Mucus Inspissation Of Respiratory Tract

Clinical Features

Perlman et al. (1975) observed 2 sibs, with first-cousin parents, who had chronic obstructive airway disease from birth. Abnormal mucus of the respiratory tract seemed to be the primary defect. The clinical picture in some ways resembled that of cystic fibrosis (219700), Kartagener syndrome (244400), and alpha-1-antitrypsin deficiency (613490), but these disorders were excluded. The authors suggested that the 2 sisters reported by Cogswell et al. (1974) may have had the same condition.