Clinical Pathway for Pancreatic Enzyme Replacement Therapy (PERT) in Children with Suspected or Confirmed Exocrine Pancreatic Insufficiency (EPI)

Child with Confirmed EPI
  • Symptoms Suggestive of EPI
    • Poor weight gain/linear growth
    • Steatorrhea
    • Loose/frequent stools after meals
      or snacks
    • Foul smelling stools
    • Flatulence
    • Abdominal pain associated with eating
    • Voracious appetite
    • Rectal prolapse
Team Assessment
  • History and Physical Exam
    • Assess prenatal testing, newborn screen, sweat test
    • Feeding history, growth pattern
    • Surgical history suggestive for acquired EPI
    • Conditions with high risk for EPI
    • Symptoms of fat malabsorption
    • Previous enzyme replacement therapy
  • Child presents with any of the following:
    • 2 positive PI CF variants
    • Meconium ileus
    • Rectal prolapse
    • Poor weight gain
    • Steatorrhea
Initiate PERT at First Visit

Formulations | Dosing Recommendations | Examples

Do not initiate PERT
Screen for Exocrine Pancreatic Insufficiency
  • Obtain Fecal Elastase
    • Requires a single spot stool sample
    • Perform on solid stool
    • Results inaccurate if:
      • Stool sample is loose/watery
      • Test is performed in term infants < 72 hrs of age or preterm infants < 2 wks of age
  • Low result must be confirmed with repeat testing
Normal Fecal Elastase
> 500 mcg/g
Borderline Fecal Elastase
200-500 mcg/g
Abnormal Fecal Elastase
< 200 mcg/g
  • PERT not indicated; discontinue if previously initiated
  • Consider subspecialist for evaluation
    • Pulmonary/CF Center
    • GI
  • Repeat fecal elastase to confirm diagnosis
  • Refer to subspecialist for evaluation
    • Pulmonary/CF Center
    • GI
  • Refer to clinical nutrition as indicated
  • Consider RUQ ultrasound
  • Additional testing by subspecialist
  • Consider PERT while awaiting
    subspecialist evaluation
  • Repeat fecal elastase unless genetic variants consistent with CF and EPI
  • Refer to subspecialist for evaluation
    • Pulmonary/CF Center
    • GI
  • Refer to clinical nutrition as indicated
  • Consider RUQ ultrasound
  • Additional testing by subspecialist
  • Monitor
    • Growth
    • Fat malabsorption
  • Monitor
    • Growth
    • Fat malabsorption
    • Repeat fecal elastase in 1 yr or sooner if clinical symptoms
  • Monitor
    • Growth
    • Fat malabsorption
    • Annually as clinically indicated
      • Fecal elastase
      • Fat-soluble vitamin levels
        • Vitamin A
        • 25-OH vitamin D
        • Vitamin E
        • PT/INR as a measure of vitamin K
Consider Titrating PERT Dose
Refer to clinical nutrition as indicated
Posted: March 2019
Last Revised: April 2024
Editors: Clinical Pathways Team