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July 6, 2000


Question from Iowa, USA:

My daughter was diagnosed with ketotic hypoglycemia at 18 months of age. She is now 3 1/2 years old. At the University of Iowa they told us that she is the youngest case that they have ever seen. We find it very frustrating that every time that she gets sick. They admit her to the hospital and place her on an I.V. We are just wondering if they are doing this due to a lack of knowledge (due to her age or some factor) or if this is currently the only treatment. We have a very difficult time finding info on ketotic hypoglycemia and so do our doctors/endocrinologists. Do you have any current information that we can add to her file? Going to the ER is a nightmare needless to say. When she gets sick time is limited (she becomes so lethargic and lifeless). One thing that I do understand is that they talk about her body not releasing glucagon to signal the liver to release fat stores and we understand diet. We just want to know about treatments and how it all comes about. It is just heartbreaking to not understand and she is so young that it is hard for her to communicate her status.


So-called “ketotic hypoglycemia” is the commonest cause of severe hypoglycemia in childhood outside the neonatal period. In the great majority of instances it can be rapidly and effectively contained by intravenous glucose. In a small proportion of cases the underlying problem is an inborn error of organic acid or carbohydrate metabolism which needs special laboratory facilities to elucidate. In the common idiopathic form [that is, without a known cause] no specific abnormality has been defined in molecular terms and the answer seems to be just that some children are especially sensitive to any diminution in glucose availability from food intake and subsequent glycogen depletion in the liver. Initially it is probably not worth embarking on elaborate biochemical tests or on a search for infectious agents.

In the case of your little daughter however it seems that you might have reached the point of needing to discuss with her doctor the need to look for one of the specific underlying metabolic causes. In the interim you might consider using concentrated glucose solution in the cheek pouch or glucose in Koolaid to sustain glucose intake at the very first sign of illness. Prophylactically I would also try to wake her up when you go to bed and see if she would accept part of an Extend Bar or any flavoured glucose solution to prevent nocturnal hypoglycemia.