Cystic lung light chain deposition disease (CL-LCDD) is a recently described rare disorder characterised by numerous cysts and diffuse monoclonal nonamyloid light chain deposits surrounded by macrophagic giant cells. The mechanisms responsible for cyst development remain unknown. The objectives of the present study were to analyse the major components of the pulmonary extracellular matrix in CL...

a wide divergence of opinion regarding the nomenclature of this abnormality. Oechsli and Miles (1934) attempt to differentiate between cystic bronchiectasis and congenital cystic disease of the lung by invoking the presence or absence of cartilage in the cyst walls. Koontz (1925) uses the terms ' honey-comb lung', congenital cystic disease of the lung, congenital bronchiectasis and foetal bronc...

lung exposure to sulfur mustard (sm) results in pulmonary complications, which is the main cause of long-term disability and morbidity. up to now, the precise mechanisms of sm-induced lung complications has not been identified. the aim of this study was to evaluate apoptosis in the lung tissue of sm-exposed individuals. the study was performed on archived lung paraffin-embedded tissue specimens...

Depletion of the periciliary liquid in "Cystic Fibrosis" airway disease results in reduced mucociliary transport, persistent mucus hypersecretion and consequently increased height of the luminal mucus layer, so hypoxic gradients in the mucus plugs are developed. Because of anaerobic lung zones, it is highly probable that anaerobic bacteria not detected by routine bacteriologic culture methods a...

Pulmonary aspergillomas usually occur when Aspergillus fungi colonise lung tissue previously damaged by disease. Pulmonary aspergillomas in three adult patients with cystic fibrosis are reported--an association not previously described. At the time of diagnosis all three patients had previous long term colonisation with Aspergillus fumigatus and severe advanced destructive lung disease with lun...

Numerous diverse entities produce cystic lung changes in neonates and young children. This review provides an evidence-based, age-appropriate, differential diagnostic framework to use when confronted with pulmonary cystic changes. The categories of diseases that have been discussed include congenital cystic bronchopulmonary malformations, neoplastic conditions, infections, collagen or soft tiss...

INTRODUCTION C ASES OF CARCINOMA ARISING IN A SOLitary congenital lung cyst are exceedingly rare if malignancies arising in lungs with chronic cystic lung disease are not considered. Womack and Graham1 first suggested the possibility of this latter coexistence in 1941 when they reported finding metaplastic masses of poorly differentiated epithelial cells lining some cysts in one-third of their ...

Cystic Fibrosis is the most common lethal autosomal recessive disorder in the white population, affecting among other organs, the lung, the pancreas and the liver. Whereas Cystic Fibrosis is a monogenic disease, many studies reveal a very complex relationship between genotype and clinical phenotype. Indeed, the broad phenotypic spectrum observed in Cystic Fibrosis is far from being explained by...