Pulmonary disease in alpha-1-antitrypsin deficiency

Article Abstract:

Alpha-1-antitrypsin (AAT) is a naturally occurring enzyme in blood that helps maintain proper tissue function. It inhibits enzymes that break down certain proteins (proteases);most commonly the protease involved is neutrophil elastase. In its absence, neutrophil elastase is then free to break down proteins that should properly be left intact. This protease imbalance ultimately destroys healthy tissue; the primary target is the lungs. Patients with severe AAT deficiency develop emphysema. The development of a source of AAT to augment the patient's inadequate blood levels provides hope for the patient and an excellent example for the physician of how modern technology poses new problems as it creates new hope. AAT supplements for a 150-pound patient would cost about $25,000 per year. This means that patients with AAT deficiency will probably not receive the drug until after they have deteriorated somewhat, despite the fact that early treatment may keep them perfectly healthy. Indeed, even patients with poor lung function may not receive treatment unless they are clearly on a downhill path. The American Thoracic Society has released some guidelines for the administration of AAT supplementation. Supplementation is not appropriate for AAT deficiency patients with only liver disease and no lung involvement. The Society considers it unethical to withhold AAT supplementation from a patient who would benefit by it, no matter how old the patient might be or how seriously deteriorated his clinical situation might be. Currently AAT supplementation is made by intravenous infusion. Development of practical aerosol administration and the reduction of cost, perhaps through recombinant DNA technology, may make AAT supplementation an even more valuable therapeutic regimen. (Consumer Summary produced by Reliance Medical Information, Inc.)

Lung diseases, Obstructive, Chronic obstructive lung disease, Emphysema, Pulmonary, Emphysema, editorial

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Anti-neutrophil-elastase defenses of the lower respiratory tract in alpha 1-antitrypsin deficiency directly augmented with an aerosol of alpha 1-antitrypsin

Article Abstract:

Alpha 1-antitrypsin deficiency is an inherited disorder characterized by low blood and lung levels of alpha 1-antitrypsin, a protein and carbohydrate compound that inhibits proteolytic or protein-degrading enzymes. Alpha 1-antitrypsin protects against neutrophil elastase, a proteolytic enzyme capable of destroying the walls of the alveoli or air sacs of the lungs. A deficiency in alpha 1-antitrypsin results in the loss of anti-neutrophil-elastase protection of the lower respiratory tract and leads to the development of emphysema, the distention of the lungs by gas or air. The effectiveness of an aerosol preparation of purified human plasma alpha 1-antitrypsin in improving anti-neutrophil elastase defenses in the lower respiratory tract was assessed in 12 patients with a deficiency of alpha 1-antitrypsin. The aerosol preparation increased alpha 1-antitrypsin levels in the epithelial cells lining the lung from 0.28 micromole (uM) before therapy to 5.86 uM after therapy. Anti-neutrophil-elastase capacity was increased from 0.78 uM before therapy to 4.16 uM after therapy. Aerosolized alpha 1-antitrypsin was shown to diffuse from the lung epithelial cells to cells within the lung tissue and eventually to the bloodstream, and was not associated with any side effects. Thus, short-term use of aerosolized alpha 1-antitrypsin is both safe and effective in treating alpha 1-antitrypsin deficiency, and improves anti-neutrophil-elastase defenses in the lower respiratory tract. (Consumer Summary produced by Reliance Medical Information, Inc.)

Evaluation, Drug therapy, Lung diseases, Aerosol therapy, Alpha 1-antitrypsin deficiency

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Tuberculosis then and now: a personal perspective on the last 50 years

Article Abstract:

The control of tuberculosis since the 1940's has been dramatic but continued control will require constant vigilance by governments, pharmaceutical industries, and health care workers worldwide. The incidence of tuberculosis in the United States has dropped from 194 infections per 100,000 persons in 1900 to 8.7 infections per 100,000 persons in 1995. This reduction was due in part to the discovery of para-aminosalicylic acid, streptomycin, and isoniazid treatments. Increases in federal funding, supervised therapies, and patient monitoring curbed the increase in infection rates during the 1980's.

Tuberculosis

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