• Newborn with family history of cystic fibrosis who has presentation consistent with meconium ileus (failure to pass meconium, bilious vomiting, inspissated tar-like meconium, etc.), and therefore should be treated with pancreatic enzyme
• Key idea: Cystic fibrosis leads to fat malabsorption because pancreatic enzymes have trouble reaching the GI tract, so patients can benefit from pancreatic enzyme

https://step-prep.org

The majority of infants with CF and MI, whether simple or complex, have pancreatic insufficiency (PI). Confirmation of PI is most efficiently and effectively done by obtaining a fecal elastase. However, the fecal elastase sample should be collected from rectally delivered, formed stools and not from an enterostomy. Watery stools from either an enterostomy or rectally delivered can result in falsely low fecal elastase values. Therefore, in infants with MI and an enterostomy, PI should be assumed and fecal elastase collected at a later date after the gastrointestinal tract is back in continuity and stools are at least of a pasty consistency to confirm diagnosis. Once the infant is able to take a minimal amount of formula or breastmilk by mouth or feeding tube, PERT should be initiated at 2000–4000 lipase units per 120 mL of formula [[37]]. PERT contains lipase to digest to lipids, amylase to digest carbohydrates, and protease

https://www.cysticfibrosisjournal.com/article/S1569-1993(17)30809-3/fulltext#secst0030