The family lives in the district of Eksjo, SmAland. The pedigree is seen in fig. 1. Clinical and laboratory data on the propositus (11, in fig. 1, case 3 in case reports) have been given in brief previously (12). A brother (11, in fig. 1, case 1 in case reports) was treated at his local hospital for emphysema. Clinical data on this patient were obtained from the archives of that hospital and from his relatives. The patient died at home in 1954, ie before the present investigation was started. This explains the lack of electrophoretic data. The propositus (11,) and his sister (11, in fig. 1, case 2 in case reports) were repeatedly admitted to the department of chest diseases of their