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From Potomac, Maryland, USA:

My 31 year old daughter has/had nesidioblastosis from infancy. She had a partial pancreatectomy, 85 to 90 percent, at seven months of age. It made no difference to her glucose levels. She has the diffuse kind of beta cells. She was one of the first treated with diazoxide, first in liquid form. She was also fed through a gastrostomy tube at night. She developed petit mal type seizures which were staring spells that signaled to us that her blood sugar was going too low. She was on diazoxide until she was about 12 with significant hirsutism and fluid retention. Then, she had a grand mal seizure at gym class at school and afterwards, everything somehow got better. Her blood sugars became more manageable and the petit mals disappeared. She was taken off both diazoxide and epilepsy medicines. She has since been diligent about checking her blood sugars and adjusting her food intake accordingly. She is overweight, but not obese.

I have two sets of questions:

  1. What is her prognosis? Her blood sugars are now edging up and our endocrinologist put her on Glucophage. What are the long-term side effects of diazoxide?

  2. My other child has no hyperinsulinism, but what are the chances of his future children (and indeed, my daughter's future children, if any) of developing the disease? Is genetic testing available now and where?


Great questions, but you should really ask these to your diabetes team since they know the details better than anyone else. We think that this is extremely rare and, so, not likely high risk for future children or for your other relatives. The weight you describe is a major health risk factor so it should not be minimized. This may be key to longevity following pancreatectomy and needs to be taken into consideration for current insulin and glucose management. I suspect this is the reason for prescribing the metformin (Glucophage). Blood pressure control, and lipid monitoring and control are also very important particularly in light of excess weight since all these factors are cumulative and add enormous risks if not brought under optimal control with diet, exercise and/or medications.


Original posting 28 Jan 2006
Posted to Nesidioblastosis and Other


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