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Cystic fibrosis creon & stool elastase

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cystic fibrosis- The role of pancreatic enzymew replacement therapy and stool elastase

Publié dans : Sciences
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Cystic fibrosis creon & stool elastase

  2. 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. 3. PANCREATIC SUFFICIENT 10-15% PANCREATIC INSUFFICIENT 85-90% CYSTIC FIBROSIS PANCREATIC SUFFICIENT Pancreatic function correlates strongly with genotype in patients with CF
  4. 4. Pancreatic function tests . DIRECT PFT INDIRECT PFT Lundth Secretin CCK Stool elastase Fecal fat Serum trypsinogen Fecal chymotrypsin Pancreolauryl test Breath tests
  5. 5. 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.
  6. 6. • 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
  7. 7. Fecal chymotrypsin and elastase-1 • Enzymatic products of pancreatic secretion that remain relatively stable during transport through the gastrointestinal tract.
  8. 8. 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.
  9. 9. 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
  10. 10. • 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.
  11. 11. 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.
  12. 12. • 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.
  13. 13. Age-All ages Range(Ugm elastase/gm stool) NORMAL >200 MODERATE TO SLIGHT ISUFFICIENCY 100-200 SEVERE INSUFFICIENCY <100
  14. 14. • In view of low sensitivity and sensitivity in early disease results of fecal elastase testing should be combined with clinical scenario.
  15. 15. PERT( Pancreatic enzyme replacement therapy) • Multiple formulations of pancreatic enzymes exist with different combinations of lipase, protease, and amylase.
  16. 16. Different formulations • PANCREAZE • CREON • ZENPEP • ULTRESA • VIOKACE • PERTYE
  17. 17. 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
  18. 18. 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.
  19. 19. • 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.
  20. 20. • 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.
  21. 21. 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
  22. 22. 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.
  23. 23. 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
  24. 24. • 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 .
  25. 25. Ref • Uptodate
  26. 26. THANK YOU