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

Adequate Response to PERT

Clinical Improvement or Resolution of Symptoms suggestive of EPI

Short Term (≤ 72 hours) Long Term (> 72 hours)
  • Resolution of
    • Steatorrhea
    • Rectal prolapse
  • Decreased
    • Frequency of stools after meals/snacks
    • Malodorous stools
    • Flatulence
    • Abdominal pain
    • Voracious appetite
  • Infants
    • Within 1 week resume/maintain median weight gain for age
      • Age Median g/day
          Females Males
        0-4 wks 29 34
        4-8 wks 34 40
        2-3 mos 24 27
        3-4 mos 20 21
        4-5 mos 16 17
        5-6 mos 13 14
        6-8 mos 11 11
        8-10 mos 9 9
        10-12 mos 8 8
        12-18 mos 7 7
        18-24 mos 7 6
  • Children
    • Within 1 month resume/maintain median weight gain for age
      • Age Median g/day
          Females Males
        2-3 yrs 6 5.5
        3-4 yrs 5.2 5.5
        4-5 yrs 4.7 5.5
        5-6 yrs 5 5.5
        6-7 yrs 6.3 6
        7-8 yrs 8.2 6.6
        8-9 yrs 10 7.7
        9-10 yrs 11 9
        10-11 yrs 12.3 10.7
        11-12 yrs 12.3 12.3
        12-13 yrs 12.6 14.2
        13-14 yrs 11.5 16
        14-15 yrs 9.3 16.2
        15-16 yrs 6 15
  • Adults
    • Sustained improvement in symptoms and nutritional parameters
      • (e.g., fat-soluble vitamin or essential fatty acid status, anthropometrics)
      • Note: due to other comorbidities, adults may not reach “normal” values
Charts in right column adapted from the WHO Child Growth Standards July 2010 and CDC Data Tables