Pulmonary function in young children with alpha 1-antitrypsin deficiency: comparison with matched control subjects.

In this paper we report the initial cross-sectional data from a prospective study of pulmonary function in children with moderately severe and severe alpha 1-antitrypsin deficiency. Using a case-control design, our cases were 19 children 3 to 7 years of age with alpha 1-antitrypsin deficiency, Pi phenotype ZZ or SZ. Control subjects were selected from healthy children participating in a study to establish reference values for functional residual capacity and maximal expiratory flow at functional residual capacity, using a 1:1 match for sex, height, age, and weight. We found no significant difference between the cases and their matched control subjects with respect to functional residual capacity and maximal expiratory flow at functional residual capacity. We conclude that through 7 years of age there is no gross impairment in overall pulmonary function in children with moderately severe and severe alpha 1-antitrypsin deficiency, Pi phenotypes ZZ and SZ.