Article
Pulmonary function in children with homozygous alpha1-protease inhibitor deficiency.
European journal of pediatrics - 1 Jul 1996
Wiebicke W, Niggemann B, Fischer A
Abstract excerpt
UNLABELLED: Alpha1-protease inhibitor (alpha1-PI) deficiency is a well-recognized cause of emphysema in adults; however, the natural history of this disorder in children is unclear. Because of the paucity of data in the paediatric age group, we performed whole body plethysmography, spirometry, and diffusing capacity, in a cohort of 17 homozygous (PiZZ phenotype) children (9 females, 8 males; mean age +/- SEM 13.4...
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