GROWTH AND ENDOCRINE FUNCTION AFTER NEAR-TOTAL PANCREATECTOMY FOR HYPERINSULINEMIC HYPOGLYCEMIA

Seven children, with a mean (SD) age of 4.6 (2.1) years, who as infant s (21 (7.5) days) underwent near total (95-98%) pancreatectomy for per sistent hyperinsulinaemic hypoglycaemia of infancy (PHHI) were studied . At birth all the infants were macrosomic. Four infants had been born after a difficult labour, of whom three had moderate birth asphyxia a nd respiratory distress. All had normal thyroid function. After surger y transient hyperglycaemia was manifest in six of the children and req uired insulin treatment for 5.8 (3.8) weeks, and transient hypoglycaem ia was encountered in one child and responded well to increased carboh ydrate intake and diazoxide for three weeks. Six of the children rapid ly crossed down their length and weight centiles during the first year after surgery. At the end of the first year these children were at or below the 5th centile of height and weight for their age and gender. After a period of 4.6 (2.1) years, their mean (SD) height score was -2 .57 (0.5), growth velocity 3.9 (0.75) cm/year, and growth velocity SD score -2.1 (0.55)1 these were significantly low and denoted significan t growth retardation. The growth hormone peak responses to provocation with clonidine were normal (13.5 (2.8) mu g/l). However, the circulat ing insulin-like growth factor-I (IGF-I) concentrations were significa ntly decreased (79 (34) ng/ml). Three of the children developed diabet es at two and a half, five, and seven years after surgery, two others had impaired oral glucose tolerance and six out of the seven children had an impaired C peptide response to glucagon. Defective insulin secr etion in these children might directly inhibit IGF-I synthesis in the liver. The body mass index of the pancreatectomised children was 14.9 (0.5) and was normal for age and gender; they had a normal 72 hour fae cal fat content and normal serum albumin concentration. These data ind icated grossly adequate exocrine pancreatic function. It appears that children requiring near total pancreatectomy for PHHI have normal deve lopmental milestones but defective linear growth with impaired insulin secretion and low IGF-I production despite normal growth hormone resp onse to provocation.