1. Use of monoclonal faecal elastase-1 concentration for pancreatic status assessment in cystic fibrosis patients.
- Author
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Gonzales AC, Vieira SM, Maurer RL, Silva FA, and Silveira TR
- Subjects
- Adolescent, Child, Child, Preschool, Cystic Fibrosis genetics, Epidemiologic Methods, Exocrine Pancreatic Insufficiency enzymology, Female, Heterozygote, Homozygote, Humans, Infant, Male, Mutation, Pancreatic Elastase genetics, Reference Values, Cystic Fibrosis enzymology, Exocrine Pancreatic Insufficiency diagnosis, Feces enzymology, Pancreatic Elastase analysis
- Abstract
Objective: To assess the concentration of faecal elastase-1 (EL-1) in pediatric patients with cystic fibrosis with mutation DeltaF508., Methods: Cross-sectional study with samples collected consecutively from 51 patients aged 4 months to 17 years old (mean 9.11±4.74); 32 (62.8%) patients were male. Clinical-demographic data were collected, as well as data on the type of mutation. Exocrine pancreatic insufficiency was established by the activity of faecal EL-1 < 200 µg/g. EL-1 was quantified through the monoclonal ELISA method (ScheBo Biotech AG, Germany). Pancreatic supplements were used in 46 (90.2%) patients., Results: Forty-one (80.4%) patients presented with pancreatic insufficiency (EL-1 fecal < 100 µg/g): 17 (41.5%) were homozygous, 14 were heterozygous (34.1%) and 10 were non-DeltaF508 (24.4%). Regarding the mutation, there was a statistically significant association of homozygosity with faecal EL-1 concentration < 100 µg/g (p = 0.010). All patients considered to be pancreatic insufficient (n = 41) by the test were using pancreatic supplements. Ten (19.6%) presented faecal EL-1 > 200 µg/g, and 5/10 (50%) used enzymes., Conclusions: The activity of faecal EL-1 < 100 µg/g, indicating pancreatic insufficiency, was observed in 17/17 (100%) of homozygous patients, as expected, and was less frequent in patients who were heterozygous for DeltaF508 and in patients without the mutation. There was no association of faecal EL-1 concentration with age and sex of patients. The test was standardized, is easy to execute, and can be used to assess the pancreatic status of patients with cystic fibrosis.
- Published
- 2011
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