
September 11, 2001
Genetics and Heredity, Nesidioblastosis
Question from Childersburg, Alabama, USA:
I am 22 years old, I was born with nesidioblastosis, I had to have half of my pancreas removed when I was a baby, and I developed diabetes at the age of 14. My 23 year old brother who also had nesidioblastosis, had 90% of his pancreas removed at birth, and he developed diabetes right away. I am married and would like to have children in the future. My glucose is in good control and I know that must take care of myself. What are the chances that my children or my brother’s children will be born with nesidioblastosis? I heard that it can be detected from the womb now.
Answer:
I do know of families with multiple generations affected with nesidioblastosis. We now know more about the genetics and mutations that seem to cause the syndrome, which we now call it persistent hyperinsulinemic hypoglycemia of infancy. I don’t pretend to be an expert, but I know that competent genetic consultation will lead to some lab work for you and your sib that can give the marker, if currently one of those recognized as such. I don’t know a medical center to refer you to at present.
LD
[Editor’s comment: See a previous answer to a question on this topic.
SS]