Nesidioblastosis of pancreas
Nesidioblastosis of the Pancreas Nesidioblastosis of the pancreas is a rare condition characterized by the proliferation of insulin-producing beta cells in the pancreas. This condition can lead to hyperinsulinemic hypoglycemia, a state where excessive insulin causes low blood sugar levels. It is most commonly seen in infants but can also occur in adults.
Pathophysiology[edit | edit source]
Nesidioblastosis involves the abnormal growth of islet cells, particularly the beta cells, which are responsible for insulin secretion. This overgrowth can result in excessive insulin production, leading to hypoglycemia. The exact cause of nesidioblastosis is not well understood, but it is thought to involve genetic factors and possibly environmental triggers.
Clinical Presentation[edit | edit source]
Patients with nesidioblastosis typically present with symptoms of hypoglycemia, which can include:
- Sweating
- Tremors
- Palpitations
- Confusion
- Seizures
- Loss of consciousness
In infants, symptoms may include poor feeding, irritability, and lethargy.
Diagnosis[edit | edit source]
The diagnosis of nesidioblastosis is challenging and involves a combination of clinical evaluation, laboratory tests, and imaging studies. Key diagnostic steps include:
- Measuring blood glucose and insulin levels during hypoglycemic episodes.
- Performing a fasting test to observe the body's response to prolonged fasting.
- Imaging studies such as an MRI or CT scan to assess the pancreas.
- A selective arterial calcium stimulation test may be used to localize insulin hypersecretion.
Treatment[edit | edit source]
Treatment of nesidioblastosis aims to manage hypoglycemia and may include:
- Dietary modifications to prevent hypoglycemic episodes.
- Medications such as diazoxide or octreotide to reduce insulin secretion.
- Surgical intervention, such as partial pancreatectomy, may be necessary in severe cases.
Prognosis[edit | edit source]
The prognosis for individuals with nesidioblastosis varies. Infants often respond well to treatment, but some may require ongoing management. In adults, the condition can be more challenging to treat, and surgical intervention may be necessary.
See Also[edit | edit source]
External Links[edit | edit source]
- [Link to a reputable medical resource]
NIH genetic and rare disease info[edit source]
Nesidioblastosis of pancreas is a rare disease.
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Contributors: Prab R. Tumpati, MD
