Question

Treatment of Congenital Hyperinsulinemia.

Hint:

Diazoxide is the primary treatment for congenital hyperinsulinemia, but surgery may be needed if medical therapy fails.

Answer 1

Step 1: Medical Management:
1. Diazoxide: The first-line treatment for congenital hyperinsulinemia is diazoxide, which inhibits insulin release from the beta cells. It helps raise blood glucose levels in affected infants.
2. Octreotide: This somatostatin analog can also be used to inhibit insulin secretion when diazoxide is ineffective or contraindicated.

Step 2: Surgical Management:
In cases where medical management fails or if there is focal hyperinsulinism, surgery may be required.
1. Pancreatectomy: Partial pancreatectomy may be performed to remove the hyperinsulinic tissue, especially in cases where the condition is localized to one part of the pancreas.
2. Subtotal Pancreatectomy: In cases of diffuse hyperinsulinism, a more extensive surgery may be required, though it carries the risk of diabetes.

Step 3: Monitoring and Support:
Continuous monitoring of blood glucose levels is essential, and intravenous glucose may be required in the acute management phase.