Effect of Exendin-(9-39) on Fasting Adaptation and Protein Sensitivity

This is a placebo controlled study with randomized crossover design to evaluate the effect of
the glucagon-like peptide-1 (GLP-1) receptor antagonist, exendin-(9-39), on fasting blood
glucose levels, protein-induced hypoglycemia, and fasting tolerance of subjects with
congenital hyperinsulinism due to mutations in the ATP- sensitive potassium channel (KATP)
channel.

Inclusion Criteria:

- Confirmed diagnosis of hyperinsulinism

- Mutation analysis results demonstrating KATP channel defect

- Age 6 months to 18 years with

- Persistent hypoglycemia

Exclusion Criteria:

- Current therapy with medications that may affect glucose metabolism such as
octreotide, diazoxide, high dose glucocorticoids, adrenergic agents, etc. Subjects
will be eligible to participate if the last dose of octreotide is given 48 hrs before
study day 1 and the last dose of diazoxide is given 72 hours before study day 1

- Evidence of a medical condition that might alter results or compromised the
elimination of the peptide, including active infection, kidney failure, severe liver
dysfunction, severe respiratory or cardiac failure

- Pregnancy

- Subjects with milk protein allergy will be excluded for participating in studies
involving protein tolerance test