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Pancreatic insufficiency without steatorrhea or other symptom

Question
Hello, my child was recently diagnosed with CF and pancreatic insufficiency (pancreatase < 50) at age 12. He used to exhibit the symptoms described for pancreatic insufficiency (fatty and many stools, smelly flatulence, tummy aches etc) to some degree until four years ago, but upon regular administration of probiotics for two years followed by a change in diet (gluten-free) all symptoms disappeared. We were now prescribed Kreon and I am wondering what this treatment will actually achieve in my child?
Answer
Dear questioner,
In this case, it would be interesting to know, how the CF- was diagnosed: did your child have a markedly positive sweat test, or did it have a genetic testing for CF mutations? Does the clinical picture and the results of the investigations hint at a “classical form” of CF with pancreatic insufficiency, lung problems, failure to thrive, the stool pattern you described…..? This form requires in general an enzyme replacement with Kreon. Besides this classical form, other froms of CF exist, with a milder course and pancreatic sufficiency.
Nevertheless, independently of this, one can test the pancreatic function. There are indirect pancreatic function tests which determine the amount of pancreatic enzymes in the stool, like chymotrysin and particularly faecal pancreatic elastase 1 (this is probably the pancreatase you mentioned). Values of this elastase 1 < 100 μg /g stool are markers of a severe pancreatic insufficiency. However, the gold-standard to test the pancreatic function is the measurement of the fat excretion over 3 days and its relation to dietary fat intake over the same time period. Normally the equivalent of less than 7% of the fat ingested is excreted i.e. there is over 93% fat absorption. Therefore, if there is any doubt, this investigation should also be done and if it is positive, there is no doubt about a necessary therapy with Kreon (replacement of pancreatic enzymes), independently of visible symptoms. Then it is proven, that your child cannot take enough fat from the food, is losing fat soluble vitamins, malnourishment and vitamin deficiency will be the consequence if lacking enzymes are not replaced.
I agree with you that it is striking, that the symptoms of your child went away some time ago; you mentioned a gluten-free diet. It has to be said, that it is of course possible, that your child suffers from CF and a gluten-allergy (so-called celiac disease) and that parts of the belly symptoms went away due to the beginning of a gluten-free nutrition. However, if CF has for sure been diagnosed and the tests of the pancreatic function unambiguously show an insufficiency, an enzyme substitution with Kreon is mandatory in order to achieve normal thriving and avoid deficiency states.
Yours sincerely,
Dr. Daniela d’Alquen (coordinator of the central English Archive of ECORN-CF)
17.03.2014