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Introduction

alpha1-Antitrypsin (alpha1-AT) is an alpha1-globulin produced by the liver. Its function is inhibition of the proteolytic enzymes trypsin and plasmin, which are released by alveolar macrophages and by bacteria in the lungs. As with many other proteins, the alpha1-AT molecule has several structural variants. Some of these variant molecules have different electrophoretic mobility and reduced ability to inhibit proteolytic enzymes.

Inherited deficiencies in normal alpha1-AT activity are associated with the development, early in life, of lung and liver disorders in which functional tissue is destroyed and replaced with excessive connective tissue; that is, emphysema and cirrhosis may develop in children and young adults who are deficient in alpha1-AT, without the usual predisposing factors associated with onset of these disorders. Such deficiencies are seen on serum protein electrophoresis as a flat area where the normal alpha1-globulin hump should be. More detailed physiochemical analysis can demonstrate which variant form is present. Decreased levels of alpha1-AT also are seen in nephrotic syndrome and malnutrition.

Reference Values

Interfering Factors

Indications

Care Before Procedure

Nursing Care Before the Procedure

Client preparation is the same as that for any test involving collection of a peripheral blood sample (see Appendix I).

Procedure

A venipuncture is performed and the sample collected in a red-topped tube. The sample should be handled gently to avoid hemolysis and frozen if not tested immediately.

Care After Procedure

Nursing Care After the Procedure

Care and assessment after the procedure are the same as for any study involving the collection of a peripheral blood sample.