From Pinellas Park, Florida, USA:
My dearest friend's new grandson (1 month old) has an extremely low blood sugar level and high blood pressure. CT-scan of the pancreas was done which came back negative. They are considering doing a tissue biopsy and/or complete or partial removal of the pancreas. He is in the Neonatal Intensive Care Unit at a major university teaching hospital in another state. The doctors cannot seem to get his blood sugar level up.
Are there any suggestions on what might be the problem? We are wondering what the normal blood sugar level for an infant is, how strong is the possibility of brain damage, what are the consequences of removing the pancreas and what type of care would be needed in the future. Any information would be extremely helpful.
You may use my e-mail address in hopes that someone else out there may be able to help guide us.
First of all I would like to say that I'm sure that the doctors on the pediatric ICU at that hospital know exactly what they are doing.
Without blood insulin levels it's not possible to be absolutely sure; but I suspect that your neighbor's child's doctors think this baby has a condition called nesidioblastosis in which the islets cells of the pancreas excrete an inappropriately large amount of insulin which in turn depresses the blood sugar below normal levels.
Blood glucose values can usually be maintained by intravenous infusion of glucose; but once the diagnosis is made the next step is to try a variety of drugs of which one called diazoxide is the most usually effective. Some infants can be maintained in this way; but most often it has been necessary to remove some 90% of the pancreas. The disadvantage of this procedure is of that not quite enough of the pancreas is removed and also of course, that the enzymes of what is called the exocrine pancreas need to be replaced orally. A short time ago a group at The Hospital for Sick Children at Great Ormond Street in London, England avoided surgery by using a quite different group of drugs called 'calcium channel blockers' specifically one called nifedipine [a medication used to control blood pressure]. I would expect that the hospital's doctors will already have contacted them to see if the early reports have held up; but in any case you can find more details of this approach in a previous question.
These days the prognosis is extremely good, and although there is some danger from brain damage from hypoglycemia in the earliest stages, this is very unlikely to happen once the baby is under observation in a University Hospital ICU. Without some more details, I think it would be inappropriate to comment further on the blood pressure issue; but I sense that this issue is subsidiary to the blood sugar one. Normal fasting blood sugars in a full term infant can range from 30-100 mg/dl.
Additional Comments from Dr. Lebinger:I assume that your friend's grandson has been checked to make sure cortisol deficiency is not a cause of the low blood sugar. There are very rare congenital disorders where the child is missing enzymes necessary to make cortisol. Cortisol deficiency can cause low blood sugars. Two of these disorders, 11 hydroxylase deficiency and 17 hydroxylase deficiency can be associated with high blood pressure as the adrenal glands make too much of the hormones which control blood pressure. Blood testing can diagnose these disorders.
Original posting 2 Jan 98
Updated 12 Apr 1998
Last Updated: Tuesday April 06, 2010 15:08:54
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