From the Guidelines
Nesidioblastosis, also known as Congenital Hyperinsulinism, is a rare condition characterized by an abnormal overgrowth of pancreatic islet cells, leading to excessive insulin production and hypoglycemia [ 1 ].
Key Characteristics
- It is associated with hypoglycemia and neuroglycopenic symptoms [ 1 ]
- It may be linked to Roux-en-Y gastric bypass surgery, although the exact relationship is still being researched [ 1 ]
Treatment Options
- Pancreatic resection and re-resection have been used to manage severe cases, particularly those that are refractory to other treatments [ 1 ]
- Dietary changes and medications may also be used to manage hypoglycemia, although the specific treatment approach will depend on the individual case
Important Considerations
- Individualized treatment is essential to balance the risk of hypoglycemia and potential surgical complications [ 1 ]
- Close monitoring of blood glucose levels and adjustment of treatment as needed is crucial to prevent long-term complications [ 1 ]
From the Research
Definition and Characteristics of Nesidioblastosis
- Nesidioblastosis is defined as the neoformation of the islets of Langerhans from the pancreatic ductal epithelium 2.
- It is recognized as the most common cause of hyperinsulinemic hypoglycemia in infants 2.
- In adults, nesidioblastosis is a rare form of abnormal islet cell proliferation arising from the pancreatic ductal epithelium, usually associated with severe hyperinsulinemic hypoglycemia 3.
- The condition is characterized by diffuse islet cell hyperplasia arising from the ductal epithelium 4.
Clinical Presentation and Diagnosis
- Nesidioblastosis can present with symptoms of hyperinsulinemic hypoglycemia, including fasting hypoglycemia 5.
- The diagnosis of nesidioblastosis can be challenging, as it may mimic insulinoma, and requires a combination of imaging, laboratory examinations, and histopathological analysis 2, 3.
- Selective arterial calcium stimulation (SACST) can be used to localize the source of insulin production and aid in the diagnosis of nesidioblastosis 5.
Treatment and Management
- Treatment of nesidioblastosis may involve medical management with diazoxide, although some patients may require pancreatectomy to control hypoglycemia 4.
- Greater than 90% resection of the pancreas frequently results in insulin dependency and permanent diabetes 4.
- Subtotal distal pancreatectomy has been shown to be effective in managing nesidioblastosis and preventing further hypoglycemic episodes 5.
Prevalence and Rarity
- Nesidioblastosis is a rare entity in adults, representing 0.5-5% of cases of organic hyperinsulinemia 6.
- Only 71 cases of adult-onset nesidioblastosis associated with hyperinsulinemic hypoglycemia have been published since 1981 6.
- The condition is more commonly associated with infants, where it is the primary cause of persistent hyperinsulinemic hypoglycemia 6.