Nesidioblastosis

Nesidioblastosis
Synonyms	Congenital hyperinsulinism, Persistent hyperinsulinemic hypoglycemia of infancy (PHHI)
Pronounce	N/A
Specialty	N/A
Symptoms	Hypoglycemia, seizures, lethargy, poor feeding
Complications	Neurological damage, developmental delay
Onset	Infancy
Duration	Chronic
Types	N/A
Causes	Beta cell hyperplasia in the pancreas
Risks	Genetic predisposition, mutations in ABCC8 or KCNJ11 genes
Diagnosis	Blood tests, insulin levels, genetic testing, pancreatic biopsy
Differential diagnosis	Insulinoma, Beckwith-Wiedemann syndrome, congenital adrenal hyperplasia
Prevention	N/A
Treatment	Diazoxide, octreotide, pancreatectomy
Medication	Diazoxide, octreotide
Prognosis	Variable, depends on severity and treatment
Frequency	Rare
Deaths	N/A

Nesidioblastosis is a rare medical condition characterized by the overproduction of insulin due to the enlargement of the beta cells in the pancreas. This condition is most commonly diagnosed in infants and children, but can also occur in adults. The excessive insulin production can lead to persistent hypoglycemia (low blood sugar levels).

Symptoms

The primary symptom of nesidioblastosis is hypoglycemia, which can manifest as:

• Trembling or shakiness
• Sweating
• Rapid heartbeat
• Blurred vision
• Dizziness or lightheadedness
• Hunger
• Irritability or moodiness
• Fatigue or weakness
• Headache

Causes

The exact cause of nesidioblastosis is unknown, but it is believed to be related to genetic mutations. Some cases have been associated with specific genetic disorders, such as Beckwith-Wiedemann syndrome and Turner syndrome.

Diagnosis

Diagnosis of nesidioblastosis typically involves blood tests to measure insulin and glucose levels. Imaging tests, such as a CT scan or MRI, may be used to visualize the pancreas. In some cases, a biopsy of the pancreas may be necessary.

Treatment

Treatment for nesidioblastosis typically involves medications to manage blood sugar levels. In severe cases, partial or total removal of the pancreas (pancreatectomy) may be necessary.

See also

• Pancreas
• Insulin
• Hypoglycemia
• Beckwith-Wiedemann syndrome
• Turner syndrome

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