PEDIATRICS Vol. 114 No. 4 October 2004, pp. e520-e522 (doi:10.1542/peds.2003-1180-L)
ELECTRONIC ARTICLE |
Laparoscopic Diagnosis and Cure of Hyperinsulinism in Two Cases of Focal Adenomatous Hyperplasia in Infancy


* Departments of Pediatric Endocrinology
Pediatric Surgery
¶ Perinatology, Wilhelmina Childrens Hospital, University Medical Center Utrecht, Utrecht, Netherlands
Department of Clinical Genetics, Odense University Hospital, Odense, Denmark
|| School of Biological Sciences, University of Manchester, Manchester, United Kingdom
Persistent hyperinsulinemic hypoglycemia of infancy or congenital hyperinsulinism of the neonate is a rare condition that may cause severe neurologic damage if the disease is unrecognized or inadequately treated. Current treatment aims to restore normal blood glucose levels by providing a carbohydrate-enriched diet and drugs that inhibit insulin secretion. If medical treatment fails, then surgery is required. Because congenital hyperinsulinism may be caused either by diffuse involvement of pancreatic ß-cells or by a focal cluster of abnormal ß-cells, the extent of pancreatectomy varies. We report on 2 patients with a focal form of the disease for whom diagnosis was made with laparoscopy. Laparoscopic enucleation of the lesion was curative.
Key Words: congenital hyperinsulinism pancreatectomy laparoscopy neurodevelopmental outcome
Abbreviations: PHHI, persistent hyperinsulinemic hypoglycemia of infancy PVS, pancreatic venous sampling KATP channel, adenosine triphosphate-sensitive potassium channel SUR, sulfonylurea receptor Kir, potassium inward rectifier
Accepted May 18, 2004.
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