017
p249-255

Exercise

Blood flow regulation and oxidative stress during submaximal cycling exercise in patients with cystic fibrosis
• Matthew A. Tucker,
• Breana Berry,
• Nichole Seigler,
• ...
• Dabney Eidson,
• Kathleen T. McKie,
• Ryan A. Harris
Published online: September 08, 2017
p256-263

Gastroenterology/Hepatology

The effect of enteral tube feeding in cystic fibrosis: A registry based study
• Denis Libeert,
• Dimitri Declercq,
• Simeon Wanyama,
• ...
• Sabine Van daele,
• Frans De Baets,
• Stephanie Van Biervliet
Published online: January 21, 2018
p264-270

Endocrinology

Effect of Lumacaftor/Ivacaftor on glucose metabolism and insulin secretion in Phe508del homozygous cystic fibrosis patients
• Jan C. Thomassen,
• Matthias I. Mueller,
• Miguel A. Alejandre Alcazar,
• Ernst Rietschel,
• Silke van Koningsbruggen-Rietschel
Published online: December 15, 2017
p271-275

Psychosocial

Screening for ADHD in adults with cystic fibrosis: Prevalence, health-related quality of life, and adherence
• Anna M. Georgiopoulos,
• Deborah Friedman,
• Elizabeth A. Porter,
• ...
• Breanna K. Glaeser,
• Siena C. Napoleon,
• Janet Wozniak
Published online: September 01, 2017
p276-280
Attention deficit hyperactivity disorder symptoms in patients with cystic fibrosis
• Malena Cohen-Cymberknoh,
• Tzlil Tanny,
• Oded Breuer,
• ...
• David Shoseyov,
• Eitan Kerem,
• Itai Berger
Published online: December 18, 2017
p281-285

Online Only Publications

Ivacaftor withdrawal syndrome in cystic fibrosis patients with the G551D mutation
• Aaron T. Trimble,
• Scott H. Donaldson
Published online: October 24, 2017
e13-e16


Rare cases of Blastobotrys raffinosifermentans as cause of FEV1 decline in two CF patients – Whole genome sequencing to exclude transmission
• K. Jahn,
• V. Baettig,
• H.M.B. Seth-Smith,
• A. Egli,
• M. Tamm
Published online: December 03, 2017
e17-e19

Exploring probiotic use in a regional cystic fibrosis consortium
• K.D. Gonzalez,
• J.B. Zuckerman,
• E.H. Sears,
• ...
• T. Lever,
• M.M. Griffin,
• L.W. Leclair
Published online: August 16, 2017
e20-e21

Colistin resistance in Pseudomonas aeruginosa and Achromobacter spp. cultured from Danish cystic fibrosis patients is not related to plasmid-mediated expression of mcr-1
• Maya G. Pedersen,
• Hanne V. Olesen,
• Søren Jensen-Fangel,
• Niels Nørskov-Lauritsen,
• Mikala Wang
Published online: December 15, 2017
e22-e23