title: Use of monoclonal faecal elastase-1 concentration for pancreatic status assessment in cystic fibrosis patients.
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.
authors.affiliation: Universidade Federal do Rio Grande do Sul, Rua Ramiro Barcellos 2350, Porto Alegre, RS, Brazil. andreacgonzales@yahoo.com.br
authors.forename: Andréa C S
authors.initials: AC
authors.lastname: Gonzales
authors.forename: Sandra M G
authors.initials: SM
authors.lastname: Vieira
authors.forename: Rafael L
authors.initials: RL
authors.lastname: Maurer
authors.forename: Fernando A A e
authors.initials: FA
authors.lastname: Silva
authors.forename: Themis R
authors.initials: TR
authors.lastname: Silveira
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003550:Cystic Fibrosis / Q000201:enzymology* / Q000235:genetics; D004812:Epidemiologic Methods; D010188:Exocrine Pancreatic Insufficiency / Q000175:diagnosis* / Q000201:enzymology; D005243:Feces / Q000201:enzymology*; D005260:Female; D006579:Heterozygote; D006720:Homozygote; D006801:Humans; D007223:Infant; D008297:Male; D009154:Mutation; D010196:Pancreatic Elastase / Q000032:analysis* / Q000235:genetics; D012016:Reference Values
chemical_list: D010196:Pancreatic Elastase