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September 4, 2001

Nesidioblastosis

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Question from Watertown, Massachusetts, USA:

A family friend has a new baby diagnosed with persistent hyperinsulinemic hypoglycemia of infancy (nesidioblastosis). What is the incidence in Caucasians (specifically Italian and Irish populations)?

Answer:

From: DTeam Staff

Two recent Scandinavian reports put the incidence at between 1:40,000 and 1:50,000 live births, but I know of no assessments limited to Irish or Italian populations nor do there seem to be any individual population figures for the two main forms of PHHI.

DOB
Additional comments from Dr. Marco Songini:

As far as the Italian data, I’m not aware of any published or reliable source regarding the incidence of persistent hyperinsulinemic hypoglycemia of infancy (nesidioblastosis) from my country.

MS
Additional comments from Dr. Jim Lane:

It is a very uncommon disorder in which there is continued production of insulin, despite the fact that the blood sugar is low or normal. This condition has to do with the activation of a receptor on the insulin-secreting beta cells which leaves the switch always on for insulin secretion. We are still learning a lot about this disorder. The receptor shares activity with the function of the group of hypoglycemic medications known as sulfonylureas.

JTL