Lung clearance index in CF: a sensitive marker of lung disease severity

Lung clearance index in CF: a sensitive marker of lung disease severity.

Is Lung Clearance Index (LCI) Affected by the Severity of Lung Disease in CF?

Background: LCI has been proven an effective tool in the detection of lung disease in CF. Objectives: a) To assess the correlation of ventilation inhomogeneity indices with structural damages of the lung, among different groups of disease severity and b) To compare the associations among MBW parameters with spirometry and CT. Method: Forty-four children and adolescents with CF participated in t...

Lung Clearance Index and HRCT are complementary markers of lung abnormalities in young children with CF.

RATIONALE High resolution computed tomography (HRCT) is a more sensitive tool for detecting early cystic fibrosis (CF) lung disease than either spirometry or plain radiography, but its relationship to other measures of lung function has not been established in young children. OBJECTIVES (1) To assess whether the lung clearance index (LCI) derived from multiple breath inert-gas washout (MBW) i...

Cystic Lung Disease in a Young man

A 32-year-old man was admitted in the Emergency Department with acute dyspnea secondary to spontaneous pneumothorax. He had history of spontaneous pneumothorax 2 years ago.He was active cigarette smoker (10 pack/year). The family history was unremarkable for lung diseases. Additionally, he had no systemic complaints.The lung HRCT revealed multiple bizarre-shaped cysts distributed in both lungs ...

Regulatory dynamics of 11p13 suggest a role for EHF in modifying CF lung disease severity

Mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene cause cystic fibrosis (CF), but are not good predictors of lung phenotype. Genome-wide association studies (GWAS) previously identified additional genomic sites associated with CF lung disease severity. One of these, at chromosome 11p13, is an intergenic region between Ets homologous factor (EHF) and Apaf-1 interac...