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Previous Volume 340:1169-1175 April 15, 1999 Number 15 Next

	Full Text PDF Editorial by Stanley, C. A. Letters Add to Personal Archive Add to Citation Manager Notify a Friend E-mail When Cited PubMed Citation

ABSTRACT

Background Neonatal hyperinsulinemic hypoglycemia is often resistant to medical therapy and is often treated with near-total pancreatectomy. However, the pancreatic lesions may be focal and treatable by partial pancreatic resection.

Methods We studied 52 neonates with hyperinsulinism who were treated surgically. The type and location of the pancreatic lesions were determined by preoperative pancreatic catheterization and intraoperative histologic studies. Partial pancreatectomy was performed in infants with focal lesions, and near-total pancreatectomy was performed in those with diffuse lesions. The postoperative outcome was determined by measurements of plasma glucose and glycosylated hemoglobin and by oral glucose-tolerance tests.

Results Thirty neonates had diffuse beta-cell hyperfunction, and 22 had focal adenomatous islet-cell hyperplasia. Among the latter, the lesions were in the head of the pancreas in nine, the isthmus in three, the body in eight, and the tail in two. The clinical manifestations were similar in both groups. The infants with focal lesions had no symptoms of hypoglycemia and had normal preprandial and postprandial plasma glucose and glycosylated hemoglobin values and normal results on oral glucose-tolerance tests after partial pancreatectomy (performed in 19 of 22 neonates). By contrast, after near-total pancreatectomy, 13 of the patients with diffuse lesions had persistent hypoglycemia, type 1 diabetes mellitus developed in 8, and hyperglycemia developed in another 7; overall, only 2 patients with diffuse lesions had normal plasma glucose concentrations in the first year after surgery.

Conclusions Among neonates with hyperinsulinism, about half may have focal islet-cell hyperplasia that can be treated with partial pancreatectomy. These neonates can be identified through pancreatic catheterization and intraoperative histologic studies.

Source Information

From the Departments of Pediatrics (P.L.-D., F.P.-T., G.T., J.-J.R., J.-M.S.), Pathology (J.-C.F.), Radiology (F.B.), and Surgery (C.N.-F.) and INSERM Unité 383, Génétique, Chromosome, et Cancer (C.J.), Hôpital des Enfants Malades, Paris; the Department of Pathology, Cliniques Universitaires St. Luc, Université de Louvain, Brussels, Belgium (C.S., J.R.); and the Division of Metabolism, Ospedale Bambino Gesu, Rome (C.D.V.).

Address reprint requests to Dr. Saudubray at the Fédération de Pédiatrie, Service de Métabolisme, Hôpital des Enfants Malades, 149 rue de Sèvres, 75743 Paris CEDEX 15, France.

Full Text of this Article

Related Letters:

Clinical Features of Neonates with Hyperinsulinism
Hawdon J.M., Kalhan S. C., Platt M. P. W., Cornblath M., Saudubray J.-M., de Lonlay-Debeney P., Robert J.-J., Stanley C. A., Baker L.
Extract | Full Text  
N Engl J Med 1999; 341:701-702, Aug 26, 1999. Correspondence

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