Evolution of pancreatic function during the first year in infants with cystic fibrosis
Department of Medicine, Division of Preventive and Behavorial Medicine; Department of Pediatrics
Cohort Studies; Cystic Fibrosis; Docosahexaenoic Acids; Exocrine Pancreatic Insufficiency; Feces; Female; Genotype; Homozygote; Humans; Infant; Infant, Newborn; Longitudinal Studies; Male; Neonatal Screening; Pancreatic Elastase; Pancreatic Function Tests
Congenital, Hereditary, and Neonatal Diseases and Abnormalities | Pediatrics | Respiratory Tract Diseases
OBJECTIVE: To describe pancreatic function during the first year of life in infants diagnosed with cystic fibrosis (CF) using serial fecal elastase measurements.
STUDY DESIGN: This was a longitudinal study of 82 infants diagnosed with CF through newborn screening. Monthly stool samples were sent to a central laboratory for fecal elastase measurements.
RESULTS: A total of 61 infants had an initial stool sample obtained at age 9 months. Twenty-six of 29 infants with a fecal elastase value /g at study entry had a fecal elastase value /g (the accepted cutoff value for pancreatic insufficiency) on all measurements during the year; all 29 had a value /g at the end of the study. Of the 48 infants with initial fecal elastase value /g, 13 had at least 1 fecal elastase value >200 mug/g but had a final stool fecal elastase value /g; however, 4 infants with an initial fecal elastase value /g ended the year with a value >200 mug/g. Eleven of 13 infants with an initial fecal elastase value of >200 mug/g still had a value >200 mug/g at the end of the first year.
CONCLUSION: Infants with CF exhibit variability in fecal elastase values during the first year. Infants with a fecal elastase level of 50-200 mug/g at diagnosis should be treated with pancreatic enzyme replacement therapy, but fecal elastase should be remeasured at age 1 year to ensure that those with a falsely low value do not continue to receive pancreatic enzyme replacement therapy unnecessarily. Those with a fecal elastase value >200 mug/g initially can become pancreatic insufficient with time.
DOI of Published Version
J Pediatr. 2013 Apr;162(4):808-812.e1. doi: 10.1016/j.jpeds.2012.10.008. Link to article on publisher's site
The Journal of pediatrics
O'Sullivan, Brian P.; Baker, Dawn; Leung, Katherine G.; Reed, George W.; Baker, Susan S.; and Borowitz, Drucy, "Evolution of pancreatic function during the first year in infants with cystic fibrosis" (2013). University of Massachusetts Medical School Faculty Publications. 116.