Pathophysiology and management of pulmonary infections in cystic fibrosis

RL Gibson, JL Burns, BW Ramsey - American journal of respiratory …, 2003 - atsjournals.org
This comprehensive State of the Art review summarizes the current published knowledge
base regarding the pathophysiology and microbiology of pulmonary disease in cystic …

Mucociliary clearance in the airways.

A Wanner, M Salathé, TG O'Riordan - American journal of …, 1996 - atsjournals.org
The ventilation of the lung ranges between 1,000 and 21,000 liters per 24 hours in humans
depending on body size and physical activity. This exposes the extensive epithelial surface …

The diagnosis of cystic fibrosis

RC Stern - New England Journal of Medicine, 1997 - Mass Medical Soc
Cystic fibrosis is an autosomal recessive disease caused by mutations of a gene located on
the long arm of chromosome 7.1 The gene product is the 1480-amino-acid cystic fibrosis …

[HTML][HTML] Activation by extracellular nucleotides of chloride secretion in the airway epithelia of patients with cystic fibrosis

MR Knowles, LL Clarke… - New England Journal of …, 1991 - Mass Medical Soc
Background. Cystic fibrosis is characterized by abnormal electrolyte transport across the
epithelia of the airways. In particular, there is excessive sodium absorption and de.. cient …

Correction of the ion transport defect in cystic fibrosis transgenic mice by gene therapy

SC Hyde, DR Gill, CF Higgins, AEO Trezise… - Nature, 1993 - nature.com
CYSTIC fibrosis (CF) is a lethal inherited disorder affecting about 1 in 2,000 Caucasians.
The major cause of morbidity is permanent lung damage resulting from ion transport …

Effect of hypertonic saline, amiloride, and cough on mucociliary clearance in patients with cystic fibrosis.

M Robinson, JA Regnis, DL Bailey, M King… - American journal of …, 1996 - atsjournals.org
In patients with cystic fibrosis (CF), dehydration of airway secretions leads to a decrease in
mucociliary clearance (MCC). We examined the acute effect of MCC of a single …

Sputum rheology changes in cystic fibrosis lung disease following two different types of physiotherapy: flutter vs autogenic drainage

EM App, R Kieselmann, D Reinhardt, H Lindemann… - Chest, 1998 - Elsevier
Objective The aim of the present study was to investigate the efficacy of two frequently used
physiotherapies (PTs) for the removal of bronchial secretions in cystic fibrosis (CF) lung …

Outcome measures for clinical trials in cystic fibrosis Summary of a Cystic Fibrosis Foundation Consensus Conference

BW Ramsey, TF Boat - The Journal of pediatrics, 1994 - jpeds.com
Cystic fibrosis is the most common life-shortening autosomal recessive disease of white
persons in the United States, affecting approximately 30,000 individuals. The median 1992 …

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

AF Hoo, LP Thia, A Bush, J Chudleigh, S Lum… - Thorax, 2012 - thorax.bmj.com
Background Long-term benefits of newborn screening (NBS) for cystic fibrosis (CF) have
been established with respect to nutritional status, but effects on pulmonary health remain …

Effects of drugs on mucus clearance

E Houtmeyers, R Gosselink… - European …, 1999 - publications.ersnet.org
Mucociliary clearance (MCC), the process in which airway mucus together with substances
trapped within are moved out of the lungs, is an important defence mechanism of the human …