We evaluated the detailed pancreatic endocrine and exocrine function in children with persistent hyperinsulinaemic hypoglycaemia of infancy (PHHI) 95% pancreatectomy. Seven children with PHHI between 0.9 and 5.2 years after pancreatic resection underwent clinical and investigative follow up. Three children with PHHI who had not had pancreatectomy were also assessed. Standard endocrine assessment, pancreatic magnetic resonance imaging (MRI), and detailed direct and indirect tests of exocrine pancreatic function were performed. Pancreozymin-secretin stimulation test results were deficient in four out of the seven patients, one of whom had frank steatorrhea required daily pancreatic enzyme supplements.

One child developed insulin dependent diabetes at 3 years and two children had impaired glucose tolerance. MRI showed no major re-growth of the pancreatic remnant after resection (n = 4). The height SD score, growth velocity SD score and BMI were significantly lower in Children who underwent near-total pancreatectomy vs. non-pancreactomised children. The head circumference was markedly smaller in the non-pancreactomised children and all of them had poor neuro-developmental outcome, with global developmental delay and neurological abnormalities. Two out of the seven pancreactomised children had developmental delay and spastic cerebral palsy. Circulating IG.F-I and basal GH concentrations were lower in the pancreactomised group. Their basal and glucagon-stimulated C-peptide concentrations were significantly decreased compared to the non-pancreactomised children. Basal growth hormone (GH) levels were higher in the non-pancreatectomized group.

Growth hormone response to provocation was adequate in both groups. HbA1C concentration was significantly lower in the non-pancreactomised group as well as their fasting and 2h-post prandial blood glucose levels compared to pancreactomised children. Clinical evidence of endocrine dysfunction has developed in three patients (1 with IDDM, and 2 with IGT). Three patients had subclinical deficiency of one or two exocrine pancreatic enzymes but only one had multiple enzyme deficiencies and steatorrhea and required pancreatic enzyme replacement. Although 95% pancreatectomy results in postoperative control of blood glucose, the development of IDDM, impaired linear growth, and exocrine failure remain ongoing risks.