April 8, 2002
Nesidioblastosis
Question from Scotland:
My friends have a wee 21 month old boy who has been diagnosed with nesidioblastosis and is neutropenic. They have had a terrible time — he is on diazoxide and chlorothiazide, is covered from head to toe in hair, and is in hospital at least once a month as he catches every virus there is. They are talking to doctors about partial removal of the pancreas as he has recently started taking fits. Are they doing the right thing? What is the success rate of this operation?
Answer:
Nesidioblastosis is a rare but serious disease which is not directly related to diabetes. The basic problem is hypoglycaemia because of too much insulin production by the pancreas. Sometimes it can be alleviated by the use of the drugs you mention, but very often surgery is required to remove the offending tissue. Because around 90% of the pancreas has to be removed to reduce insulin production enough, there is a risk of diabetes in later life.
KJR
