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August 2, 2000

Diagnosis and Symptoms

Question from Wausau, Wisconsin, USA:

My son was 17 months old when I found him in a comatose like state about 3 months ago. I took him to the ER and was told his sugar level was at 35 mg/dl (1.9 mmol/L). During the next 3 days in the hospital, his levels varied between 60-488 mg/dl (3.3-27.1 mmol/L). He was diagnosed with ketotic hypoglycemia. I was told to feed him six times a day, and to wake him about 12-2 am for a feeding if he's sick (starch, proteins, etc.). Now, when he's sick, his level goes to 277 mg/dl (15.4 mmol/L) after two-and-one-half hours without eating (at one point, it was 217 mg/dl (12.1 mmol/L) after going six hours without eating). He experiences very high ketones at times in his urine. Even when he's not sick, he still has trace to small amounts of ketones (on a near daily basis). Our pediatrician said that he looks like he could be in a prediabetic state right now, and we may need to give him insulin in the near future, if these levels continue. His endocrinologist, however, says that my son has no greater risk of diabetes than anyone without hypoglycemia. My brother passed away about 7 years ago at the age of 26 from complications of diabetes. (His sugar had gone too low. He was type 1.) My son's A1c test was taken last month. It was 5.9 percent. The endocrinologist said the number is a little high for someone with hypoglycemia.

Answer:

It is easy to see how there is some confusion over your small son’s story. I don’t think that there is any doubt that his seizure was the result of hypoglycemia secondary to carbohydrate restriction. The ketosis is because in the absence of calories from carbohydrate the body metabolises fat for energy. It is an essentially normal process which seems to be exaggerated in some children so that it is then called Ketotic Hypoglycemia.

This event may also have accelerated an autoimmune process that was already destroying the insulin producing cells in the pancreas, and it is even possible that the remaining damaged cells were secreting insulin inappropriately which would have accentuated the hypoglycemia. At first sight the Hemoglobin A1c of 5.9% seems rather unexceptional, but, without knowing the normal range of the method used and the day to day confidence limits in ordinary daily use, it’s impossible to bas a judgement on a single sample. In any case, the loss of glucose tolerance may have been quite short lived and not have had time to push up the A1c.

In short, I suspect that your pediatrician is right and that your small son may have Type�1A (autoimmune) diabetes and in the light of the fasting blood glucose levels that you report. I believe that he has passed through the prediabetes stage and has already reached the point of insulin dependence and that there is some risk in just awaiting events.

I think you need to see the doctor and insist on an antibody test, which would, almost certainly, confirm the diagnosis if positive and indicate the need for immediate insulin.

Your son is too young to participate in the national DPT-1 study, but if you call 1-800-425-8361, they would be able to tell you where the nearest participating laboratory is. Alternatively, the test can be done by Quest Laboratories in California at 1-949-728-4235. You need to discuss all this with your son’s doctor, but I would be concerned that, at this stage, an intercurrent infection might precipitate an episode of ketoacidosis.

DOB
Additional comments from Dr. Tessa Lebinger:

It is possible that this child has a rare form of ketotic hypoglycemia. In some of these disorders, patients sometimes have glucose intolerance.

If the antibody test is negative, your son’s doctor might have to consider one of the rare inborn errors of metabolism that also give rise to Ketotic Hypoglycemia.

TGL