Table of Contents page: Journal of Cystic Fibrosis

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Editorial Board

Published in issue: March 2022

ii

News article

Published in issue: March 2022

p189-190

A year in review: Real world evidence, functional monitoring and emerging therapeutics in 2021

H.L. Barr,
T. Bihouee,
A.M. Zwitserloot

Published online: March 08, 2022

p191-196

From the Editor's Desk

Patrick A. Flume

Published in issue: March 2022

p197-198

Defining and Treating Cystic Fibrosis Liver Disease: Some Things Old and Some Things New

Gary J. Galante

Published in issue: March 2022

p199-201

The impact of liver disease on mortality in cystic fibrosis–A systematic review

Ao Sasame,
Diarmuid Stokes,
Billy Bourke,
Lucy Connolly,
Emer Fitzpatrick,
Marion Rowland

Published online: August 08, 2021

p202-211

Open Access

Lumacaftor-ivacaftor effects on cystic fibrosis-related liver involvement in adolescents with homozygous F508 del-CFTR

David Drummond,
Jérémy Dana,
Laureline Berteloot,
...
Dominique Debray,
Muriel Girard,
Isabelle Sermet-Gaudelus

Published online: August 25, 2021

p212-219

Ursodeoxycholic acid and liver disease associated with cystic fibrosis: A multicenter cohort study

Carla Colombo,
Gianfranco Alicandro,
Mark Oliver,
...
Birgitta Strandvik,
Anders Lindblad
for the CF UDCA study group

Published online: April 01, 2021

p220-226

Use of elexacaftor/tezacaftor/ivacaftor in liver transplant patients with cystic fibrosis

Cameron J. McKinzie,
Christina T. Doligalski,
Steven J. Lobritto,
Raymond D. Coakley,
W. Adam Gower

Published online: August 09, 2021

p227-229

Advanced but not mild liver disease is a predictor of decreased survival in children with cystic fibrosis, with far greater impact in females: A 27-year real-life cohort study

Marilisa Baldissera,
Peter J. Lewindon,
Louise E. Ramm,
Gunter F. Hartel,
Rita Mattiello,
Grant A. Ramm

Published online: December 20, 2021

p230-235

Gamma-glutamyl transpeptidase-to-platelet ratio as a biomarker of liver disease and hepatic fibrosis severity in paediatric Cystic Fibrosis

Diego A. Calvopina,
Peter J. Lewindon,
Louise E. Ramm,
...
Gunter F. Hartel,
Daniel H Leung,
Grant A. Ramm

Published online: December 22, 2021

p236-242

Response to elexacaftor/tezacaftor/ivacaftor in intestinal organoids derived from people with cystic fibrosis

Eva Furstova,
Tereza Dousova,
Jakub Beranek,
...
Ondrej Cinek,
Milan Macek Jr,
Pavel Drevinek

Published online: August 02, 2021

p243-245

Functional Restoration of CFTR Nonsense Mutations in Intestinal Organoids

E. de Poel,
S. Spelier,
S.W.F. Suen,
...
G.H. Koppelman,
C.K. van der Ent,
J.M. Beekman

Published online: October 17, 2021

p246-253

Open Access

Forskolin induced swelling (FIS) assay in intestinal organoids to guide eligibility for compassionate use treatment in a CF patient with a rare genotype

B.L. Aalbers,
J.E. Brunsveld,
C.K. van der Ent,
J.C. van den Eijnden,
J.M. Beekman,
H.G.M. Heijerman

Published online: January 30, 2022

p254-257

Open Access

The effect of elexacaftor/tezacaftor/ivacaftor (ETI) on glycemia in adults with cystic fibrosis

Kevin J. Scully,
Peter Marchetti,
Gregory S. Sawicki,
...
Rebecca E. Cagnina,
John C. Kennedy,
Melissa S. Putman

Published online: September 13, 2021

p258-263

Restoration of exocrine pancreatic function in child with lumacaftor/ivacaftor therapy in cystic fibrosis

J. Crowley,
K. Croinin,
D. Mullane,
M. Ní Chróinín

Published online: September 09, 2021

p264

Effect of elexacaftor-tezacaftor-ivacaftor on body weight and metabolic parameters in adults with cystic fibrosis

Max C. Petersen,
Lauren Begnel,
Michael Wallendorf,
Marina Litvin

Published online: November 30, 2021

p265-271

The effectiveness of exercise interventions to increase physical activity in Cystic Fibrosis: A systematic review

M. Curran,
AC. Tierney,
B. Button,
...
C. McDonnell,
B. Casserly,
R. Cahalan

Published online: November 06, 2021

p272-281

Exercise intolerance in cystic fibrosis-the role of CFTR modulator therapies

Jessica E. Caterini,
Felix Ratjen,
Alan R. Barker,
...
Kate Rendall,
Jane E Schneiderman,
Greg D. Wells

Published online: December 23, 2021

p282-292

A new path for CF clinical trials through the use of historical controls

Amalia S. Magaret,
Mark Warden,
Noah Simon,
...
George Z. Retsch-Bogart,
Bonnie W. Ramsey,
Nicole Mayer-Hamblett

Published online: December 05, 2021

p293-299

Working in partnership with the patient community to develop outline trial designs in CF

NJ Rowbotham,
S Smith,
G Davies,
...
Z Elliott,
T Daniels,
AR Smyth

Published online: December 16, 2021

p300-301

Incidence and risk factors of cancer in individuals with cystic fibrosis in the UK; a case-control study

Olga Archangelidi,
Paul Cullinan,
Nicholas J. Simmonds,
...
Daniel Peckham,
Diana Bilton,
Siobhán B. Carr

Published online: August 02, 2021

p302-308

Barriers implementing infection prevention and control experienced by healthcare workers, people with CF and parents

Lisa Saiman,
Juyan J. Zhou,
Kushal S. Shah,
...
Michael R. Kosorok,
Marianne S. Muhlebach
CF IP&C Study Group

Published online: September 11, 2021

p309-315

Treatment patterns in people with cystic fibrosis: have they changed since the introduction of ivacaftor?

Emily Granger,
Gwyneth Davies,
Ruth H. Keogh

Published online: September 06, 2021

p316-322

Open Access

Using a learning health system to understand the mismatch between medicines supply and actual medicines use among adults with cystic fibrosis

Amanda Bevan,
Zhe Hui Hoo,
Nikki Totton,
...
Frank P Edenborough,
Rachael Curley,
Martin J. Wildman

Published online: September 23, 2021

p323-331

Pilot RCT of a telehealth intervention to reduce symptoms of depression and anxiety in adults with cystic fibrosis

Christina J. Bathgate,
Kristin M. Kilbourn,
Nora H. Murphy,
Frederick S. Wamboldt,
Kristen E. Holm

Published online: August 05, 2021

p332-338

Mental status changes during elexacaftor/tezacaftor / ivacaftor therapy

Suyeon Heo,
David C. Young,
Julie Safirstein,
...
Martine H. Antell,
Stefanie Diloreto,
Shannon M. Rotolo

Published online: November 03, 2021

p339-343

A new perspective on opportunistic pathogens of the genus Bordetella in cystic fibrosis

Quentin Menetrey,
Fabien Aujoulat,
Raphaël Chiron,
Estelle Jumas-Bilak,
Hélène Marchandin,
Chloé Dupont

Published online: August 10, 2021

p344-347

Administration and monitoring of clofazimine for NTM infections in children with and without cystic fibrosis

LH Cameron,
CA Peloquin,
P Hiatt,
...
JC McNeil,
A Patel,
F Ruiz

Published online: August 31, 2021

p348-352

Serological biomarkers for the diagnosis of Mycobacterium abscessus infections in cystic fibrosis patients

Vincent Le Moigne,
Anne-Laure Roux,
Hélène Mahoudo,
...
Stéphane Canaan,
Geneviève Héry-Arnaud,
Jean-Louis Herrmann

Published online: September 09, 2021

p353-360

The impact of cystic fibrosis on the working life of patients: A systematic review

Veruscka Leso,
Rosaria Romano,
Carolina Santocono,
...
Paola Iacotucci,
Vincenzo Carnovale,
Ivo Iavicoli

Published online: August 29, 2021

p361-369

Macrophages from gut-corrected CF mice express human CFTR and lack a pro-inflammatory phenotype

Jonathan L Gillan,
Gareth R Hardisty,
Donald J Davidson,
Robert D Gray

Published online: November 16, 2021

p370-374

Open Access

Clinical presentation and basic defect of the CFTR genotype p.Phe508del / p.Arg117His in a mother and her monozygous twin daughters

Christiane Lex,
Rebecca Minso,
Nadine Alfeis,
Hendrik Rosewich,
Sylvia Schucht,
Burkhard Tümmler

Published online: September 03, 2021

p375-377

Actigraphic and patient and family reported sleep outcomes in children and youth with cystic fibrosis: A systematic review

Jordana McMurray,
Kimberley Widger,
Anne L. Stephenson,
Robyn Stremler

Published online: May 23, 2021

e49-e82

Rethinking physical exercise training in the modern era of cystic fibrosis: A step towards optimising short-term efficacy and long-term engagement

Mathieu Gruet,
Zoe L. Saynor,
Don S. Urquhart,
Thomas Radtke

Published online: September 04, 2021

e83-e98

Drug-induced liver injury from elexacaftor/ivacaftor/tezacaftor

Sarah Lowry,
Peter J. Mogayzel,
Kiyoko Oshima,
Wikrom Karnsakul

Published online: July 16, 2021

e99-e101

Aquagenic wrinkling of the palms in cystic fibrosis patients treated with ivacaftor

Eyal Jacobi,
Melinda Solomon,
Julie Avolio,
...
Tanja Gonska,
Felix Ratjen,
Hartmut Grasemann

Published in issue: March 2022

e102-e105

Management of Cystic Fibrosis during COVID-19: Patient Reported Outcomes based remote follow-up among CF patients in Denmark – A feasibility study

Lotte Rodkjær,
Majbritt Jeppesen,
Liv Schougaard

Published online: November 13, 2021

e106-e112

Open Access

SARS-CoV-2 antibodies among people with cystic fibrosis prior to the vaccination campaign: A seroprevalence study in two specialized centres in Northern Italy

Gianfranco Alicandro,
Valeria Daccó,
Lisa Cariani,
...
Andrea Gramegna,
Francesco Blasi,
Carla Colombo

Published online: December 12, 2021

e113-e116

TLR5 signalling is hyper-responsive in porcine cystic fibrosis airways epithelium

Isabelle Fleurot,
Raquel López-Gálvez,
Pascal Barbry,
...
Nikolai Klymiuk,
Jean-Claude Sirard,
Ignacio Caballero

Published online: August 19, 2021

e117-e121

Open Access

Arm muscle area for the longitudinal assessment of nutritional status in paediatric patients with cystic fibrosis - A single centre experience

Helmut Ellemunter,
Markus Dumke,
Gratiana Steinkamp

Published online: October 30, 2021

e122-e128

Open Access

Indoor air pollution exposure is associated with greater morbidity in cystic fibrosis

Sara W. Carson,
Kevin Psoter,
Kirsten Koehler,
...
Nadia N. Hansel,
Joseph M. Collaco,
Christian A Merlo

Published online: September 13, 2021

e129-e135

$Urinary sodium/creatinine ratio is a predictor for fractional sodium excretion and related to age in patients with cystic fibrosis$

Urinary sodium/creatinine ratio is a predictor for fractional sodium excretion and related to age in patients with cystic fibrosis

Dimitri Declercq,
Lieselot Peremans,
Michiel Glorieus,
...
Evelien Snauwaert,
Sabine Van daele,
Stephanie Van Biervliet

Published online: November 18, 2021

e136-e140

Olfactory dysfunction in cystic fibrosis: Impact of CFTR modulator therapy

Daniel M. Beswick,
Stephen M. Humphries,
Connor D. Balkissoon,
...
Eszter K. Vladar,
Vijay R. Ramakrishnan,
Jennifer L. Taylor-Cousar

Published online: September 28, 2021

e141-e147

Open Access

Magnetic resonance imaging of cystic fibrosis: Multi-organ imaging in the age of CFTR modulator therapies

Kimberly McBennett,
Christina J. MacAskill,
Elise Keshock,
...
Samya Z. Nasr,
Katherine Kutney,
Chris A. Flask

Published online: December 05, 2021

e148-e157

Unexpected associations between respiratory viruses and bacteria with Pulmonary Function Testing in children suffering from Cystic Fibrosis (MUCOVIB study)

Valentin Scherz,
Giorgia Caruana,
Patrick Taffé,
...
Laurent Kaiser,
Gilbert Greub,
Sandra A. Asner

Published online: October 29, 2021

e158-e164

Open Access

Structural lung disease in preschool children with cystic fibrosis: An 18 month natural history study

Alan S. Brody,
Rui Huang,
Bin Zhang,
Frederick R. Long,
Scott W. Powers

Published online: December 24, 2021

e165-e171

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