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February 22, 2004

Nesidioblastosis

Question from New Delhi, India:

My 21 day old granddaughter weighing three kilograms at birth is suspected to be suffering from hypoglycemia from day one of her birth. Immediately after coming to this world, her body temperature dipped and the attending doctor tested her blood sugar which was found to be 23 mg/dl [1.27 mmol/L]. Her blood serum insulin level was 12.5 after fasting. Since then, she is being treated for hypoglycemia in the nursery with Diazoxide each dose of 12.5 to 25mg thrice a day but her sugar levels still fluctuate between 17 mg/dl [.94 mmol/L] to 23 mg/dl [1.27 mmol/L] or so whenever she is put off the glucose drip and put to breast feed. Could you please tell us how the baby's diagnosis of hypoglycemia can be confirmed and what treatment we should pursue. Is this disease fully curable?

Answer:

Your granddaughter would appear to be suffering from a rare condition called PHHI, Persistant Hyperinsulinemic Hypoglycemia of Infancy, and the early onset suggests that it may be difficult to manage. Diazoxide is the usual initial therapy; but if intraveous glucose continues to be needed, the addition of the calcium channel blocker nifedipine may help. However, blood sugar levels of 12 to 25 mg/dl (0.7 to 1.4 mmol/l) leave no doubt about the severity of the hypoglycemia and the threat this poses to cognitive developement, so that I am sure the doctors are considering an almost complete pancreatectomy.

In this connection, there has been recent work showing that the increase in insulin producing cells can be diffuse or localized and, if the rather sophisticated techniques for detecting the latter are available, the long term results of appropriate surgery are significantly better than with almost complete removal. A much more complete account of this problem and of its genetic and spontaneous causes, can be found at Practical management of hyperinsulinism in infancy for which you may need the help of a medical librarian as well as more briefly in a previous question. A significant proportion of these infants do recover spontaneously before he end of the first year, if initial treatment can contain the hypoglycemia.

DOB