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This genotype association is stronger for the pancreatic insufficiency phenotype, and much weaker for pulmonary disease phenotype.
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
• Most common gastrointestinal complication of
• Approximately 85 percent of patients at some
time in their lives.
• Fat malabsorption (Major consequence)
Pancreatic function correlates strongly with
genotype in patients with CF
Pancreatic function tests
DIRECT PFT INDIRECT PFT
INDIRECT PANCREATIC FUNCTION
• 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
• Fecal fat- steatorrhea implies a loss of greater
than 90 percent of normal enzyme secretory
• Collection & processing is cumbersome
Fecal chymotrypsin and elastase-1
• Enzymatic products of pancreatic secretion that
remain relatively stable during transport
through the gastrointestinal tract.
• 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
• 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
• 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
• 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)
MODERATE TO SLIGHT
• 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.
• 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
• 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
• 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
• 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