Endocrine Abstracts

Introduction: Congenital hyperinsulinism (CHI) is the commonest cause of recurrent and persistent hypoglycaemia during the newborn period. The management of CHI in patients who are unresponsive or do not tolerate diazoxide includes the use of octreotide therapy which is given as a SUBCUTANEOUS injection, three to four times daily. We report a case of persistent CHI successfully treated with once monthly Lanreotide (a long acting somatostatin analogue).

Case: A 15-year-old girl with a diagnosis of CHI from infancy was managed with diazoxide therapy. Genetic analysis revealed a denovo ABCC8 mutation. 18-Fluro dopa positron emission tomography (PET) CT scan revealed diffuse disease. Although the glycaemic control was stable on diazoxide, she experienced troublesome hypertrichosis not amenable to therapies including waxing and laser. This had a huge impact on the quality of life with episodes of deliberate self-harm needing psychological assistance. A trial off diazoxide resulted in hyperinsulinaemic hypoglycaemia. Hence SUBCUTANEOUS octreotide was commenced (four daily injections) and a good glycaemic response was noted. The baseline ultrasound scan of the liver and gallbladder prior to starting octreotide was within normal limits. However, she disliked the daily SUBCUTANEOUS therapy. Hence a long acting somatostatin analogue (Lanreotide, 30 mg) was given subcutaneously following which the daily octreotide was gradually weaned and stopped. The continuous blood glucose monitoring system following the administration of Lanreotide revealed good glycaemic control with no episodes of hypoglycaemia. Currently she is on monthly Lanreotide therapy and off octreotide and diazoxide with good glycaemic control. This has led to a significant improvement in her quality of life.

Conclusion: Lanreotide is a safe and effective therapeutic option for patients who experience significant side effects of diazoxide. The long acting effect of once monthly injection confers an improved quality of life in these patients.