We report five children who required total pancreatectomy in the neonatal period for life threatening hypoglycaemia. Subsequent diabetes was managed with once daily subcutaneous insulin, oral pancreatic extract, and a controlled carbohydrate diet. Daily home blood glucose profiles in four of these children were different from those of 14 C-peptide negative children with type 1 insulin dependent diabetes--in the pancreatectomy group there was less variation in blood glucose throughout the day with a fall after breakfast as against a rise after breakfast in the type 1 group. Hyperglycaemia (greater than 10 mmol/l) with or without ketonaemia was extremely rare in the pancreatectomy group. Despite neonatal convulsions, physical growth and development have been normal. Hypoglycaemia, usually after food refusal, has been a major problem. In addition, there have been considerable emotional and family disturbances despite the excellent metabolic control, which presumably reflect the cumulative difficulties in managing such young children with diabetes.
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