I am 15 years old and as an infant, I had a sub-total pancreatectomy because of nesidioblastosis which has left me with type 1 diabetes. Are there any tests I need to go through before I can have children? If I had children in the future would they be at a higher risk of developing nesidioblastosis?

The recommendations to you would be the same as for anyone else with type 1 diabetes. Essentially, you should be in good glucose control prior to conception and maintain that throughout the pregnancy. You should also be taking extra folic acid (1 mg/day) to reduce the risk of neural tube defects.

Nesidioblastosis is an autosomal recessive disorder. This means that if you have a child with someone who carries the gene, but is not affected, then the risk of a child with nesidioblastosis is 50% and of being a carrier (no disease) is also 50%. However, if you have a child with someone who does not carry the defect, then your child would only be a carrier. Nesidioblastosis is rare (I do not know the incidence in the population), thus the chances of having a child with someone who is a carrier is low. I would strongly suggest that you meet with a genetic counselor before you decide to have children to discuss this further.

OWJ