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
Improved consistence and frequency of stools Sustained improvement in symptoms and nutritional parameters
  • (Ex: fat-soluble vitamin or essential fatty acid status, anthropometrics)
  • Note: due to other comorbidities, adults may not reach “normal” values

 

Short Term (≤ 72 hours) Long Term (> 72 hours)
  • Improved consistency and frequency of stools
  • 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 weeks 29 34
        4-8 weeks 34 40
        2-3 months 24 27
        3-4 months 20 21
        4-5 months 16 17
        5-6 months 13 14
        6-8 months 11 11
        8-10 months 9 9
        10-12 months 8 8
        12-18 months 7 7
        18-24 months 7 6
  • Children
    • Within 1 month resume/maintain median weight gain for age
      • Age Median g/day
          Females Males
        2-3 years 6 5.5
        3-4 years 5.2 5.5
        4-5 years 4.7 5.5
        5-6 years 5 5.5
        6-7 years 6.3 6
        7-8 years 8.2 6.6
        8-9 years 10 7.7
        9-10 years 11 9
        10-11 years 12.3 10.7
        11-12 years 12.3 12.3
        12-13 years 12.6 14.2
        13-14 years 11.5 16
        14-15 years 9.3 16.2
        15-16 years 6 15
  • Adults
    • Sustained improvement in symptoms and nutritional parameters
      • (Ex: fat-soluble vitamin or essential fatty acid status, anthropometrics)
      • Note: due to other comorbidities, adults may not reach “normal” values

  • Weight Gain Velocity Chart
(reference charts at right adapted from the WHO Child Growth Standards July 2010 and CDC Data Tables)

Inadequate Response to PERT

  • Symptoms of malabsorption
  • Poor weight gain or linear growth
  • Low fat soluble vitamin levels despite proper adherence to vitamin regimen
  • Abdominal pain or distention associated with eating