Chest
Volume 89, Issue 3, March 1986, Pages 370-373
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Clinical Investigations
Alpha1-Antitrypsin Pi-Types in 965 COPD Patients

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To study further the role of intermediate alpha1-antitrypsin (AAT) deficiency in chronic obstructive pulmonary disease (COPD), AAT Pi-types and serum-trypsin-inhibitory-capacity (STIC) were measured in 965 patients with COPD. Heterozygosity of the Z variant was the major cause of intermediate AAT deficiency (primarily the MZ phenotype), accounting for 8.0 percent of the patients compared to 2.9 percent of control subjects (p<.0005). ZZ homozygosity was detected in 1.9 percent of the patients, compared to 0.04 percent of control studies performed by others (none was present in our own control group of 1,380 subjects). The mean age for MS or MZ patients did not differ from that of the COPD patients as a whole, whereas the ZZ homozygotes were younger (55.9±9.8 vs 65.3±7.5 years). These results resemble those of a previous study in 66 male veterans with pulmonary emphysema suggesting that the MZ phenotype, or intermediate AAT deficiency in general, probably does predispose to the development of COPD. However, the prevalence of AAT deficiency in COPD patients is small (approximately 10 percent). The number with an MS phenotype was not increased in this group of COPD patients.

Section snippets

Battent Selection

Blood samples were obtained from consecutive patients hospitalized for carotid body surgery by BW; serum was separated and delivered to our laboratory at the Sepulveda VA Medical Center. All patients were Caucasian, ranging in age from 41 to 85 years, with a mean± SD of 65.3±7.5 yrs; 28 percent of the patients were women. All had far-advanced COPD with severe dyspnea as their main complaint. Information such as age of onset of lung disease was not available.

STIC Assay

STIC was assayed essentially as

Prevalence of Abnormal Phenotypes

Two separate formats were used for evaluating the Pi-type of these sera. Initially, for the first 223 specimens, phenotyping was performed only when the STIC value was less than 1.2 units. Subsequently, for the next 742 specimens, phenotyping was performed on all sera, irrespective of the STIC value. Table 1 shows that approximately half of the MS Pi-types were missed initially by not typing all sera; 4.9 percent MS types appeared with STIC values less than 1.2 units, whereas 10.1 percent

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    Supported by the Medical Research Service of the Veterans Administration.

    Manuscript received July 15; revision accepted September 12.

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