May 24, 2001
Hypoglycemia
Question from Wetumpka, Alabama, USA:
Last year, my three year was diagnosed with ketotic hypoglycemia, and she is unresponsive to the injectable glucagon. The doctors hospitalized her during her last bout of hypoglycemia so they could do fasting blood work for a more definite diagnosis. They did obtain dozens of labs (all of which came back within normal limits), but still no answers. The amino acid test is not back yet, however she did not respond to the glucagon as anticipated when her blood sugar dropped to 32 mg/dl [1.8 mmol/L], she had to have intravenous dextrose to make it rise. The doctors were all surprised by that, and it left many unanswered questions. Can you tell me if there are other reported cases of such a reaction? What other tests should be performed?.
I assure you that my daughter is on an appropriate diet and is not starved by any means. Its very nerve racking when people suggest that she is not on the proper diet. We follow a very strict diet on a daily basis, and there seems to be no pattern what so ever for her sudden declines in her readings. I would love some answers!
Answer:
Clearly your daughter does not have the conventional form of ketotic hypoglycemia which is due to carbohydrate deprivation, and it now seems as though her doctors are exploring the much rarer possibilities of some of the inborn errors of carbohydrate, organic acid and amino acid metabolism. This may require the rather sophisticated support of a special laboratory with advanced techniques available like gas chromatography/mass spectroscopy and I wouldn’t want to speculate on the precise answer without having all of this information. At all events, I am sure that diabetes is not one of the possibilities, and the only possible ‘dietary’ explanation would be that of hereditary fructose intolerance which has probably already been excluded.
DOB
