PERSISTENT HYPOGLYCEMIA - CONGENITAL HYPERINSULINISM OF INFANCY A CASE REPORT

Congenital hyperinsulinism of infancy (CHI) is a condition in which there is inappropriate insulin secretion by the pancreatic beta islet cells secondary to various genetic disorders. Recurrent episodes of hyperinsulinemic hypoglycemia may expose the infant to the risk of brain damage. CHI is characterized by high glucose requirement to correct hypoglycemia, hypoglycemia which responds to exogenous glucagon and absence of ketone bodies during the hypoglycemic period. CHI presents with two main clinically indistinguishable histopathological lesions diffuse and focal. Partial pancreatectomy effects complete cure in focal lesions whereas in diffuse variety even after Near total pancreatectomy the long term outcomes may not be satisfactory. Here we report about an infant who presented with altered sensorium, recurrent seizures, persistent hypoglycaemia, respiratory failure and shock due to congenital hyperinsulinism. She required glucose infusion rates upto 20mgkgmin and Octreotide 20mcgkgday to maintain euglycemia until thirty days of life. She did not respond to medical management with Diazoxide and Nifedipine. Subsequently 68 Ga -DOTANOC PET scan of the pancreas revealed diffuse pancreatic hyperplasia. She required Near-total pancreatectomy and subsequently the infant maintained euglycemia.