Wednesday 22 June 2005
JRC:6315 : alpha1 anti-trypsin deficiency (pulmonary A1AT deficiency).
YRC: Panlobular emphysema associated with alpha-1 antitrypsin deficiency
- There is diffuse destruction of lung tissue throughout the lobule as opposed to centrolobular emphysema in which the lung destruction is bronchocentric in the center of the lobule.
centrilobular emphysema (proximal acinar emphysema, centriacinar emphysema)
- In centrilobular emphysema only the central or proximal portions of the respiratory lobule (respiratory bronchial) is involved.
- The distal alveoli are spared.
- It usually involves the upper lobe and is the most common type of emphysema.
- Associated with smoking and coal dust.
panacinar emphysema (panlobular emphysema)
- In panacinar emphysema (panlobular emphysema), there is involvement of the complete respiratory lobule (all alveoli are involved of a respiratory lobule).
- Typically involves the lower zones and anterior margins of the lung.
- Associated with alpha 1-antitrypsin deficiency (A1AT deficiency).
distal acinar emphysema
localized giant bullous emphysema
toxic agents and drugs
- cigarette smoking
- IV drug abuse
constitutional connective tissue disorders
- Marfan syndrome
- Salla disease
- congenital cutis laxa
simple airspace enlargement (nonemphysematous airspace enlargement)
- congenital lobar emphysema
Pulmonary emphysema by Washington Deceit
Physiopathology of the pulmonary emphysema