Initiating Pancreatic Enzyme Replacement Therapy (PERT) — Inadequate Response to PERT — Clinical Pathway: All Settings

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

Inadequate Response to PERT

No change or minimal improvement in symptoms suggestive of EPI

Short Term (≤ 72 hours) Long Term (> 72 hours)
  • No change in consistency or frequency of stools
  • Persistent
    • Steatorrhea
    • Rectal prolapse
    • Frequency of stools after meals/snacks
    • Malodorous stools
    • Flatulence
    • Abdominal pain
    • Voracious appetite
  • Infants
    • Within 1 week suboptimal weight gain for age (below the median)
      • 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 suboptimal weight gain for age (below the median)
      • 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
    • Persistent symptoms or unchanged nutritional parameters
      • (e.g., fat-soluble vitamin or essential fatty acid status, anthropometrics)
Charts in right column adapted from the WHO Child Growth Standards July 2010 and CDC Data Tables