Panacinar emphysema

Alpha-1-Protease Inhibitor (Prolastin) [Respiratory Agent/Alpha Protease Inhibitor Replacement] Uses a,-Antit-rypsin deficiency panacinar emphysema Action Replace human aj-protease inhibitor Dose 60 mg/kg IV once/wk Caution [C, ] Contra Selective IgA deficiencies w/ known IgA antibodies Disp Inj SE Fever, dizziness, flu-like Sxs, allergic Rxns EMS Pt may have chronic lung Dz/damage, monitor resp status OD Not expected to produce life-threatening Sxs... 

Indications Replacement therapy in patients with congenital alpha-1 antitrypsin deficiency who have panacinar emphysema... 

Clinical pharmacology Alpha-1 antitrypsin deficiency is a chronic, hereditary, usually fatal, autosomal recessive disorder in which a low concentration of alphai-proteinase inhibitor is associated with slowly progressive, severe, panacinar emphysema that most often manifests itself in the third to fourth decades of fife. The pathogenesis of development of emphysema in alpha-1 antitrypsin deficiency is believed to be due to a chronic biochemical imbalance between elastase and alphai-proteinase inhibitor (the principal inhibitor of neutrophil elastase), which is deficient in alpha-1 antitrypsin disease. As a result it is believed that alveolar structures are unprotected from chronic exposure to elastase released from a chronic low-level burden of neutrophils in the lower respiratory tract, resulting in progressive degradation... 

Morphologically, emphysema is associated with a destruction of the alveolar septum, which results in a dilation and consequent enlargement of the alveolar spaces (Fig. 19). This is apparently caused by a breakdown of the interstitial connective tissue proteins (primarily elastin) that provide the major structural framework of the lung parenchyma. Two types of emphysema have been defined on the basis of the types of destruction of the alveolar septa observed and the type of dilation of the terminal respiratory unit (the acini) that is observed. A typical acinus branches from a terminal bronchiole and consists of the respiratory bronchioles that have alveolated walls and lead to the alveolar ducts and ultimately to the alveolar sacs (see Fig. 3). In centrilobular (or centriacinar) emphysema, the sites of degradation and dilation are limited to the region of the terminal and respiratory bronchioles. In panlobular (or panacinar) emphysema, the entire acinus (including the alveolar ducts and sacs) is more uniformly affected. 

Freedman PM et al. (1981) Skin testing in farmers lung disease. J Allergy Clin Immunol 67(l) 51-58 Fukuda Y et al. (1989) Morphogenesis of abnormal elastic fibers in lungs of patients with panacinar and centriacinar emphysema. Hum Pathol 20(7) 652-659 Fuller CJ (1953) Farmer s lung a review of present knowledge. Thorax 8(l) 59-64... 

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