Literature DB >> 27710011

Cystic Fibrosis: The Dawn of a New Therapeutic Era.

Sonya L Heltshe1,2, Jonathan Cogen1, Kathleen J Ramos3, Christopher H Goss1,3,2.   

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Year:  2017        PMID： 27710011      PMCID： PMC5422649          DOI： 10.1164/rccm.201606-1250PP

Source DB:  PubMed          Journal:  Am J Respir Crit Care Med        ISSN： 1073-449X            Impact factor:   21.405


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  57 in total

Review 1.  Cystic fibrosis in Jews: frequency and mutation distribution.

Authors:  B Kerem; O Chiba-Falek; E Kerem
Journal:  Genet Test       Date:  1997

Review 2.  Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis.

Authors:  M J Welsh; A E Smith
Journal:  Cell       Date:  1993-07-02       Impact factor: 41.582

3.  Continuous alternating inhaled antibiotics for chronic pseudomonal infection in cystic fibrosis.

Authors:  Patrick A Flume; John P Clancy; George Z Retsch-Bogart; D Elizabeth Tullis; Mark Bresnik; P Alex Derchak; Sandra A Lewis; Bonnie W Ramsey
Journal:  J Cyst Fibros       Date:  2016-05-24       Impact factor: 5.482

4.  Outcomes of infants with indeterminate diagnosis detected by cystic fibrosis newborn screening.

Authors:  Clement L Ren; Aliza K Fink; Kristofer Petren; Drucy S Borowitz; Susanna A McColley; Don B Sanders; Margaret Rosenfeld; Bruce C Marshall
Journal:  Pediatrics       Date:  2015-05-11       Impact factor: 7.124

5.  Longevity of patients with cystic fibrosis in 2000 to 2010 and beyond: survival analysis of the Cystic Fibrosis Foundation patient registry.

Authors:  Todd MacKenzie; Alex H Gifford; Kathryn A Sabadosa; Hebe B Quinton; Emily A Knapp; Christopher H Goss; Bruce C Marshall
Journal:  Ann Intern Med       Date:  2014-08-19       Impact factor: 25.391

6.  Lung disease at diagnosis in infants with cystic fibrosis detected by newborn screening.

Authors:  Peter D Sly; Siobhain Brennan; Catherine Gangell; Nicholas de Klerk; Conor Murray; Lauren Mott; Stephen M Stick; Philip J Robinson; Colin F Robertson; Sarath C Ranganathan
Journal:  Am J Respir Crit Care Med       Date:  2009-04-16       Impact factor: 21.405

7.  Some gating potentiators, including VX-770, diminish ΔF508-CFTR functional expression.

Authors:  Guido Veit; Radu G Avramescu; Doranda Perdomo; Puay-Wah Phuan; Miklos Bagdany; Pirjo M Apaja; Florence Borot; Daniel Szollosi; Yu-Sheng Wu; Walter E Finkbeiner; Tamas Hegedus; Alan S Verkman; Gergely L Lukacs
Journal:  Sci Transl Med       Date:  2014-07-23       Impact factor: 17.956

8.  Cystic Fibrosis Foundation evidence-based guidelines for management of infants with cystic fibrosis.

Authors:  Drucy Borowitz; Karen A Robinson; Margaret Rosenfeld; Stephanie D Davis; Kathryn A Sabadosa; Stephanie L Spear; Suzanne H Michel; Richard B Parad; Terry B White; Philip M Farrell; Bruce C Marshall; Frank J Accurso
Journal:  J Pediatr       Date:  2009-12       Impact factor: 4.406

9.  Ataluren for the treatment of nonsense-mutation cystic fibrosis: a randomised, double-blind, placebo-controlled phase 3 trial.

Authors:  Eitan Kerem; Michael W Konstan; Kris De Boeck; Frank J Accurso; Isabelle Sermet-Gaudelus; Michael Wilschanski; J Stuart Elborn; Paola Melotti; Inez Bronsveld; Isabelle Fajac; Anne Malfroot; Daniel B Rosenbluth; Patricia A Walker; Susanna A McColley; Christiane Knoop; Serena Quattrucci; Ernst Rietschel; Pamela L Zeitlin; Jay Barth; Gary L Elfring; Ellen M Welch; Arthur Branstrom; Robert J Spiegel; Stuart W Peltz; Temitayo Ajayi; Steven M Rowe
Journal:  Lancet Respir Med       Date:  2014-05-15       Impact factor: 30.700

10.  Targeted correction and restored function of the CFTR gene in cystic fibrosis induced pluripotent stem cells.

Authors:  Ana M Crane; Philipp Kramer; Jacquelin H Bui; Wook Joon Chung; Xuan Shirley Li; Manuel L Gonzalez-Garay; Finn Hawkins; Wei Liao; Daniela Mora; Sangbum Choi; Jianbin Wang; Helena C Sun; David E Paschon; Dmitry Y Guschin; Philip D Gregory; Darrell N Kotton; Michael C Holmes; Eric J Sorscher; Brian R Davis
Journal:  Stem Cell Reports       Date:  2015-03-12       Impact factor: 7.765
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  9 in total

1.  Organic Acids and Their Salts Potentiate the Activity of Selected Antibiotics against Pseudomonas aeruginosa Biofilms Grown in a Synthetic Cystic Fibrosis Sputum Medium.

Authors:  Xuerui Bao; Mona Bové; Tom Coenye
Journal:  Antimicrob Agents Chemother       Date:  2021-11-22       Impact factor: 5.938

2.  Elastase Exocytosis by Airway Neutrophils Is Associated with Early Lung Damage in Children with Cystic Fibrosis.

Authors:  Camilla Margaroli; Luke W Garratt; Hamed Horati; A Susanne Dittrich; Timothy Rosenow; Samuel T Montgomery; Dario L Frey; Milton R Brown; Carsten Schultz; Lokesh Guglani; Anthony Kicic; Limin Peng; Bob J Scholte; Marcus A Mall; Hettie M Janssens; Stephen M Stick; Rabindra Tirouvanziam
Journal:  Am J Respir Crit Care Med       Date:  2019-04-01       Impact factor: 21.405

3.  Folding and Misfolding of Human Membrane Proteins in Health and Disease: From Single Molecules to Cellular Proteostasis.

Authors:  Justin T Marinko; Hui Huang; Wesley D Penn; John A Capra; Jonathan P Schlebach; Charles R Sanders
Journal:  Chem Rev       Date:  2019-01-04       Impact factor: 60.622

Review 4.  Bridging the Gap between Scientific Advancement and Real-World Application: Pediatric Genetic Counseling for Common Syndromes and Single-Gene Disorders.

Authors:  Julie A McGlynn; Elinor Langfelder-Schwind
Journal:  Cold Spring Harb Perspect Med       Date:  2020-10-01       Impact factor: 5.159

5.  New Perspectives in Underlying Molecular Defects Based Cystic Fibrosis Therapeutics.

Authors:  Rajendra Prasad
Journal:  Indian J Clin Biochem       Date:  2021-06-15

6.  A prospective pilot study of home monitoring in adults with cystic fibrosis (HOME-CF): protocol for a randomised controlled trial.

Authors:  Jocelyn Choyce; Karen L Shaw; Alice J Sitch; Hema Mistry; Joanna L Whitehouse; Edward F Nash
Journal:  BMC Pulm Med       Date:  2017-01-23       Impact factor: 3.317

7.  Critical illness among adults with cystic fibrosis in Texas, 2004-2013: Patterns of ICU utilization, characteristics, and outcomes.

Authors:  Lavi Oud
Journal:  PLoS One       Date:  2017-10-24       Impact factor: 3.240

Review 8.  Altered intravenous drug disposition in people living with cystic fibrosis: A meta-analysis integrating top-down and bottom-up data.

Authors:  Pieter-Jan De Sutter; Maxime Van Haeverbeke; Eva Van Braeckel; Stephanie Van Biervliet; Jan Van Bocxlaer; An Vermeulen; Elke Gasthuys
Journal:  CPT Pharmacometrics Syst Pharmacol       Date:  2022-06-29

9.  Ivacaftor decreases monocyte sensitivity to interferon-γ in people with cystic fibrosis.

Authors:  Katherine B Hisert; Timothy P Birkland; Kelly Q Schoenfelt; Matthew E Long; Brenda Grogan; Suzanne Carter; W Conrad Liles; Edward F McKone; Lev Becker; Anne M Manicone
Journal:  ERJ Open Res       Date:  2020-04-19
  9 in total

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