Clinical features of 52 neonates with hyperinsulinism.
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.
de Lonlay-Debeney
P
P
Department of Pediatrics, Hôpital des Enfants Malades, Paris, France.
Poggi-Travert
F
F
Fournet
J C
JC
Sempoux
C
C
Dionisi Vici
C
C
Brunelle
F
F
Touati
G
G
Rahier
J
J
Junien
C
C
Nihoul-Fékété
C
C
Robert
J J
JJ
Saudubray
J M
JM
D018528:ATP-Binding Cassette Transporters; D001786:Blood Glucose / Q000032:analysis; D005951:Glucose Tolerance Test; D006442:Glycated Hemoglobin / Q000032:analysis; D006801:Humans; D006946:Hyperinsulinism / Q000150:complications / Q000151:congenital* / Q000473:pathology / Q000601:surgery; D006965:Hyperplasia / Q000150:complications / Q000235:genetics / Q000601:surgery; D007003:Hypoglycemia / Q000209:etiology; D007231:Infant, Newborn; D007328:Insulin / Q000097:blood; D007515:Islets of Langerhans / Q000473:pathology* / Q000503:physiopathology; D009154:Mutation; D010180:Pancreatectomy / Q000379:methods; D015221:Potassium Channels / Q000235:genetics; D024661:Potassium Channels, Inwardly Rectifying; D011955:Receptors, Drug / Q000235:genetics; D064233:Sulfonylurea Receptors
D018528:ATP-Binding Cassette Transporters; D001786:Blood Glucose; D006442:Glycated Hemoglobin; D007328:Insulin; D015221:Potassium Channels; D024661:Potassium Channels, Inwardly Rectifying; D011955:Receptors, Drug; D064233:Sulfonylurea Receptors