Changes in the CA 19‐9 antigen and lewis blood group with pulmonary disease severity in cystic fibrosis

Abstract

The altered carbohydrate structure of sputum from patients with cystic fibrosis (CF) has been thought to be due to the inflammatory airway response. Carcinoembryonic antigen (CEA) and CA 19-9 detect sialosylated carbohydrates in mucus. The epitope of CA 19-9 is part of the Lewis A (Le(a)) blood group antigen. Serum concentrations of CEA and CA 19-9 were determined by radioimmunoassay in 41 CF patients, aged 6-34 years; 16 were asymptomatic Outpatients, and 25 had been admitted for pulmonary exacerbations. There was no difference in CEA between groups. The CA 19-9 serum concentration was elevated in 90% of patients who had at least one of the two Lewis antigens. The CA 19-9 concentration of Inpatients with exacerbations was 2.7 times that of stable Outpatients (263 +/- 44 versus 99 +/- 13 U/mL; P less than 0.02). CA 19-9 correlated significantly with age (r = 0.35, P less than 0.05), Brasfield score (r = 0.39, P less than 0.015), pulmonary function tests, cough severity (r = 0.50, P less than 0.001) and NIH clinical score (r = 0.57, P less than 0.001). CA 19-9 concentration of Inpatients decreased by 44% from admission to discharge (302 +/- 45 to 169 +/- 39, P less than 0.02). Fourteen of 25 (56%) of the Inpatients were Le(a) positive versus only 3/15 (20%) of Outpatients who had milder lung disease (P less than 0.002). Of the Inpatients, 25% with more advanced lung disease were Le(a+b+), a rare blood group in the normal population, and one not observed in the Outpatients with milder disease.(ABSTRACT TRUNCATED AT 250 WORDS)

Infants and Young Children with Cystic Fibrosis Have High Levels of Serum Sialyl Lewisa Antigen

“…Other investigators have confirmed our findings (8). The present study extends these observations by demonstrating that even very young CF patients have elevated sialyl Le" levcls compared with serum antigen levels of normal babies or infants and young children who have lung diseases.…”

“…First, serum mucin from a single CF patient most resembled human lung mucin with respect to carbohydrate and amino acid composition (5). Second, serum sialyl Lea antigen levels correlated with measures of lung disease in two studies of groups of CF patients (7,8). Third, of 39 CF patients in the present study, 22 had some evidence of lung disease when their blood was drawn.…”

“…Sialyl Lea antigen may be used as a tumor marker (6). Sialyl Lea antigen (or mucin) levels in serum are related to the age, clinical status, and pulmonary status of CF patients (7,8). The purpose of the present study was to compare serum antigen levels of healthy newborns or infants and young children who had lung diseases (e.g.…”

“…Therefore, absolute levels of antigen cannot be directly correlated with disease status in individual patients. By population studies and longitudinal studies of individual patients, however, antigen levels have been correlated with recurrence or progression of tumors in patients with mucin-secreting adenocarcinomas and with age, clinical status, and pulmonary status of patients with CF (7,8).…”

Serum CA 19?9 levels as a diagnostic marker in cystic fibrosis patients with borderline sweat tests

“…antigen was originally used as a tumor marker in gastrointestinal malignancies. Increased serum CA 19-9 values have also been observed in CF patients [8][9][10], especially in those with progressive pulmonary disease [11]. However, it is not clear whether patients with mild disease and a borderline sweat test also have high levels.…”