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From Delaware, USA:

A year ago, our 5 year old daughter was diagnosed with Type 1 diabetes as a result of a glucose tolerance test (she was made to eat a large quantity of sweets and then checked for blood glucose). Her blood glucose was about 250 mg/dl one hour after eating. However, when the blood tests came back months later, her insulin level was 94 units and the GAD antibodies came back negative. She was on decreasing levels of insulin from a year ago to a month later and has not needed it since. We are currently seeking a second opinion, still waiting on the blood work to come back, and one thing mentioned was the possibility of MODY. Never having heard of this, I am looking for more info on the condition (how it was originally discovered, diagnosis, treatment, etc.) I am hoping you could help.


I am assuming that your query about MODY (Maturity Onset Diabetes in the Young) is linked to some doubts about the original diagnosis of Type 1 Diabetes. First of all, by far the most probable cause of diabetes at this age is autoimmune or Type 1 Diabetes. The apparent need for insulin in the beginning (albeit for a rather short time and the single abnormal blood sugar) support this. At the same time it would be important to know whether this diagnostic blood sugar was measured in a clinical laboratory or simply with a portable meter and whether there was glucosuria or ketonuria at the same time, and finally whether the single abnormal result was confirmed on other occasions.

On the other hand, the insulin level was within normal limits although you don't say whether this was reported in uU/ml or pmols/l. The negative antibody test is not necessarily against Type 1 Diabetes. About 10% of Caucasian children with new onset diabetes are negative and over half of Hispanic and African American children are and the exact nature of this diabetes is not yet understood. It is also customary to test not only for anti-GAD; but for islet cell antibodies (ICA512) and anti insulin antibodies (IAA). The reason for this is that only one or two of this triad may be positive. If the original sample still exists, it might be worth discussing with your doctor whether the sample could be retested for the remaining antibodies. IAA cannot be tested though if insulin has already been given for more than a week or two.

At the moment, three kinds of MODY are recognised. They are all linked to abnormalities on specific chromosomes. The diabetes is usually mild and is not recognised clinically until a later age and because of dominant inheritance there is nearly always a striking family history. Except in MODY3, insulin is for the most part not needed. The commonest form MODY2 represent a deficiency of the enzyme Glucokinase and several variants are recognised. MODY1 and MODY3 are deficiencies of proteins called 'Hepatocyte Nuclear Factors'. Treatment is by diet and exercise and sometimes with hypoglycemic agents orally. There is a substantial literature on these forms of diabetes which is well beyond the scope of a single e-mail; but you could access this information by going to your nearest medical library.

There are other rare forms of diabetes that occur in children which are called mitochondrial diabetes; but they are nearly always associated with other problems such as deafness, muscle weakness and so on.

One problem with all of these rare forms of diabetes is that the specific diagnosis can only be made in research laboratories so that a final decision may take time. In the meantime, though, I would suggest that you have the means of testing urine and blood sugars at home and that you do it occasionally in the coming months and frequently if your daughter gets sick. Something that you might discuss with your new doctor is whether, if he agrees this really is diabetes, there is a case to continue with very small doses of insulin for the coming months. The purpose is not to control blood sugar; but to modify the lymphocyte response that damages the insulin-producing cells.


Original posting 9 Sep 97


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