Targeting Proteases in Cystic Fibrosis Lung Disease: Paradigms, Progress, and Potential

Initial acquisition and succession of the cystic fibrosis lung microbiome is associated with disease progression in infants and preschool children

PLoS Pathog.

Muhlebach MS, Zorn BT, Esther CR, Hatch JE, Murray CP, Turkovic L, Ranganathan SC, Boucher RC, Stick SM, Wolfgang MC

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Pulmonary Fibrosis

Phenotypes of Rapid Cystic Fibrosis Lung Disease Progression during Adolescence and Young Adulthood

Am J Respir Crit Care Med.

Szczesniak RD, Li D, Su W, Brokamp C, Pestian J, Seid M, Clancy JP

doi: 10.1164/rccm.

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Pulmonary Fibrosis

Airway profile of bioactive lipids predicts early progression of lung disease in cystic fibrosis

★ 1 Recommended by Dr Martin Frey, Faculty Member

J Cyst Fibros.

Horati H, Janssens HM, Margaroli C, Veltman M, Stolarczyk M, Kilgore MB, Chou J, Peng L, Tiddens HAMW, Chandler JD, Tirouvanziam R, Scholte BJ

doi: 10.1016/j.jcf.2020.01.010.

Pulmonary Fibrosis

Early follow-up of lung disease in infants with cystic fibrosis using the raised volume rapid thoracic compression technique and computed tomography during quiet breathing

Pediatr Pulmonol.

Gauthier R, Cabon Y, Giroux-Metges MA, Du Boisbaudry C, Reix P, Le Bourgeois M, Chiron R, Molinari N, Saguintaah M, Amsallem F, Matecki S

doi: 10.1002/ppul.23786.

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Determinants of lung disease progression measured by lung clearance index in children with cystic fibrosis

★ 2 Recommended by Dr Martin Frey, Faculty Member

Eur Respir J

Stanojevic S, Davis SD, Perrem L, Shaw M, Retsch-Bogart G, Davis M, Jensen R, Clem CC, Isaac SM, Guido J, Jara S, France L, McDonald N, Solomon M, Sweezey N, Grasemann H, Waters V, Sanders DB, Ratjen FA

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Magnetic Resonance Imaging Detects Progression of Lung Disease and Impact of Newborn Screening in Preschool Children with Cystic Fibrosis

★ 1 Recommended by Dr Martin Frey, Faculty Member

Am J Respir Crit Care Med

Stahl M, Steinke E, Graeber SY, Joachim C, Seitz C, Kauczor HU, Eichinger M, Hämmerling S, Sommerburg O, Wielpütz MO, Mall MA

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Mucus plugging, air trapping, and bronchiectasis are important outcome measures in assessing progressive childhood cystic fibrosis lung disease

★ 2 Recommended by Dr Martin Frey, Faculty Member

★ 1 Recommended by Dr Marcus Hesse, Faculty Member

Pediatr Pulmonol.

Robinson TE, Goris ML, Moss RB, Tian L, Kan P, Yilma M, McCoy KS, Newman B, de Jong PA, Long FR, Brody AS, Behrje R, Yates DP, Cornfield DN

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Pulmonary Fibrosis

Tracheal diverticula in advanced cystic fibrosis: Prevalence, features, and outcomes after lung transplantation

J Cyst Fibros.

Kapnadak SG, Kicska GA, Ramos KJ, Marshall DA, Carroll TY, Pipavath SN, Mulligan MS, Goss CH, Aitken ML

doi: 10.1016/j.jcf.2017.07.015.

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Cystic Fibrosis Foundation consensus guidelines for the care of individuals with advanced cystic fibrosis lung disease

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★ 3 Recommended by Dr Marcus Hesse, Faculty Member

J Cyst Fibros.

Kapnadak SG, Dimango E, Hadjiliadis D, Hempstead SE, Tallarico E, Pilewski JM, Faro A, Albright J, Benden C, Blair S, Dellon EP, Gochenour D, Michelson P, Moshiree B, Neuringer I, Riedy C, Schindler T, Singer LG, Young D, Vignola L, Zukosky J, Simon RH

doi: 10.1016/j.jcf.2020.02.015.

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Initial acquisition and succession of the cystic fibrosis lung microbiome is associated with disease progression in infants and preschool children

Phenotypes of Rapid Cystic Fibrosis Lung Disease Progression during Adolescence and Young Adulthood

Airway profile of bioactive lipids predicts early progression of lung disease in cystic fibrosis

Early follow-up of lung disease in infants with cystic fibrosis using the raised volume rapid thoracic compression technique and computed tomography during quiet breathing

Determinants of lung disease progression measured by lung clearance index in children with cystic fibrosis

Magnetic Resonance Imaging Detects Progression of Lung Disease and Impact of Newborn Screening in Preschool Children with Cystic Fibrosis

Mucus plugging, air trapping, and bronchiectasis are important outcome measures in assessing progressive childhood cystic fibrosis lung disease

Tracheal diverticula in advanced cystic fibrosis: Prevalence, features, and outcomes after lung transplantation

Cystic Fibrosis Foundation consensus guidelines for the care of individuals with advanced cystic fibrosis lung disease