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Frequency of ASCA seropositivity in children with cystic fibrosis
Adria A Condino1, Edward J Hoffenberg, Frank Accurso
1The Children's Hospital, University of Colorado Health Sciences Center, Department of Pediatrics, Denver, USA.
Insights
Pediatric cystic fibrosis (CF) patients show a higher frequency of anti-Saccharomyces cerevisiae antibodies (ASCA) seropositivity compared to the general population. This finding suggests ASCA should be used cautiously when assessing CF patients for Crohn disease.
Area of Science:
- Immunology
- Pediatric Gastroenterology
- Pulmonology
Background:
- Cystic Fibrosis (CF) is a genetic disorder affecting multiple organs.
- Anti-Saccharomyces cerevisiae antibodies (ASCA) are associated with inflammatory bowel diseases, particularly Crohn disease.
- The prevalence of ASCA in pediatric CF patients is not well-established.
Purpose of the Study:
- To determine the frequency of ASCA seropositivity in pediatric patients with CF.
- To investigate the correlation between ASCA seropositivity and clinical features in CF patients.
Main Methods:
- Prospective study of 82 pediatric CF patients (ages 2-21).
- ASCA (IgA and IgG) levels measured using enzyme-linked immunosorbent assay.
- Clinical data collected from the CF Foundation database.
Main Results:
- 17 (20.7%) of 82 CF patients were ASCA seropositive.
- ASCA seropositivity was significantly higher in CF patients than the general population (20.7% vs. 4%, P < 0.0001).
- ASCA-positive patients frequently had DeltaF508 mutation (82.4%) and positive fungal sputum cultures (52.9%).
Conclusions:
- Pediatric CF patients exhibit a higher frequency of ASCA seropositivity.
- ASCA should be interpreted with caution in CF patients evaluated for Crohn disease.
- Increased ASCA may be linked to fungal organism exposure in CF patients.
Objective:
To determine the frequency of anti-Saccharomyces cerevisiae antibodies (ASCA) seropositivity in pediatric patients with cystic fibrosis (CF) and correlate ASCA with clinical features.
Methods:
Prospective study of children with CF aged 2 to 21 years enrolled from The Children's Hospital Cystic Fibrosis Center. Exclusion criteria included Crohn disease, immunodeficiency or immunoglobulin A deficiency. The frequency of ASCA (ASCA immunoglobulin A and immunoglobulin G) was measured by an enzyme-linked immunosorbent assay kit provided by Inova, Inc. (San Diego, CA). The CF Foundation database was queried for clinical data on ASCA seropositive patients.
Results:
Seventeen (20.7%) of 82 patients were seropositive for ASCA. Of these, eight had immunoglobulin A antibodies, six had immunoglobulin G antibodies and three had both. ASCA seropositivity in CF patients was significantly greater than the general population (20.7% versus 4%, P < 0.0001), using an exact binomial test on a single proportion. The 95% confidence limits around our observed proportion were 12.6% to 31.1%. Of 17 ASCA positive patients, 1 (5.8%) had a history of meconium ileus, 14 (82.4%) had DeltaF508 gene mutation, 9 (52.9%) had positive sputum cultures for fungal organisms, and 17 (100%) had an ideal body weight % >or=85%.
Conclusion:
Patients with CF have a higher frequency of ASCA seropositivity than the general population. When evaluating CF patients for Crohn disease, ASCA should be used with caution. The reasons for higher ASCA seropositivity in CF patients are unknown, but may include exposure to fungal organisms via intestinal or pulmonary sources.
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