What is Congenital Hyperinsulinism?

CHI is a rare genetic disorder affecting newborns and children. It is essentially the "opposite" of diabetes. In diabetes, the pancreas makes too little insulin (causing high sugar). In CHI, the pancreas releases too much insulin constantly, causing severe Hypoglycemia (Low Blood Sugar).

Why is it dangerous?

Glucose (sugar) is the brain's only fuel source. If insulin keeps pushing sugar into muscles and fat instead of the brain, the baby can suffer seizures or permanent brain damage.

Signs in Babies:

• Jitteriness or tremors.
• Lethargy (too sleepy to feed).
• Blue spells (cyanosis) or stopping breathing (apnea).
• Seizures.

Treatment:

• Medication: Diazoxide is the first-line drug. It acts on the pancreas to stop insulin release. Octreotide (injections) is used if Diazoxide fails.
• Diet: Frequent feedings with added carbohydrates.
• Surgery: If the problem is isolated to one spot in the pancreas (focal disease), surgery can cure it. If it affects the whole pancreas (diffuse), near-total removal may be needed.