INDIAN PEDIATRICS VOLUME 35-APRIL 1998 PERSISTENT HYPERINSULINEMIC HYPOGLYCEMIA OF INFANCY

Objective To study the nature and clinical course of persistent hyperinsulinemic hypoglycemia of infancy (PHHI) due to nesidioblastosis Design Clinical, laboratory and therapeutic evaluation of infants with this disorder and study the outcome Setting Hospital born neonates and infants referred from other hospitals Subjects Thirteen infants from 9 families inclusive of four pans of siblings referred within few hours of birth to 3 months of age, for seizures Mean birth weight was 3 68±0 45 kg Consanguinity documented in one sporadic and one familial case Methods ' Clinical and laboratory evaluation by standard biochemical and radioimmunoassay techniques Results: The mean serum insulin level of 24 2±12.5 mlu/ml was in the normal range but inappi opnately high for the con evponding hypoglycemic blood sugar (23.1±9.1 mg/dl) value, with an I/G ratio of 1 36±0 97; as in hyperinsulinemia (normal <0.4) Investigations excluded other causes of persistent hypoglycemia A trial of TV/oral glucose, frequent carbohydrate rich feeds in all, oral diazoxide (10 to 20 mg/kg) in 9/13 cases along with subcutaneous octreotide (20 Hg/kg QID) in one helped, but pancreatic tesection (85 to 90%) zvas opted for in two (1 familial, 1 sporadic) Six infants including one with pancreatic resection succumbed to hypoglycemia (n=1) or fulminating infection (n=3) or brain damage Of the seven survivors, on