Air Trapping and Airflow Obstruction in Newborn Cystic Fibrosis Piglets

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

BACKGROUND In school-aged children with cystic fibrosis (CF) structural lung damage assessed using chest CT is associated with abnormal ventilation distribution. The primary objective of this analysis was to determine the relationships between ventilation distribution outcomes and the presence and extent of structural damage as assessed by chest CT in infants and young children with CF. METHO...

Prednisolone in the treatment of airflow obstruction in adults with cystic fibrosis.

The effect of oral prednisolone on the lung function of 20 adult patients with cystic fibrosis who had severe stable airflow obstruction was assessed in a placebo controlled study, blind to the patients. Placebo tablets were followed by prednisolone given in a median dose of 0.48 mg/kg body weight/day (20 mg/day in 11 patients, 30 mg/day in nine patients), each for three weeks. No significant i...

Free DNA in Cystic Fibrosis Airway Fluids Correlates with Airflow Obstruction

Chronic obstructive lung disease determines morbidity and mortality of patients with cystic fibrosis (CF). CF airways are characterized by a nonresolving neutrophilic inflammation. After pathogen contact or prolonged activation, neutrophils release DNA fibres decorated with antimicrobial proteins, forming neutrophil extracellular traps (NETs). NETs have been described to act in a beneficial way...

Newborn Screening for Cystic Fibrosis in California.

OBJECTIVES This article describes the methods used and the program performance results for the first 5 years of newborn screening for cystic fibrosis (CF) in California. METHODS From July 16, 2007, to June 30, 2012, a total of 2,573,293 newborns were screened for CF by using a 3-step model: (1) measuring immunoreactive trypsinogen in all dried blood spot specimens; (2) testing 28 to 40 select...

Superior vena cava obstruction in cystic fibrosis.