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June 2, 2000


Question from Machilipatnam, Andhra Pradesh, India:

I am caring for a 67 day old pre-term baby with intractable hypoglycemia and hepatosplenomegaly [enlargement of the liver and spleen]. No other anomalies have been found. The hypoglycemia is corrected by hydrocortisone. Please direct me toward me further management.


It is not easy to provide guidance for a history like this without a great deal more information. Obviously though, assuming that the infant is taking feedings and making reasonable progress, the next step is to define the cause of the hypoglycemia. With hepatosplenomegaly it is clearly tempting to start with hepatic causes of hypoglycemia like glycogen storage disease, rare as this may be. It might be helpful to initially find out if there is a response to a dose of glucagon adjusted on a surface area basis. After that and depending on the infant’s progress, you might want to consider a biopsy. A lot would depend on the experience of the pathologist and on the ability to get tissue either by needle or by a minimal incision under a local anaesthetic. At some point it would be important, if it is at all possible, to have the infant seen a paediatrician with a special interest in inherited metabolic disease so that rare possibilities like disorders of organic acid metabolism and lyposomal storage disease could be considered. I don’t think, by the way, that the problem has anything to do with diabetes.