[Hyperinsulinism in children: new concepts - the role of imaging].
Hyperinsulinism is a rare disorder, affecting one in more than 50,000 births. It was initially thought to be due to a diffuse anomaly called nesidioblastosis, but interventional radiology-based studies demonstrated the existence of two separate forms, one difuse and the other focal. These invasive techniques have now been replaced by PET studies with 18F fluorodopa. Focal forms can be cured by surgical removal of the lesion, while the diffuse form can be treated medically or by subtotal resection of the pancreas. Biochemical and genetic studies show that focal and diffuse forms are due to various mutations of chromosome 11.
Brunelle
Francis
F
Radiologie pédiatrique, Hôpital Necker-Enfants Malades, Paris.
Ribeiro
Maria
M
Boddaert
Nathalie
N
Nihoul-Fekete
Claire
C
Jaubert
Francis
F
Rahier
Jacques
J
de Lonlay
Pascale
P
D003937:Diagnosis, Differential; D003952:Diagnostic Imaging; D004351:Drug Resistance; D005260:Female; D006801:Humans; D006946:Hyperinsulinism / Q000175:diagnosis* / Q000235:genetics / Q000628:therapy*; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D010179:Pancreas / Q000473:pathology; D012189:Retrospective Studies