My six year old cousin was just diagnosed with MODY. Can he be managed by oral drugs? If yes, which ones can be used?

I am assuming that your son’s doctor is using the term MODY (Maturity Onset Diabetes in the Young) in its broadest sense of non-insulin dependent diabetes mellitus (NIDDM) in children. This involves a number of different types of diabetes including a very small number with a specific inherited inborn error metabolism of which the most common is deficiency of the enzyme hexokinase. As you might imagine, these require very special laboratory facilities to diagnose, but nowadays, the term MODY tends to be used exclusively for this subgroup.

At this time, probably the most common form of NIDDM in a six year old living in Pakistan would be type 2 diabetes, which is a form of insulin resistance most commonly seen in middle aged adults. Its incidence has however been increasing rather dramatically in the younger age groups all over the world. It seems to be linked to lack of physical exercise and to obesity from a high calorie, high fat diet. There is often a family history of type 2 diabetes.

The initial treatment here in the USA is with exercise and diet. The latter needs to applied with care in a growing child. If it is possible, I would hope that you might get help with this from a paediatric dietitian. If hypoglycemic drugs are needed the one most commonly used is Glucophage [metformin], and, in the unlikely event that another is needed for good control, the choice would be a sulfonylurea. A very few centers are using the new “glitazone” (thiazolidinedione) drugs like pioglitazone, but not as a first choice.

Finally, you might have this child’s parents ask the doctor about getting a GAD test done because a scattering of these cases are in fact an autoimmune variant, and therefore more likely to need insulin in the future.

DOB