Management of Hypoglycemia in Congenital Hyperinsulinism with the Somatostatin-Analogue Pasireotide

Presentation Number: MON 603
Date of Presentation: April 3rd, 2017

Julie Lytton Lorton*1, Erika Brutsaert1, Jill P Crandall2 and Norman Fleischer2
1Montefiore Medical Center, Bronx, NY, 2Albert Einstein College of Medicine, Bronx, NY


Background: Pasireotide is a somatostatin analogue with high affinity for several somatostatin receptors, including somatostatin receptor 2 and somatostatin receptor 5, both found on pancreatic beta cells. Pasireotide is currently FDA-approved for treatment of acromegaly and Cushing’s disease, and has a documented side effect of hyperglycemia. In addition, pasireotide has shown some benefit in reducing hypoglycemia in a few cases of insulinoma and post-bariatric hyperinsulinemic hypoglycemia. These effects are likely due, in part, to a direct inhibitory effect of pasireotide on pancreatic beta cells. Octreotide, a somatostatin analogue with a narrower binding affinity than pasireotide, has been used in both congenital hyperinsulinism and insulinoma with mixed results. We herein report a unique case of congenital hyperinsulinemic hypoglycemia that improved with pasireotide treatment.

Clinical case: A 30 year old man with a history of congenital hyperinsulinism due to a compound heterozygous ABCC8 mutation, in remission for 10 years, had recurrence of hyperinsulinemic hypoglycemia at age 26. Imaging at time of hypoglycemia recurrence including CT scan, MRI and 18F-fluorodopa PET revealed no evidence of new onset insulinoma but were consistent with congenital hyperinsulinism. Due to an allergic reaction to diazoxide, an attempt was made to treat his hypoglycemia with octreotide. A trial of octreotide did not reduce the frequency or severity of hypoglycemic episodes. Subsequently, he was started on subcutaneous pasireotide.

The patient experienced a reduction in the occurrence of hypoglycemic events following initiation of pasireotide. This effect has been durable with treatment duration of 2 years to date. At his most recent clinical evaluation he reported hypoglycemic episodes less than once per week, none severe or requiring assistance in management.

Conclusion: This is the first case report describing the use of pasireotide in congenital hyperinsulinsm, demonstrating a potential role for pasireotide in the reduction of hypoglycemic events in this disorder.


Nothing to Disclose: JLL, EB, JPC, NF