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CF-diabetes

Question
Does a very strong parncreatic insufficient CF patent will inevitably develop sometime a diabetes? Thus, is there a connection between a strong exocrine impairment and a strong endocrine impairment?
If yes, at what timepoint should one investigate a very strong pancreatic insufficient child concerning a diabetes?
Would a strong consumption of sugar support the occurrence of a diabetes?
Many thanks for your help.
Answer
Hello,
you ask if a CF patient with a very strong pancreatic insufficiency will develop inevitably a diabetes and from what timepoint on one should investigate a child with CF for diabetes. Furthermore you ask, if a high consumption of sugar will support the occurrence of a diabetes.
First of all it can be said in general, that about 80% of CF patients have already at birth a complete impairment of the pancreatic function. This is measured by an extreme low value for the pancreatic elastase I. A severity grade of the pancreatic insufficiency cannot be defined in these patients. However it is known, that pancreatic insufficent patients can respond very differently to the classic therapy with pancreatic enzymes. A number of factors influence the efficacy of the enzyme therapy. Here I will only enumerate some examples: velocity of the gut passage, problems in emptying the stomach, production of gastric acid, metabolism of the liver and bile, gut flora etc.
In general it can be said, that pancreatic insufficient patients are at risk, of developing a CF diabetes with advanced age. As the risk is in general only going to be a problem in the 2nd decade of life, there is the rule, that all CF patients over the age of 10 should receive an oral glucose tolerance test once per year. Of course in case of clinical suspicion (e.g. unclear weight loss or deterioration of the lung function) it has to be searched for a diabetic metabolism even before in the single case.
To the correlation of sugar intake and the development of a diabetes only general rules can be cited. From the nutritional guidelines for CF patients it can be concluded, that the calory need should be covered by fat and carbohydrates to 40% each and to 20% by proteins. One knows from nutritional studies of the general population, that an excessive consumption of carbohydrates and here especially sweets mostly in correlation with other supporting factors, increase the risk of development of a diabestes. Therefore, one should pay attention to a weighed composition of carbohydrates in the nutrition of CF patients. As a badly adjusted therapy of the pancreatic insufficiency puts the whole digestional system under stress, it should be in general paid attention to an optimal therapy with pancreatic enzymes. As it is for sure been proven, that via a cortisone therapy a diabetic metabolism can become manifest, it should be paid attention to the fact, that all cortisone preparations should be given only according to a clear indication and possibly as short-term as possible.

Best regards,
Dr. H.-G. Posselt
16.06.2015