CLINICAL SYNDROMES ASSOCIATED WITH DEFICIENT FIBRINOLYTIC ACTIVITY OF THE LUNG
II. Cystic Fibrosis of the Pancreas
Jack Lieberman M.D.1
1 Department of Medicine, University of California Medical Center, Los Angeles, and the Los Angeles County Harbor General Hospital, Torrance, California
Plasminogen-activator activity in lung was investigated in 211 controls and 8 children with fibrocystic disease of the pancreas. Twenty-seven of the 211 controls lacked enzyme activity of the whole lung homogenate, while 7 out of the 8 specimens from children with fibrocystic disease lacked activity. The defect in all 7 of the specimens from patients with fibrocystic disease was shown to be due to excessive inhibition, and the inhibitor could easily be removed by washing the lung sediment with normal saline solution. Eight of the controls showed a similar pattern of enzyme inhibition.
A new hypothesis as to the pathogenesis of fibrocystic disease is proposed which postulates the existence of an abnormal protease inhibitor in both the lungs and pancreas. The manner in which such an enzyme defect could account for the manifestations of the disease, and the possible role of a proteolytic enzyme in mucoprotein synthesis are discussed.
Submitted on June 16, 1959
Accepted on September 23, 1959
