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Diabetes In Infants: The Mystery of Monogenic Diabetes

One of the most difficult types of diabetes to treat is that which starts in infants.  Some forms are temporary while others appear like classical type one diabetes.  The temporary one is usually found in premature babies.  It was assumed to be due to the pancreas not being ready to produce enough insulin to deal with oral feedings.  This conclusion seemed reasonable for often these infants began dealing with their own blood sugars.  Since this happened, there was not much interest in following or studying these children. 
 
The second group developed diabetes a little later but certainly by 6 months of age.  The majority of these infants had great similarities to the diabetes developed by older children.  They were placed on insulin assumedly for the rest of their lives.  My bet is that the next thing that happened did so by mistake.  Do not know this for a fact; however, if I had to make up a story it would go this way:  A doctor in Europe got an adult with diabetes who had taken insulin for many years.  He had rather stable blood sugars with not so much insulin.  There had been so much talk about missed Adult Onset diabetes and this was, after all, an adult.  So the doctor put this previously thought to be type 1 diabetic on oral agents.  Rather than the expected loss of blood sugar control, the patient did better and stopped insulin altogether. 

This newborn type of diabetes has gained the name "Monogenic Diabetes."  It is caused by a defect that prevents the release of insulin from the pancreas.  It does not prevent the production--it is just not available to the rest of the body.  Since these children essentially make no insulin from birth, they must be diagnosed under 6 months if they have this "syndrome."  An oral antidiabetic pill made for Type 2 diabetes allows the pancreas to release the insulin.  Patients we have followed for 6 years now continue to not need insulin.  Even if the person has taken insulin for years, this treatment still works.  Remember, most have a firm diagnosis of diabetes by 2 months, definitly by 6 months.  People who "think" they had diabetes since birth but not actually were not firmly diagnosed undoubtedly do not have monogenic diabetes--sorry!
 
Contact us if you fit the criteria of diagnosis under 6 months and we will give you the information about how to be tested.

Author: Sam Wentworth, MD.

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