Air Trapping and Airflow Obstruction in Newborn Cystic Fibrosis Piglets

Loss of Cftr function exacerbates the phenotype of Na+ hyperabsorption in murine airways

(2013) Alessandra Livraghi-Butrico et al.   AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY

Impact of bronchiectasis and trapped air on quality of life and exacerbations in cystic fibrosis

(2013) Leonie A. Tepper et al.   EUROPEAN RESPIRATORY JOURNAL

Distribution of Early Structural Lung Changes due to Cystic Fibrosis Detected with Chest Computed Tomography

(2013) Lauren S. Mott et al.   JOURNAL OF PEDIATRICS

Risk Factors for Bronchiectasis in Children with Cystic Fibrosis

(2013) Peter D. Sly et al.   NEW ENGLAND JOURNAL OF MEDICINE

CFTR-deficient pigs display peripheral nervous system defects at birth

(2013) L. R. Reznikov et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

CFTR, Mucins, and Mucus Obstruction in Cystic Fibrosis

(2013) S. M. Kreda et al.   Cold Spring Harbor Perspectives in Medicine

Computer-aided analysis of airway trees in micro-CT scans of ex vivo porcine lung tissue

(2012) Christian Bauer et al.   COMPUTERIZED MEDICAL IMAGING AND GRAPHICS

Reduced airway surface pH impairs bacterial killing in the porcine cystic fibrosis lung

(2012) Alejandro A. Pezzulo et al.   NATURE

Lung function is abnormal in 3-month-old infants with cystic fibrosis diagnosed by newborn screening

(2012) Ah-Fong Hoo et al.   THORAX

Air Trapping on Chest CT Is Associated with Worse Ventilation Distribution in Infants with Cystic Fibrosis Diagnosed following Newborn Screening

(2011) Graham L. Hall et al.   PLoS One

The  F508 Mutation Causes CFTR Misprocessing and Cystic Fibrosis-Like Disease in Pigs

(2011) L. S. Ostedgaard et al.   Science Translational Medicine

Progression of early structural lung disease in young children with cystic fibrosis assessed using CT

(2011) Lauren S Mott et al.   THORAX

Loss of Cystic Fibrosis Transmembrane Conductance Regulator Function Produces Abnormalities in Tracheal Development in Neonatal Pigs and Young Children

(2010) David K. Meyerholz et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Loss of Anion Transport without Increased Sodium Absorption Characterizes Newborn Porcine Cystic Fibrosis Airway Epithelia

(2010) Jeng-Haur Chen et al.   CELL

Cystic Fibrosis Pigs Develop Lung Disease and Exhibit Defective Bacterial Eradication at Birth

(2010) D. A. Stoltz et al.   Science Translational Medicine

Lung Disease at Diagnosis in Infants with Cystic Fibrosis Detected by Newborn Screening

(2009) Peter D. Sly et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Bronchiectasis in Infants and Preschool Children Diagnosed with Cystic Fibrosis after Newborn Screening

(2009) Stephen M. Stick et al.   JOURNAL OF PEDIATRICS

Cystic fibrosis

(2009) Brian P O'Sullivan et al.   LANCET

Increased Airway Smooth Muscle Mass in Children with Asthma, Cystic Fibrosis, and Non-Cystic Fibrosis Bronchiectasis

(2008) Nicolas Regamey et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Role of the Cystic Fibrosis Transmembrane Conductance Channel in Human Airway Smooth Muscle

(2008) Marie-Claire Michoud et al.   AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY

Production of CFTR-null and CFTR-ΔF508 heterozygous pigs by adeno-associated virus–mediated gene targeting and somatic cell nuclear transfer

(2008) Christopher S. Rogers et al.   JOURNAL OF CLINICAL INVESTIGATION

Congenital tracheal malformation in cystic fibrosis transmembrane conductance regulator-deficient mice

(2008) Elise Bonvin et al.   JOURNAL OF PHYSIOLOGY-LONDON

Cystic fibrosis: impaired bicarbonate secretion and mucoviscidosis

(2008) Paul M Quinton   LANCET

Disruption of the CFTR Gene Produces a Model of Cystic Fibrosis in Newborn Pigs

(2008) C. S. Rogers et al.   SCIENCE