PMID- 10202168
OWN - NLM
STAT- MEDLINE
DCOM- 19990415
LR  - 20220331
IS  - 0028-4793 (Print)
IS  - 0028-4793 (Linking)
VI  - 340
IP  - 15
DP  - 1999 Apr 15
TI  - Clinical features of 52 neonates with hyperinsulinism.
PG  - 1169-75
AB  - 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.
FAU - de Lonlay-Debeney, P
AU  - de Lonlay-Debeney P
AD  - Department of Pediatrics, Hopital des Enfants Malades, Paris, France.
FAU - Poggi-Travert, F
AU  - Poggi-Travert F
FAU - Fournet, J C
AU  - Fournet JC
FAU - Sempoux, C
AU  - Sempoux C
FAU - Dionisi Vici, C
AU  - Dionisi Vici C
FAU - Brunelle, F
AU  - Brunelle F
FAU - Touati, G
AU  - Touati G
FAU - Rahier, J
AU  - Rahier J
FAU - Junien, C
AU  - Junien C
FAU - Nihoul-Fekete, C
AU  - Nihoul-Fekete C
FAU - Robert, J J
AU  - Robert JJ
FAU - Saudubray, J M
AU  - Saudubray JM
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - N Engl J Med
JT  - The New England journal of medicine
JID - 0255562
RN  - 0 (ATP-Binding Cassette Transporters)
RN  - 0 (Blood Glucose)
RN  - 0 (Glycated Hemoglobin A)
RN  - 0 (Insulin)
RN  - 0 (Potassium Channels)
RN  - 0 (Potassium Channels, Inwardly Rectifying)
RN  - 0 (Receptors, Drug)
RN  - 0 (Sulfonylurea Receptors)
SB  - IM
CIN - N Engl J Med. 1999 Apr 15;340(15):1200-1. PMID: 10202173
CIN - N Engl J Med. 1999 Aug 26;341(9):701-2. PMID: 10475832
MH  - *ATP-Binding Cassette Transporters
MH  - Blood Glucose/analysis
MH  - Glucose Tolerance Test
MH  - Glycated Hemoglobin A/analysis
MH  - Humans
MH  - Hyperinsulinism/complications/*congenital/pathology/surgery
MH  - Hyperplasia/complications/genetics/surgery
MH  - Hypoglycemia/etiology
MH  - Infant, Newborn
MH  - Insulin/blood
MH  - Islets of Langerhans/*pathology/physiopathology
MH  - Mutation
MH  - *Pancreatectomy/methods
MH  - Potassium Channels/genetics
MH  - *Potassium Channels, Inwardly Rectifying
MH  - Receptors, Drug/genetics
MH  - Sulfonylurea Receptors
EDAT- 1999/04/15 00:00
MHDA- 1999/04/15 00:01
CRDT- 1999/04/15 00:00
PHST- 1999/04/15 00:00 [pubmed]
PHST- 1999/04/15 00:01 [medline]
PHST- 1999/04/15 00:00 [entrez]
AID - 10.1056/NEJM199904153401505 [doi]
PST - ppublish
SO  - N Engl J Med. 1999 Apr 15;340(15):1169-75. doi: 10.1056/NEJM199904153401505.