Abstract
In cystic fibrosis (CF), mucus plugging in the airways and in the gastrointestinal tract leads to severe morbidity and mortality. The mucin‐associated antigens CA 19–9 and CA 125 are markers of gastrointestinal malignancy, and CA 19–9 has also been reported in association with pulmonary function in CF. Aim: To test whether these antigens might serve as markers for the severity of pulmonary and gastrointestinal disease in CF. Methods: In 99 patients, aged 1 to 48 y, serum levels of CA 19–9 and CA 125 were measured by RIA and ELISA and related to clinical data. Results: Patients with severe mutations had significantly increased serum levels of CA 125, indicating an association with a more severe CF phenotype. This was further supported by the association with lung function, chronic pulmonary colonization of Pseudomonas aeruginosa and pancreatic insufficiency. CA 19–9 was also shown to be associated with lung function and Ps. aeruginosa colonization. No gastrointestinal malignancy was found in our patients despite very high values of CA 19–9 in some patients. During a 5‐y follow‐up, the very high serum levels of CA 19–9 decreased along with improved general condition of the patients. Conclusion: Increased serum levels of CA 125 in CF patients were associated with severe cystic fibrosis transmembrane conductance regulator mutations and a severe phenotype. Both antigens were associated with pseudomonas colonization and lung function and CA 125 also with pancreatic insufficiency. The estimates of CA 19–9 are hampered by the influence of the Lewis histo‐blood group system on the synthesis of CA 19–9.
Published Version
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