ABNORMAL SERUM FACTOR IN PATIENTS WITH CYSTIC FIBROSIS OF PANCREAS

Serum from patients with cystic fibrosis of the pancreas (CFP) disorganizes the ciliary rhythm in explants of respiratory epithelium. This serum factor was present in all 75 patients with CFP and absent from the serum of 75 other patients having a variety of diseases with similar clinical features. A bio-assay method to detect the disorganized ciliary beat was performed using cultures of rabbit tracheal mucosa. The serum factor is heat labile, nondialys-able, and precipitated with the euglobulins. Chromatographic examination of serum from a patient with CFP showed that the serum factor was eluted with the macroglobulin fraction as well as between the gamma globulin and albumin peaks. The serum factor was also detected but as a lower concentration in the parents of children with cystic fibrosis; The native sera from parents produced a disorganized beat in 7 of 25 cases; concentrating the euglobulin fraction 2.5:1, permitted detection in the remaining 18 sera. Similar concentration of sera from control patients resulted in only 1 positive reaction in the 25 sera tested. Inaddition, a 10-fold concentration of euglobulin from normal controls failed to disorganize the ciliary beat [Pedigree of family with CFP given].