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CYSTIC FIBROSIS-CREON & STOOL
ELASTASE
Dr.Vinod.P
Pancreatic insufficiency
• Most common gastrointestinal complication of
cystic fibrosis.
• Approximately 85 percent of patients at some
time in their lives.
• Fat malabsorption (Major consequence)
PANCREATIC SUFFICIENT
10-15%
PANCREATIC INSUFFICIENT
85-90%
CYSTIC FIBROSIS
PANCREATIC SUFFICIENT
Pancreatic function correlates strongly with
genotype in patients with CF
Cystic fibrosis creon & stool elastase
Pancreatic function tests
.
DIRECT PFT INDIRECT PFT
Lundth
Secretin
CCK
Stool elastase
Fecal fat
Serum trypsinogen
Fecal chymotrypsin
Pancreolauryl test
Breath tests
INDIRECT PANCREATIC FUNCTION
TESTS
• Simpler and easier to perform.
• Main role in diagnosis of advanced PEI since
they are much less sensitive than direct tests for
diagnosis of earlier stages of PEI.
• Serum trypsinogen- not sensitive for advanced
disease
• Fecal fat- steatorrhea implies a loss of greater
than 90 percent of normal enzyme secretory
output.
• Collection & processing is cumbersome
Fecal chymotrypsin and elastase-1
• Enzymatic products of pancreatic secretion that
remain relatively stable during transport
through the gastrointestinal tract.
Fecal chymotrypsin
• Using the secretin-CCK test as reference
standard, sensitivity is 85 % for advanced PEI,
but only 49% for mild and moderate PEI
• Affected during intestinal transport and may be
diluted in the presence of concomitant
diarrhea.
• Patients must stop exogenous enzymes for two
days prior to the collection.
Fecal elastase -1
• Pancreatic elastase-1 appears to be more stable
than chymotrypsin during intestinal transit.
• Exogenous enzymes do not interfere with the
elastase test assay as they do with the
chymotrypsin assay
• Using direct PFT as reference standard, fecal
elastase-1 has approximately 100 % sensitivity for
severe, 77 to 100 % for moderate, and 0 to 63
percent sensitivity for mild PEI .
• Specificity is approximately 93 percent .
• These studies suggest higher sensitivity and
specificity of fecal elastase compared with fecal
chymotrypsin.
Pancreatic elastase
• In addition to extending a protolytic activity, it
combines with bile acids and neutral steroids in
the intestinal lumen to transport cholesterol and
its metabolites along the intestinal tract.
• Possible because of the extraordinary stability of
this enzyme during its passage.
• Elastase concentration in stool is five times
higher than pancreatic juice, hence fecal elastase
concentration accurately reflects the amount of
enzyme secreted from the pancreas.
Age-All ages Range(Ugm elastase/gm stool)
NORMAL >200
MODERATE TO SLIGHT
ISUFFICIENCY
100-200
SEVERE
INSUFFICIENCY
<100
• In view of low sensitivity and sensitivity in early
disease results of fecal elastase testing should be
combined with clinical scenario.
PERT( Pancreatic enzyme replacement therapy)
• Multiple formulations of pancreatic enzymes
exist with different combinations of lipase,
protease, and amylase.
Different formulations
• PANCREAZE
• CREON
• ZENPEP
• ULTRESA
• VIOKACE
• PERTYE
CREON
LIPASE AMYLASE PROTEASE
Creon 3 3000 15000 9500
Creon 6 6000 30000 19000
Creon 12 12000 60000 38000
Creon 24 24000 120000 76000
Creon 36 36000 180000 11400
DOSING
• Weight based- <4yrs- 1000lipase units/kg/meal
>4yrs- 500lipase units/kg/meal
Dose can be increased up to 2500units/kg/meal
depending upon response.
• Fat based- For infants with known amount of
formula or NG feedings.
• 2000lipase units/120ml of formula or DBF
• Maximum lipase units per day not more than
10000/kg.
• Patients who fail to respond to maximal doses of
PERT- ranitidine or omeprazole.
• Higher pH for the entire product to be released.
• But limited evidence along with adverse effects.
Adequacy of PERT
• Elastase not useful as a measure to monitor the
effectiveness of PERT because it is a measure of
pancreatic function and not a measure of
malabsorption.
• Can check fecal fat
Adverse effects of PERT
• Prolonged contact of the enzyme supplements
with oral mucosa may cause ulcers, especially
with the powdered form.
• To prevent this complication, children should
learn to swallow capsules as early as possible.
Administration in younger children
• Open the capsules and microspheres should be
administered with food (eg, applesauce), even in
infants.
• The mouth should be inspected after eating and
rinsed with water, milk, or formula if necessary
to remove any beads clinging to the oral mucosa
• Fibrosing colonopathy- Limited evidence with
no established causal relation
• Maximum dose of 2500 lipase units/kg body
weight per meal (or less than 10,000
lipase units/kg body weight per day) is
recommended .
Ref
• Uptodate
THANK YOU

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Cystic fibrosis creon &amp; stool elastase

  • 1. CYSTIC FIBROSIS-CREON & STOOL ELASTASE Dr.Vinod.P
  • 2. Pancreatic insufficiency • Most common gastrointestinal complication of cystic fibrosis. • Approximately 85 percent of patients at some time in their lives. • Fat malabsorption (Major consequence)
  • 3. PANCREATIC SUFFICIENT 10-15% PANCREATIC INSUFFICIENT 85-90% CYSTIC FIBROSIS PANCREATIC SUFFICIENT Pancreatic function correlates strongly with genotype in patients with CF
  • 5. Pancreatic function tests . DIRECT PFT INDIRECT PFT Lundth Secretin CCK Stool elastase Fecal fat Serum trypsinogen Fecal chymotrypsin Pancreolauryl test Breath tests
  • 6. INDIRECT PANCREATIC FUNCTION TESTS • Simpler and easier to perform. • Main role in diagnosis of advanced PEI since they are much less sensitive than direct tests for diagnosis of earlier stages of PEI.
  • 7. • Serum trypsinogen- not sensitive for advanced disease • Fecal fat- steatorrhea implies a loss of greater than 90 percent of normal enzyme secretory output. • Collection & processing is cumbersome
  • 8. Fecal chymotrypsin and elastase-1 • Enzymatic products of pancreatic secretion that remain relatively stable during transport through the gastrointestinal tract.
  • 9. Fecal chymotrypsin • Using the secretin-CCK test as reference standard, sensitivity is 85 % for advanced PEI, but only 49% for mild and moderate PEI • Affected during intestinal transport and may be diluted in the presence of concomitant diarrhea. • Patients must stop exogenous enzymes for two days prior to the collection.
  • 10. Fecal elastase -1 • Pancreatic elastase-1 appears to be more stable than chymotrypsin during intestinal transit. • Exogenous enzymes do not interfere with the elastase test assay as they do with the chymotrypsin assay
  • 11. • Using direct PFT as reference standard, fecal elastase-1 has approximately 100 % sensitivity for severe, 77 to 100 % for moderate, and 0 to 63 percent sensitivity for mild PEI . • Specificity is approximately 93 percent . • These studies suggest higher sensitivity and specificity of fecal elastase compared with fecal chymotrypsin.
  • 12. Pancreatic elastase • In addition to extending a protolytic activity, it combines with bile acids and neutral steroids in the intestinal lumen to transport cholesterol and its metabolites along the intestinal tract. • Possible because of the extraordinary stability of this enzyme during its passage.
  • 13. • Elastase concentration in stool is five times higher than pancreatic juice, hence fecal elastase concentration accurately reflects the amount of enzyme secreted from the pancreas.
  • 14. Age-All ages Range(Ugm elastase/gm stool) NORMAL >200 MODERATE TO SLIGHT ISUFFICIENCY 100-200 SEVERE INSUFFICIENCY <100
  • 15. • In view of low sensitivity and sensitivity in early disease results of fecal elastase testing should be combined with clinical scenario.
  • 16. PERT( Pancreatic enzyme replacement therapy) • Multiple formulations of pancreatic enzymes exist with different combinations of lipase, protease, and amylase.
  • 17. Different formulations • PANCREAZE • CREON • ZENPEP • ULTRESA • VIOKACE • PERTYE
  • 18. CREON LIPASE AMYLASE PROTEASE Creon 3 3000 15000 9500 Creon 6 6000 30000 19000 Creon 12 12000 60000 38000 Creon 24 24000 120000 76000 Creon 36 36000 180000 11400
  • 19. DOSING • Weight based- <4yrs- 1000lipase units/kg/meal >4yrs- 500lipase units/kg/meal Dose can be increased up to 2500units/kg/meal depending upon response.
  • 20. • Fat based- For infants with known amount of formula or NG feedings. • 2000lipase units/120ml of formula or DBF • Maximum lipase units per day not more than 10000/kg.
  • 21. • Patients who fail to respond to maximal doses of PERT- ranitidine or omeprazole. • Higher pH for the entire product to be released. • But limited evidence along with adverse effects.
  • 22. Adequacy of PERT • Elastase not useful as a measure to monitor the effectiveness of PERT because it is a measure of pancreatic function and not a measure of malabsorption. • Can check fecal fat
  • 23. Adverse effects of PERT • Prolonged contact of the enzyme supplements with oral mucosa may cause ulcers, especially with the powdered form. • To prevent this complication, children should learn to swallow capsules as early as possible.
  • 24. Administration in younger children • Open the capsules and microspheres should be administered with food (eg, applesauce), even in infants. • The mouth should be inspected after eating and rinsed with water, milk, or formula if necessary to remove any beads clinging to the oral mucosa
  • 25. • Fibrosing colonopathy- Limited evidence with no established causal relation • Maximum dose of 2500 lipase units/kg body weight per meal (or less than 10,000 lipase units/kg body weight per day) is recommended .

Editor's Notes

  1.  This genotype association is stronger for the pancreatic insufficiency phenotype, and much weaker for pulmonary disease phenotype. 
  2. The use of H2RAs is limited by the development of tachyphylaxis, and the use of PPIs is limited by potential adverse effects, including possibly increased risks for vitamin B12 deficiency and bone health