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Unusual course of neonatal hyperinsulinaemic hypoglycaemia (nesidioblastosis)
  1. Children’s Hospital
  2. University of Erlangen-Nürnberg
  3. Loschgestr. I S.
  4. D-91054 Erlangen
  5. Germany

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    Editor—Neonatal hyperinsulinaemic hypoglycaemia (NHH) is characterised by hypoglycaemia, inadequate hyperinsulinism, a lack of urinary ketones, and requires instant and effective treatment to prevent cerebral damage. This consists of diazoxide, glucagon, and octreotide, a somatostatin analogue. None the less, most patients require subtotal pancreatectomy.

    We report the second child of a healthy family, who was delivered at 37 weeks after an uneventful pregnancy (birthweight 3430 g). Initially, hypoglycaemia (blood glucose of 80 mg/l) was noted. Table 1 summarises the most important laboratory values. The glucose:insulin ratio was decreased in controlled hypoglycaemia (days 3 and 15) whereas a glucagon test yielded a normal response. An oral glucose tolerance test was performed in the mother with normal results. The glucose supplementation exceeded 16 mg/kg/min to achieve normoglycaemia. It could be tapered to 11.8 mg/kg/min without inducing hypoglycaemia.

    Table 1

    Values of blood glucose, insulin, C peptide and glucose:insulin ratio (normal range in parenthesis); controlled hypoglycemia on days 3 and 15

    Treatment with octreotide (4 × 30 μg subcutaneously from day 18) achieved normoglycaemia despite reduced glucose supplementation (5.6 mg/kg/min on the second treatment day). This was maintained even when the octreotide regimen was completed (day 52). No adverse effects of octreotide treatment were noted. On day 59 the patient was discharged. His diet consisted of four formula meals supplemented by 3g maltodextrin/kg/day. There were no further episodes of hypoglycaemia.

    At 12 months of age, the patient was healthy with no neurological deficits, age related nutrition, and normal laboratory tests (table1).

    Treatment with diazoxide (15–20 mg/kg/day) is often used to inhibit insulin secretion and increase gluconeogenesis, but it produces a wide range of side effects.1 Surgery had to be carried out in a further six patients we treated.2-4 However, pancreatectomy with partial resection carries a risk of continued hypoglycaemia, and total pancreatectomy carries a risk of life long insulin dependent diabetes mellitus and exocrine pancreatic insufficiency.2 5 Even if the immediate postoperative result is normoglycaemia, the risk of insulin dependent diabetes mellitus is increased.6

    Based on a report of somatostatin deficiency in pancreatic tissue and effective supplementation,3 we started a course of octreotide. Normoglycaemia was maintained despite reduced glucose supplementation and we stopped the treatment after only 34 days. Because length of treatment was significantly shorter than that reported previously and the glucose requirement decreased just before octreotide treatment, we suspect that maturation of the endocrine pancreas occurred. A similar conclusion was reached by Horev et al for three of four patients who became normoglycaemic.7 Therefore, we suggest a trial of stepwise reduction of glucose supplementation in combination with octreotide in NHH before subtotal pancreatectomy.