May 26, 2003
Nesidioblastosis
Question from Ra'anana, Israel:
My brother, sister, and I all were born with nesidioblastosis. What is the percentage of people in the world who have nesidioblastosis?
Answer:
The incidence of nesidioblastosis is about 1:50,000 live births, which means that the likelihood of three siblings in one family being affected is very small indeed and suggests some unusual pathology. The condition is now more usually referred to as PHHI or Persistent Hyperinsulinemic Hypoglycemia of Infancy, and there are two subtypes, one in which there is a diffuse hyperplasia of the insulin-producing cells and a second in which this hyperplasia is limited to a discrete area. Treatment is normally with Diazoxide, Octreotide or Calcium channel blockers or by surgery.
DOB