November 20, 2022

Clinical outcomes associated with Achromobacter species infection in people with cystic fibrosis

  • Eitan Kerem,
  • Annalisa Orenti,
  • Anna Zolin,
  • Luigi Annicchiarico,
  • Pavel Drevinek
  • on behalf of ECFSPR with the list of contributing authors
Published online: November 20, 2022

November 16, 2022

November 15, 2022

The U UGA C sequence provides a favorable context to ELX-02 induced CFTR readthrough

  • Iwona M. Pranke,
  • Jessica Varilh,
  • Aurélie Hatton,
  • ...
  • Magali Taulan,
  • Alexandre Hinzpeter,
  • Isabelle Sermet-Gaudelus
Published online: November 15, 2022

November 10, 2022

Treatment effects of Elexacaftor/Tezacaftor/Ivacaftor in people with CF carrying non-F508del mutations

  • Galit Livnat,
  • Adi Dagan,
  • Moshe Heching,
  • ...
  • Michal Gur,
  • Malena Cohen-Cymberknoh,
  • Michal Shteinberg
Published online: November 10, 2022

European survey of newborn bloodspot screening for CF: opportunity to address challenges and improve performance

  • Anne Munck,
  • Daria O Berger,
  • Kevin W Southern,
  • ...
  • Carlo Castellani,
  • Jürg Barben
  • For the European CF Society Neonatal Screening Working Group (ECFS NSWG)
Published online: November 10, 2022

November 9, 2022

Contemporary cystic fibrosis incidence rates in Canada and the United States

  • Anne L. Stephenson,
  • Sana Swaleh,
  • Jenna Sykes,
  • ...
  • Sameer Desai,
  • Elizabeth Cromwell,
  • Christopher H. Goss
Published online: November 09, 2022

November 5, 2022

Epidemiology and management of nontuberculous mycobacterial disease in people with cystic fibrosis, the Netherlands

  • Domenique Zomer,
  • Jakko van Ingen,
  • Regina Hofland
  • on behalf of the Dutch CF Registry Steering group
Published online: November 05, 2022
Open Access

October 30, 2022

Development of novel therapeutics for all individuals with CF (the future goes on)

  • Margarida D. Amaral,
  • Patrick T. Harrison
Published online: October 30, 2022

October 29, 2022

Multicenter prospective study showing a high gastrointestinal symptom burden in cystic fibrosis

  • Baha Moshiree,
  • A. Jay Freeman,
  • Phuong T. Vu,
  • ...
  • Drucy Borowitz,
  • Meghana Sathe
  • On behalf of the GALAXY Study Group
Published online: October 29, 2022

October 27, 2022

Standards of care for CFTR variant-specific therapy (including modulators) for people with cystic fibrosis

  • Kevin W. Southern,
  • Carlo Castellani,
  • Elise Lammertyn,
  • ...
  • Marieke Verkleij,
  • Michael D. Waller,
  • Alistair Duff
Published online: October 27, 2022
Open Access

October 21, 2022

Elexacaftor/tezacaftor/ivacaftor and gastrointestinal outcomes in cystic fibrosis: Report of promise-GI

  • Sarah Jane Schwarzenberg,
  • Phuong T. Vu,
  • Michelle Skalland,
  • ...
  • Steven M. Rowe,
  • Steven D. Freedman
  • for the Promise Study Group
Published online: October 21, 2022

October 14, 2022

The risk of colorectal cancer in individuals with mutations of the cystic fibrosis transmembrane conductance regulator (CFTR) gene: An English population-based study

  • Rebecca J. Birch,
  • Daniel Peckham,
  • Henry M. Wood,
  • ...
  • Genomics England Research Consortium,
  • Nicholas Burr,
  • Amy Downing
Published online: October 14, 2022
Open Access

October 7, 2022

Structure basis of CFTR folding, function and pharmacology

  • Tzyh-Chang Hwang,
  • Ineke Braakman,
  • Peter van der Sluijs,
  • Isabelle Callebaut
Published online: October 07, 2022

Restoring airway epithelial homeostasis in Cystic Fibrosis

  • Marco Cafora,
  • Marc Chanson,
  • Anna Pistocchi
Published online: October 07, 2022

October 6, 2022

Sexual and reproductive health experiences and care of adult women with cystic fibrosis

  • Traci M. Kazmerski,
  • Olivia M. Stransky,
  • Daniel R. Lavage,
  • ...
  • Joanne Billings,
  • Denis Hadjiliadis,
  • Raksha Jain
Published online: October 06, 2022

Quantity and quality of airway clearance in children and young people with cystic fibrosis

  • Emma Raywood,
  • Harriet Shannon,
  • Nicole Filipow,
  • ...
  • Nicky Murray,
  • Bridget Black,
  • Eleanor Main
Published online: October 06, 2022
Open Access

September 15, 2022

Clinical outcomes of adults and children with cystic fibrosis during the COVID-19 pandemic

  • Michael Doumit,
  • Sandra Chuang,
  • Peter Middleton,
  • ...
  • Verity Pacey,
  • Kelly Gray,
  • Adam Jaffe
Published online: September 15, 2022

Mucus aberrant properties in CF: Insights from cells and animal models

  • Camille Ehre,
  • Gunnar C. Hansson,
  • David J. Thornton,
  • Lynda S. Ostedgaard
Published online: September 15, 2022

September 13, 2022

Non-respiratory health-related quality of life in people with cystic fibrosis receiving elexacaftor/tezacaftor/ivacaftor

  • Isabelle Fajac,
  • Cori Daines,
  • Isabelle Durieu,
  • ...
  • Kate Van Brunt,
  • Yaohua Zhang,
  • Alexandra Quittner
Published online: September 13, 2022
Open Access

September 7, 2022

Trajectories of early growth and subsequent lung function in cystic fibrosis: An observational study using UK and Canadian registry data

  • Amy Macdougall,
  • Deborah Jarvis,
  • Ruth H Keogh,
  • ...
  • Gwyneth Davies,
  • Siobhán B Carr,
  • Sanja Stanojevic
Published online: September 07, 2022
Open Access

CFTR regulates brown adipocyte thermogenesis via the cAMP/PKA signaling pathway

  • Kyung-Mi Choi,
  • Sung-Hee Cho,
  • Jung Hak Kim,
  • Ae-Rhee Lilian Kim,
  • Xiangmudong Kong,
  • John C. Yoon
Published online: September 07, 2022
Open Access

September 4, 2022

Glycemic indices at night measured by CGM are predictive for a lower pulmonary function in adults but not in children with cystic fibrosis

  • Dimitri Declercq,
  • Simon Helleputte,
  • Sophie Marchand,
  • ...
  • Guy T'Sjoen,
  • Stephanie Van Biervliet,
  • Bruno Lapauw
Published online: September 04, 2022

August 28, 2022

August 27, 2022

Evaluation of an association between RANKL and OPG with bone disease in people with cystic fibrosis

  • Shadaan Abid,
  • MinJae Lee,
  • Bailey Rodich,
  • ...
  • Ashley Keller,
  • Gregory Ratti,
  • Raksha Jain
Published online: August 27, 2022
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August 24, 2022

Insulinogenic index and early phase insulin secretion predict increased risk of worsening glucose tolerance and of cystic fibrosis-related diabetes

  • Kathryn J. Potter,
  • Valérie Boudreau,
  • Anne Bonhoure,
  • ...
  • Maité Carricart,
  • Peter A. Senior,
  • Rémi Rabasa-Lhoret
Published online: August 24, 2022

August 23, 2022

Cystic fibrosis transmembrane conductance regulator modulators and the exocrine pancreas: A scoping review

  • Mitchell L. Ramsey,
  • Susan S. Li,
  • Luis F. Lara,
  • ...
  • Alpa Patel,
  • Vikesh K. Singh,
  • Phil A. Hart
Published online: August 23, 2022

August 21, 2022

August 19, 2022

Impact of antibiotic eradication therapy of Pseudomonas aeruginosa on long term lung function in cystic fibrosis

  • Isabel Gascon Casaredi,
  • Michelle Shaw,
  • Valerie Waters,
  • Ryan Seeto,
  • Ana C. Blanchard,
  • Felix Ratjen
Published online: August 19, 2022

August 16, 2022

Long-term follow-up and liver outcomes in children with cystic fibrosis and nodular liver on ultrasound in a multi-center study

  • Daniel H. Leung,
  • Wen Ye,
  • Sarah J. Schwarzenberg,
  • ...
  • Michael R. Narkewicz,
  • Jean P. Molleston
  • CFLD Research Network
Published online: August 16, 2022

August 14, 2022

Prevalence of unmet pain and symptom management needs in adults with cystic fibrosis

  • Ethan Dubin,
  • Jane Lowers,
  • Elisabeth P. Dellon,
  • ...
  • Anne Fitzpatrick,
  • William R Hunt,
  • Dio Kavalieratos
Published online: August 14, 2022

August 13, 2022

β-sitosterol ameliorates inflammation and Pseudomonas aeruginosa lung infection in a mouse model

  • Alice Rossi,
  • Alessandra Bragonzi,
  • Melessike Medede,
  • ...
  • Anna Tamanini,
  • Giulio Cabrini,
  • Maria Cristina Dechecchi
Published online: August 13, 2022
Open Access

August 12, 2022

Partner perspectives on women's sexual and reproductive healthcare in cystic fibrosis

  • Sarah Ehrenberg,
  • Olivia M. Stransky,
  • Catherine E. Wright,
  • Raksha Jain,
  • Traci M. Kazmerski
Published online: August 12, 2022

August 10, 2022

Super resolution microscopy analysis reveals increased Orai1 activity in asthma and cystic fibrosis lungs

  • Alexandra S. Goriounova,
  • Rodney C. Gilmore,
  • Joe A. Wrennall,
  • Robert Tarran
Published online: August 10, 2022

August 7, 2022

Antibiotics and outcomes of CF pulmonary exacerbations in children infected with MRSA and Pseudomonas aeruginosa

  • Jonathan D. Cogen,
  • Matthew Hall,
  • Anna V. Faino,
  • ...
  • Daniel J. Shapiro,
  • Mark I. Neuman,
  • Jeffrey S. Gerber
Published online: August 07, 2022

August 4, 2022

Disease severity of people with cystic fibrosis carrying residual function mutations: Data from the ECFS Patient Registry

  • Meir Mei Zahav,
  • Annalisa Orenti,
  • Andreas Jung,
  • ...
  • Hanne Vebert Olesen,
  • Eitan Kerem
  • on behalf of ECFSPR with the list of contributing authors
Published online: August 04, 2022

July 27, 2022

July 22, 2022

Telehealth and CFTR modulators: Accelerating innovative models of cystic fibrosis care

  • Michelle H. Prickett,
  • Patrick A. Flume,
  • Kathryn A. Sabadosa,
  • Quynh T. Tran,
  • Bruce C. Marshall
Published online: July 22, 2022
Open Access

July 21, 2022

An invisible threat? Aspergillus positive cultures and co-infecting bacteria in airway samples

  • Dominic A Hughes,
  • Mark Rosenthal,
  • Leah Cuthbertson,
  • ...
  • William O Cookson,
  • Miriam F Moffatt,
  • Jane C Davies
Published online: July 21, 2022
Open Access

Characterizing CFTR modulated sweat chloride response across the cf population: Initial results from the CHEC-SC study

  • N Mayer-Hamblett,
  • ET Zemanick,
  • K Odem-Davis,
  • ...
  • J Young,
  • MW Konstan,
  • for-the-CHEC-SC-Study-Group
Published online: July 21, 2022

Acceptability and feasibility of a brief behavioral sleep intervention for youth with CF

  • Kimberly S. Canter,
  • Abigail Strang,
  • Sophie Wilks,
  • Katherine Okonak,
  • Aaron Chidekel
Published online: July 21, 2022

Disparities in first evaluation of infants with cystic fibrosis since implementation of newborn screening

  • Susanna A. McColley,
  • Stacey L. Martiniano,
  • Clement L. Ren,
  • ...
  • Alexander Elbert,
  • Runyu Wu,
  • Philip M. Farrell
Published online: July 21, 2022
Open Access

Clinician attitudes and practices on pregnancy planning and care in cystic fibrosis

  • Kristina Montemayor,
  • Traci M. Kazmerski,
  • Kristin A. Riekert,
  • ...
  • Rebecca L. Boyle,
  • Shivani Patel,
  • Natalie E. West
Published online: July 21, 2022

July 19, 2022

July 13, 2022

Development and outcomes of a patient driven cystic fibrosis quality of care improvement project

  • Jacquelien J. Noordhoek,
  • Joshena Jeyaratnam,
  • Domenique Zomer,
  • Vincent A.M. Gulmans,
  • Cornelis K. van der Ent,
  • Harry G.M. Heijerman
Published online: July 13, 2022

July 6, 2022

Cystic fibrosis related diabetes is not associated with maximal aerobic exercise capacity in cystic fibrosis: a cross-sectional analysis of an international multicenter trial

  • Thomas Radtke,
  • Susi Kriemler,
  • Lothar Stein,
  • ...
  • Sarah R Haile,
  • Helge Hebestreit
  • on behalf of the ACTIVATE-CF Study Working Group
Published online: July 06, 2022

July 5, 2022

June 23, 2022

Comparative analysis of respiratory symptom scores to detect acute respiratory events in children with cystic fibrosis

  • Lucy Perrem,
  • Sanja Stanojevic,
  • Michelle Shaw,
  • ...
  • Valerie Waters,
  • Stephanie D Davis,
  • Felix Ratjen
Published online: June 23, 2022

May 22, 2022

VOCAL: An observational study of ivacaftor for people with cystic fibrosis and selected non–G551D-CFTR gating mutations

  • Nicholas J. Simmonds,
  • C. Kors van der Ent,
  • Carla Colombo,
  • ...
  • Marci L. Chew,
  • Keval Chandarana,
  • Carlo Castellani
Published online: May 22, 2022
Open Access

May 13, 2022

Rapid chloride and bicarbonate determination by capillary electrophoresis for confirmatory testing of cystic fibrosis infants with volume-limited sweat specimens

  • Stellena Mathiaparanam,
  • Adriana Nori de Macedo,
  • Biban Gill,
  • ...
  • Linda Pedder,
  • Stephen Hill,
  • Philip Britz-McKibbin
Published online: May 13, 2022
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