Pompe disease is an autosomal recessive disorder caused by a deficiency of acid α-glucosidase (GAA), an enzyme responsible for hydrolyzing lysosomal glycogen. Deficiency of GAA leads to systemic glycogen accumulation in the lysosomes of skeletal muscle, motor neurons, and smooth muscle. Skeletal muscle and motor neuron pathology are known to contribute to respiratory insufficiency in Pompe dise...

Steroids have been found to facilitate cholinergic transmission in skeletal muscle, but possible effects in airways smooth muscles have not been studied. Therefore, choline acetyltransferase (CAT) activity, tissue content of stored acetylcholine and release of newly-synthesized [3H]acetylcholine were were measured in freshly-dissected human bronchi. All lung tissue was obtained from patients wi...

We characterized the localization and prevalence of dendritic cells (DC) in guinea pig airways before and after s.c. sensitization and aerosol challenge with ovalbumin (OVA). DC, eosinophils, macrophages, T cells and B cells in lung and trachea were identified and quantified in frozen sections using monoclonal antibodies and computer-assisted image analysis. Airway reactivity of conscious anima...

A quantitative study is here reported on the lungs of a child of 9 months with unilateral emphysema associated with hypoplasia, and with compensatory emphysema in the contralateral lung. The left lung was judged to show congenital hypoplasia because of the reduced number of bronchi and arteries, its small volume, and a total alveolar number less than that normally found at birth. This last was ...

To clarify the taxonomy of trichomonads associated with human respiratory diseases, we examined a collection of axenic trichomonad strains isolated from the oral cavity and bronchi of patients from pulmonary diseases clinics in Tallin, Estonia. The oral and bronchial strains were compared mutually as well as with a reference strain of Trichomonas tenax, a common inhabitant of the human oral cav...

The rhesus monkey lung was stained both by histological methods and histochemically for specific acetylcholinesterase (AChE). AChE-containing nerves in bundles were demonstrated in connective tissue of the hilum and in association with clusters of ganglion cells. These bundles become associated with the bronchial tree as they enter the lung parenchyma, and their numbers of myelinated fibres dim...

This study investigated the effect of cigarette smoke exposure and the potential protection N-acetylcysteine (NAC) in rat lungs. Forty-eight rats were exposed to cigarette smoke (CS) for 10 weeks, without (CS group) or with (CS+NAC group) oral intake of NAC 200 mg x rat(-1) x day(-1), or to fresh air (Control). All rat lungs were assessed in terms of lung function, ventilation distribution (nit...

OBJECTIVES General thoracic surgeons must be familiar with anatomical variation in the pulmonary vessels and bronchi. Here, we analyzed the bronchovascular pattern of the right middle lobe (RML) and right lower lobe (RLL) of the lung using three-dimensional CT angiography and bronchography (3DCTAB). METHODS We reviewed the anatomical patterns of the pulmonary vessels and bronchi in 270 patien...

Using computational fluid dynamics (CFD) method, the feasibility of simulating transient airflow in a CT-based airway tree with more than 100 outlets for a whole respiratory period is studied, and the influence of truncations of terminal bronchi on CFD characteristics is investigated. After an airway model with 122 outlets is extracted from CT images, the transient airflow is simulated. Spatial...